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Squamous cell carcinoma of the thyroid with lymph nodal and pulmonary metastases
Giant right gastroepiploic artery pseudoaneurysm with an IVC fistula
Multiple bilateral pulmonary cement emboli
2018-11-08T18:22:17-05:002018-11-08T18:22:17-05:00Carmen M. Botía González, MD; Juana M. Plasencia Martínez, MD; Alba Patricia Solano Romero, MD; and María Jesús Gayán Belmonte, MD  CASE SUMMARY   An 80-year-old man presented to the emergency department complaining of three days’ history of shortness of breath. He presented with inmobilization due to a thoracic arthrodesis performed three months previously, but did not show any other relevant signs or symptoms. The electrocardiography and cardiac markers were normal, and remaining laboratory tests showed no abnormalities except for a d-dimer level of 6110 ng/ml.   IMAGING FINDINGS   A conventional chest X-ray revealed the thoracic arthrodesis (black asterisk, Figure 1), but no acute cardiopulmonar disease. A subsequent computed tomography pulmonary angiography (CTPA) to rule out a pulmonary embolism revealed no hypodense filling defects of the pulmonary arteries. However, the study showed the presence of high-density (around 1000 HU) material, similar to cement, inside the main left pulmonary artery (white arrow, Figure 2A) and inside several bilateral segmental and subsegmental pulmonary arteries (white arrows, Figure 2B).   DIAGNOSIS   Multiple bilateral pulmonary cement emboli   DISCUSSION   Pulmonary cement emboli constitute a frequent complication in patients who undergo percutaneous vertebral cementation techniques, usually vertebroplasty and kyphoplasty, with a reported incidence of 20-25% of all procedures, 1,2,3  even though the exact rate at which cement embolizes to the lungs is unknown because patients are not routinely screened with chest imaging before and after the procedure. 3  This phenomenon occurs due to the leakage of polymethyl methacrylate (PMMA) cement (a rapidly setting bone cement injected under fluoroscopic or CT guidance), 3  through the vertebral venous plexus when injected in an extremely liquid state or by applying excessive pressure, leading to cement migration through the inferior vena cava and the azygos system to finally reach the pulmonary circulation (pulmonary arteries). 1    The condition may produce symptoms similar to those of trombotic embolisms (such as dyspnea, tachypnea or chest pain), which may appear immediately or several months after the cementation procedure, even though most patients with pulmonary cement emboli remain asymptomatic. 3,4    On chest X-ray, pulmonary cement embolisms may sometimes appear as linear high-density opacities that follow the pulmonary vessels. However, CTPA is the modality of choice for detecting pulmonary cement embolisms; occupation of the pulmonary arteries by extremely dense material compatible with cement makes the diagnosis. 2,4,5    Treatment is controversial, although it is generally the same as for thrombotic embolisms: reperfusion procedures, mainly embolectomy when unstable, and anticoagulation therapy if not. 1  Our patient was not treated with anticoagulant therapy because of the high risk of bleeding (he had an abdominal aortic aneurysm of 6.5 cm). However, he experienced complete resolution of the symptoms.   CONCLUSION   Pulmonary cement embolisms are a frequent complication of vertebral cement placement procedures; diagnosis is made by CTPA. Both radiologists and other physicians should consider this entity in all patients with cardiopulmonary symptoms and a previous history of arthrodesis.   REFERENCES     Walter Alberto Sifuentes Giraldo, José Ramón Lamúa Riazuelo, José Ignacio Gallego Rivera, Mónica Vázquez Díaz (2013) Embolismo pulmonar por cemento tras vertebroplastia.  Reumatol Clín . 9:239-242.   Kim YJ, Lee JW, Park KW, Yeom JS, Jeong HS, Park JM, Kang HS. (2009)Pulmonary cement embolism after percutaneous vertebroplasty in osteoporotic vertebral compression fractures: Incidence, characteristics, and risk factors.  Radiology . 251(1):250-259.   Choe DH, Marom EM, Ahrar K, Truong MT, Madewell JE (2004) Pulmonary embolism of polymethyl methacrylate during percutaneous vertebroplasty and kyphoplasty.  AJR Am J Roentgenol .183(4):1097-1102.   Toru Ü, Coşkun T, Acat M, Onaran H, Gül Ş, Çetinkaya E. (2014) Pulmonary cement embolism following percutaneous vertebroplasty.  Pulmonol . [Internet]. 2014:851573.   Schmidt R, Cakir B, Mattes T, Wegener M, Puhl W, Richter M. (2005) Cement leakage during vertebroplasty: an underestimated problem?  European spine journal . 14(5):466-473.   10145
2018-11-08T18:17:13-05:002018-11-08T18:17:13-05:00Tayson Lin and Matthew Rheinboldt, MD <h2>CASE SUMMARY</h2> <p>A 57-year-old female presented with a longstanding history of left-sided hearing loss that progressively worsened over the previous year with intermittent otalgia and superimposed subjective non-pulsatile tinnitus. Audiologic evaluation was notable for mixed conductive and sensorineural hearing loss on the left. Temporal bone CT imaging demonstrated resorptive changes along the margins of the oval window and cochlear promontory on the left with capsular sparing on the right compatible with clinically suspected fenestral otosclerosis. Due to the low-level gap in air/bone conduction at diagnosis, the patient was not deemed a surgical candidate for stapes replacement and referral was made for fitting of external audiological aid.</p> <h2>IMAGING FINDINGS</h2> <p>Temporal bone noncontrast CT imaging demonstrated resorptive changes along the cochlear promontory of the left otic capsule (Figures 1-3). This pathology lies in the area of the left fissula ante fenestram, which is consistent with fenestral otosclerosis. The contralateral otic capsule demonstrates the normal dense appearance of endochondral bone.</p> <h2>DIAGNOSIS</h2> <p>Fenestral otosclerosis</p> <h2>DISCUSSION</h2> <p>Affecting fewer than 1% of the U.S. adult population, otosclerosis is a primary degenerative idiopathic osteodystrophy of the osseous otic capsule that most commonly affects Caucasians in young to middle adulthood. Though the etiology remains obscure, a familial genetic predisposition has been observed with an autosomal dominant transmission displaying incomplete penetrance.<sup>1</sup> Clinically, early otosclerosis causes conductive hearing loss. This is later admixed with sensorineural hearing loss due to disease progression.<sup>2</sup> Histopathologically, the normally dense endochondral bone of the otic capsule is initially replaced by islands of vascular, demineralized Haversian bone that gradually becomes osteosclerotic over time. This osseous resorption has also given rise to the alternative term &ldquo;otospongiosis&rdquo; for the early disease phase. Initial, or fenestral, otosclerosis occurs in the area of the fissula ante fenestram, a vestigial embryonic cleft anterior to the oval window and stapes, and typically progress to involve the cochlear promontory, vestibule and otic capsule.<sup> 3,4</sup> Involvement of the stapes footplate at the oval window leads to early conductive hearing loss via annular ligament ankylosis. With disease progression, otodystrophic damage of the cochlea results in generalized retrofenestral or cochleariform otosclerosis. The end sequelae are mixed sensorineural and conductive hearing loss with possible superimposed pulsatile tinnitus.<sup>3</sup></p> <p>Diagnosis of otosclerosis is based on a composite of clinical, audiological, and imaging findings, with radiology playing an ever-increasing role, not only in initial disease detection, but also for surgical planning and complication assessment. Typical clinical findings include the otoscopic Schwartz sign, constituted by a visible pink tinge at the cochlear promontory due to neovascular osseous changes.<sup>3,4</sup> High-resolution, noncontrast CT imaging (HRCT) utilizing sub-millimeter slice acquisition with isotropic multiplanar reconstruction is the imaging modality of choice. Fenestral otosclerosis findings on HRCT include ill-defined osseous lucency typically centered at the oval window and cochlear promontory (Figures 1-3). Generalized otic demineralization is seen in more advanced cochleariform disease (Figure 4). Differential diagnostic considerations are limited, and they include otosyphilis, Paget&rsquo;s disease and osteogenesis imperfecta.<sup>4</sup></p> <p>Meta-analyses have shown wide variability in the metrics of HRCT diagnosis of otosclerosis with more recent reports supporting collective sensitivities of over 90% for early fenestral involvement.<sup>4,5</sup> Several studies have shown added utility in the use of semi-automated histogram analysis of CT bone density datasets as well as specialized multiplanar reconstructions paralleling the stapes crus for the detection of very early histopathological changes.<sup>6,7</sup></p> <p>Various imaging-based grading systems for otosclerosis have been proposed without universal acceptance. A three-tier grading system corresponding respectively to isolated fenestral involvement, progressive localized cochlear extension, and generalized otic disease is commonly used; both inter- and intraobserver interpretative agreement have been shown to be consistently high for this grading system.<sup>8</sup> Otosclerosis is managed both medically and surgically, with oral fluorides having shown proven efficacy at slowing sensorineural hearing loss.<sup>3,9</sup> Fenestral otosclerosis is most commonly treated with stapedectomy and prosthesis insertion, while more advanced cochleariform disease is often managed with direct cochlear implantation.<sup> 10</sup> Higher grades of disease extent and lower Hounsfield densities correspond to increased audiologic air and bone conduction thresholds for hearing stimulation, as well as an increased incidence of stapes footplate complications at time of surgery. Furthermore, resorption of the osseous covering of the superior semicircular canal has been shown to be an independent contraindication to stapes prosthesis placement.<sup>2</sup> Increasingly, HRCT temporal imaging assists in cochlear versus stapes implant stratification plans and serves an invaluable role in planning the surgical approach to otosclerosis.<sup>4</sup></p> <h2>CONCLUSION</h2> <p>In summary, HRCT imaging of the temporal bone plays an ever-broadening role not only in the initial diagnosis of otosclerosis, but also in treatment planning. Manifesting initially in the so-called fenestral phase with resorptive changes at the level of the oval window, otosclerosis progresses over time to involve the cochlear promontory and otic capsule. Sclerotic overgrowth may be seen with more advanced cases, commonly resulting in narrowing of the oval window and fixation of the stapes footplate. Clinically, patients initially present with conductive hearing loss. This progresses to mixed sensorineural hearing loss as the otic capsule is increasingly affected. Advanced disease findings on HRCT have been shown to correlate independently with both surgical treatment failure and increased rates of postoperative complications.</p> <h2>REFERENCES</h2> <ol> <li>Rudic M, Keogh I, Wagner R, et al. The pathophysiology of otosclerosis: Review of current research. <em>Hear Res</em>. 2015;330(Pt A):51-56.</li> <li>Whetstone J, Nguyen A, Nguyen-Huynh A, et al. Surgical and clinical confirmation of temporal bone CT findings in patients with otosclerosis with failed stapes surgery. <em>AJNR</em>. 2014; 35: 1195-1201.</li> <li>Purohit B, Hermans R, Op de beeck K. Imaging in otosclerosis: A pictorial review. <em>Insights Imaging</em>. 2014;5(2):245-252.</li> <li>Virk J, Singh A, Lingam R. The role of imaging in the diagnosis and management of otosclerosis. <em>Otology and Neurotology</em>. 2013; 34: e55-60.</li> <li>Lagleyre S, Sorrentino T, Calmels M, et al. Reliability of high-resolution CT scan in diagnosis of otosclerosis. <em>Otology and Neurotology</em>. 2009; 30: 1152-1159.</li> <li>Yamashita K, Yoshiura T, Hiwatashi A, et al. The radiological diagnosis of fenestral otosclerosis: the utility of histogram analysis using multidector row CT. <em>Eur Arch Otorhinolaryngol</em>. 2014; 271: 3277-3282.</li> <li>Mori N, Toyama Y, Kimura N, et al. Detection of small fenestral otosclerotic lesions by high-resolution computed tomography using multiplanar reconstruction. <em>Auris Nasus Larynx</em>. 2013; 40: 36-40.</li> <li>Lee TC, Aviv RI, Chen JM et-al. CT grading of otosclerosis. <em>AJNR</em>. 2009;30 (7):1435-1439.</li> <li>Cureoglu S, Baylan MY, Paparella MM. Cochlear otosclerosis. <em>Curr Opin Otolaryngol Head Neck Surg</em>. 2010;18(5):357-362.</li> <li>Burmeister J, Rathgeb S, Herzog J. Cochlear implantation in patients with otosclerosis of the otic capsule. <em>Am J Otolaryngol</em>. 2017;38(5):556-559.</li> </ol>10143
In-transit metastasis of malignant melanoma
2018-11-08T18:11:19-05:002018-11-08T18:11:19-05:00Arun Visakh R, MBBS, MD; Thara Pratap, DMRD, MD, DNB; Abin Mathew Babu, BSc; Muhammed Jasim Abdul Jalal, MBBS, DNB; and Senthil Raja, MBBS, MD  CASE SUMMARY   A 52-year-old man presented with a patchy black pigmented lesion in the plantar aspect of the left foot, which was diagnosed as a malignant melanoma. The patient underwent wide local excision of the melanomatous lesion along with sentinel lymph node removal. The lymph node was negative for metastasis. Four weeks later the patient was referred for  18F FDG PET/CT.   IMAGING FINDINGS    18F FDG PET/CT showed an FDG-avid linear, heterogeneously enhancing soft-tissue lesion of approximately 4.8 cm in length in the medial aspect of left thigh, anterior to the left sartorius muscle, suggesting in-transit metastasis (Figure 1). It also revealed postoperative changes in left foot. No other lesions were identified to suggest distant metastasis. Final histopathology of this lesion could not be obtained as the patient was lost to follow-up.   DIAGNOSIS   In-transit metastasis of malignant melanoma   DISCUSSION   The incidence of malignant melanoma, once a rare cancer, has risen, with an average incidence ranging from 2% to 20% in most developed countries. 1  Lymph nodal and in-transit metastases in malignant melanoma have significant prognostic value, but detection of in-transit metastases is challenging.   Malignant melanoma often metastasizes through lymphatic channels to the skin or subcutaneous tissues, as well as to regional lymph nodes. Skin and subcutaneous lesions that occur within 2 cm of the primary tumor are known as satellite lesions, while those that occur beyond 2 cm are considered in-transit metastases. 2  Sentinel lymph node biopsy (SLNB) is the standard management approach for regional node metastasis. 3,4  However, SLNB cannot detect in-transit metastases, which account for most locoregional recurrences. 5  High-frequency ultrasound is considered the best modality for detecting and diagnosing in-transit metastases due to its high accuracy in detecting smaller lesions. 6,7  However, this technique has several limitations, including its dependence on operator skill, availability of an expert radiologist, and long study-performance time (at least 30-40 min for each limb or body area). Sentinel lymph node biopsy was negative for metastases in our patient. Hence, he was referred for  18F FDG PET/CT scan as a part of metastatic workup, which showed in-transit metastasis in addition to postoperative changes in left foot.   CONCLUSION   Although  18F FDG PET/CT is relatively less sensitive (especially in detecting smaller lesions) compared to high-frequency ultrasound, it has better specificity and is less dependent on operator skill. In addition, the modality can serve as a “one-stop shop” investigation due to its ability to detect distant metastases.   REFERENCES     Esther Erdei, Salina M Torres.A new understanding in the epidemiology of melanoma.  Expert Rev Anticancer Ther . 2010; 10: 1811–1823.   Balch CM, Soong SJ, Gershenwald JE, et al. Prognostic factors analysis of 17,600 melanoma patients: validation of the American Joint Committee on Cancer melanoma staging system.  J Clin Oncol . 2001; 19:3622   Morton DL, Thompson JF, Cochran AJ, et al. Sentinel-node biopsy or nodal observation in melanoma.  N Engl J Med . 2006; 355:1307.   Kretschmer L, Hilgers R, Möhrle M, et al. Patients with lymphatic metastasis of cutaneous malignant melanoma benefit from sentinel lymphnodectomy and early excision of their nodal disease.  Eur J Cancer . 2004; 40:212.   Grotz TE, Mansfield AS et al. In-transit melanoma: an individualized approach.  Oncology  (Williston Park). 2011; 25:1340-1348.   Mandavan A, Rao Ravuri P, Konathan R: High-resolution ultrasound imaging of cutaneous lesions.  Indian J Radiol Imaging . 2013, 23:269–277   Solivetti FM, Di Luca Sidozzi A et al. Sonographic evaluation of clinically occult in-transit and satellite metastases from cutaneous malignant melanoma.  Radiol Med . 2006,111: 702–708.   10141
Polyorchidism with intermittent testicular torsion
2018-11-08T18:06:42-05:002018-11-08T18:06:42-05:00Dana Sylvestre; Adam S. Brown, MD; and Deborah Conway, MD <h2>CASE SUMMARY</h2> <p>A 55-year-old man with a history of seizure disorder and depression presented to the emergency department (ED) with scrotal swelling and testicular pain. The patient reported symptoms of incomplete bladder emptying, pain posterior to the scrotum, and pain with bowel movements. He also reported scrotal swelling over the last 3 years. He conveyed suspicion of a scrotal mass. On physical examination, the penis and epididymides were normal with no lesions, discharge, or palpable masses. There was no tenderness on palpation of the perineal region. Gonorrhea/chlamydia testing was negative. Testicular Doppler US of the scrotum and testicles demonstrated polyorchidism with suggestion of subacute/chronic testicular torsion. On re-examination, the patient was clinically stable with improvement of symptoms. He was discharged from the ED and advised to seek urology follow-up care.</p> <h2>IMAGING FINDINGS</h2> <p>Gray-scale ultrasound of the left hemiscrotum demonstrated a 4.5 x 2.2 x 3.7 cm mass lateral to the left testis with homogenous hypoechogenicity and microcalcifications (Figure 1). Color Doppler ultrasound of the left hemiscrotum demonstrated absent vascular perfusion to the left lateral testicle (Figure 2). Color Doppler US of the right and left testes demonstrated normal vascular flow (Figure 3).</p> <h2>DIAGNOSIS</h2> <p>Polyorchidism with absent vascular flow in the left hemiscrotum suggesting subacute/chronic testicular torsion of the left lateral testicle.</p> <h2>DISCUSSION</h2> <p>This case reveals a rare and potentially first presentation of polyorchidism with subacute/chronic testicular torsion of the left lateral testicle in a male older than 50 years of age. The left and right testicles were normal in echogenicity on US with no apparent mass, cyst, or suspicious calcification. The testicles had normal vascular perfusion on color Doppler US with the additional left lateral testicle demonstrating an absence of vascular flow, leading to the diagnosis of polyorchidism with subacute/chronic torsion.</p> <p>Polyorchidism is rare, with approximately 140 histologically confirmed cases in the literature.<sup>1</sup> The most common presentation is triorchidism with intrascrotal left-sided predominance.<sup>1</sup> Triorchidism originates from abnormal division of the genital ridge.<sup>1,2</sup> The median age of diagnosis is 17 yo.<sup>1</sup> The supernumerary testis is typically identified subsequent to symptomatic presentation of inguinal hernia, maldescended testis, testicular torsion, hydrocele, or varicocele.<sup>1,3</sup> The differential diagnosis includes hydrocele, spermatocele, varicocele, Morgagnian cyst, and testicular neoplasm.<sup>1</sup></p> <p>US and MR can be used to support the diagnosis of polyorchidism.<sup>1,4</sup> In a meta-analysis, US identified polyorchidism in 81% of confirmed cases.<sup>1</sup> In the asymptomatic patient, the supernumerary testis echotexture, MR signal intensity, and vascular flow are similar to normal testes.<sup>3,5</sup> MR can be used for confirmation if the US is equivocal.<sup>3,4,5</sup> Polyorchidism can be managed conservatively with serial US if there is no evidence of malignancy.<sup>5</sup></p> <p>Testicular torsion is the result of spermatic cord twisting causing decreased perfusion to the testicle.<sup>6</sup> Patients with torsion commonly have the anatomic variant of the tunica vaginalis joining high on the spermatic cord.<sup>6</sup> Testicular torsion can be classified as acute or intermittent. Acute testicular torsion presents with severe scrotal pain, and it is a surgical emergency due to the risk of rapid testicular necrosis.<sup>7</sup> Intermittent testicular torsion presents with recurrent scrotal pain of short duration that resolves spontaneously.<sup>8</sup> Both acute and intermittent testicular torsion are less commonly diagnosed in adults.<sup>9</sup> Scrotal swelling can be present on examination.<sup>8,9</sup> Intermittent testicular torsion can be managed conservatively or with orchidopexy. Orchidopexy is often recommended to prevent testicular necrosis and relieve recurrent testicular pain.<sup>9</sup></p> <p>Imaging plays a key role in the diagnosis of torsion. MR T2-hypointensity indicates testicular torsion and hemorrhagic necrosis.<sup>2,10</sup> While the US echogenicity may initially be unremarkable, necrosis later develops and is indicated by a heterogenous and hypoechoic appearance.<sup>7</sup> Color Doppler US is frequently used for to evaluate acute scrotum, as it demonstrates reduced or absent perfusion of the affected testicle.<sup>2,6,7</sup> However, color Doppler US does not always demonstrate compromised vascular flow in intermittent testicular torsion.<sup>8,9</sup></p> <h2>CONCLUSION</h2> <p>Polyorchidism diagnosed in the setting of subacute/chronic testicular torsion is a rare and uncommon presentation. US and color Doppler US are the main imaging modalities used to support the diagnoses of polyorchidism and testicular torsion, respectively.</p> <h2>REFERENCES</h2> <ol> <li>Bergholz R and Wenke K. Polyorchidism: a meta-analysis. <em>Pediatric Urology</em>. 2009;182(5):2422-2427.</li> <li>Kumar K, Das D, Shivaraj. Triorchidism with Torsion. <em>Ann Med Health Sci Res</em>. 2012;2(2): 199&ndash;201.</li> <li>Yal&ccedil;ınkaya S, Şahin C, Feyzullah Şahin A. Polyorchidism: sonographic and magnetic resonance imaging findings. <em>Can Urol Assoc</em> <em>J</em>. 2011;5(5):E84&ndash;E86.</li> <li>Bhogal R, Palit A, Prasad K. Conservative management of polyorchidism in a young man: a case report and review of literature. <em>Pediatr Surg Int</em>. 2007;23(7):689&ndash;691.</li> <li>Arslanoglu A, Tuncel SA, Hamarat M. Polyorchidism: color Doppler ultrasonography and magnetic resonance imaging findings. <em>Clin Imaging</em>. 2013;37(1):189-91.</li> <li>Aso C, Enr&iacute;quez G, Fit&eacute;, M, et al. Gray-scale and color doppler sonography of scrotal disorders in children: an update. <em>Radiographics</em>. 2005;25(5):1197-214.</li> <li>Agrawal AM, Tripathi PS, Shankhwar A, Naveen C. Role of Ultrasound with Color Doppler in Acute Scrotum Management. <em>J Family Med Prim Care</em>. 2014;3(4):409&ndash;412.</li> <li>Eaton SH, Cendron MA, Estrada CR, et al. Intermittent Testicular Torsion: diagnostic features and management outcomes. <em>The Journal of Urology</em>. 2005;174(4Pt2):1532-5.</li> <li>Al-Kandari AM, Kehinde EO, Khudair S, et al. Intermittent Testicular Torsion in Adults: An Overlooked Clinical Condition. <em>Med Princ Pract</em>. 2017;26(1):30-34.</li> <li>Watanabe Y, Nagayama M, Okumura A, et al. Imaging of testicular torsion: Features of testicular hemorrhagic necrosis and clinical outcomes. <em>J Magn Reson Imaging</em>. 2007;26(1):100&ndash;108.</li> </ol>10140
2018-11-08T18:01:23-05:002018-11-08T18:01:23-05:00Prapti Singh, DO; Manu Singh, MD; John K Yoon, MD; and Bernard Chow, MD <h2>CASE SUMMARY</h2> <p>A 36-year-old Hispanic female presented to the emergency department with worsening left lower quadrant pain of 3 days&rsquo; duration. She described the pain as intermittent, cyclic, and related to her menstrual cycle. The patient reported nausea and loss of appetite but denied emesis or changes to bowel movements. Patient admitted to menorrhagia and her menstrual period ended 3 days previously. Physical exam illustrated tenderness to palpation of the left lower quadrant superficially with no tenderness to deep palpation. There was a 1 &times; 3 cm area of chronic purplish skin changes with accompanying small nodules. The area was in line with her previous ceasarean delivery closure site. Patient&rsquo;s complex of abdominal and gynecological complaints coupled with physical exam findings prompted computed tomography imaging.</p> <h2>IMAGING FINDINGS</h2> <p>Computed tomography of contiguous axial images with sagittal and coronal reformats with contrast demonstrated a 2.5 &times; 3.8 &times; 3.5 cm speculated, soft-tissue mass in the left inguinal region with an associated 1.4 &times; 2.6 cm rim-enhancing, subcutaneous fluid collection at the inferior anterior aspect of the mass (Figures 1,2. Intraoperative findings included a 6-cm cystic abdominal wall mass with old, dark hematoma fluid. Pathology report verified the tissue as benign fibroconnective tissue with multiple foci of endometriosis, consistent with an endometrioma. Given the wide variety of potential deposition sites of endometrial tissue, it is imperative to be aware of its malleable clinical presentation.</p> <h2>DIAGNOSIS</h2> <p>Scar endometriosis. Differential Diagnoses: stitch granuloma, neoplasm, neuroma, hematoma, desmoid tumor, abscess, lymphadenopathy.</p> <h2>DISCUSSION</h2> <p>Scar endometriosis is defined by the implantation or/and growth of endometrial tissue postsurgically at the incision site. These lesions are a product of gynecological or obstetrical surgery, including laparotomy and laparascopic approaches of abdominal and pelvic cases. Endometriosis typically presents as the embedding of endometrial tissue and glands in nonuterine locations such as the adnexa, cul de sac, GI organs and extremities. The incidence of scar endometriosis has been shown to be less than 2%,<sup>1,2</sup> with the most common subtype being postcesarean section.<sup>3</sup> Given the wide differential diagnosis and history of incorrect diagnoses, scar endometriosis should be evaluated for its clinical signs, with radiological assessment leading to its diagnosis.</p> <p>While our patient presented with cyclical pain, it should be noted that cyclical, noncyclical, continuous, and nonexistent pain patterns have been noted.<sup>4-6</sup> The onset of pain can range from months to years.<sup>5</sup> On physical exam, many patients show mobile and immobile nodular skin changes.<sup>6 </sup></p> <p>In addition to clinical signs, radiological imaging is key to accurate diagnosis and exclusion. Studies show CT, MRI and ultrasound to be reasonable options for diagnosing scar endometriosis.<sup>3</sup> One study discussed sonography&rsquo;s advantages of visualizing fistulas, tracts and vascularity, rendering it more accurate than CT, MRI, and FNA biopsy/laparoscopy which, in chronic cases, has led to misdiagnosis.<sup>5</sup> Another study showed MRI superior in detecting scar endometriosis.<sup>6</sup> A recent case series study suggested that transabdominal sonoelastography may be beneficial in further delineating surfaces, leading to more precise margin removal preoperatively.<sup> 7</sup> While these results indicate there is no gold standard in initial assessment of suspected scar endometriosis, the advantages of each should be understood.</p> <p>The clinical picture and potential acute treatment needed for the differential diagnoses highlights the need to identify and comprehensively assess potential cases of scar endometriosis.</p> <h2>CONCLUSION</h2> <p>Given the varied and recurrent clinical presentation of scar endometriosis, it is important to take note of its wide-ranging forms. Its increasing clinical prevalence emphasizes the need to further evaluate the most beneficial diagnostic imagine modality to guide appropriate management.</p> <h2>REFERENCES</h2> <ol> <li>Danielpour PJ, Layke JC, and Durie, N. Scar endometriosis, a rare cause for a painful scar: A case report and review of the literature.&nbsp;<em>Can J Plast Surg</em>. 2010; 18(1): 19-20.</li> <li>Mistrangelo, M., Gilbo, N., Cassoni, P., et al. Surgical scar endometriosis. <em>Surgery Today.</em> 2013; 44(4): 767-772. doi:10.1007/s00595-012-0459-43.</li> <li>Gidwaney R, Badler RL, Yam, BL, et al. Endometriosis of abdominal and pelvic wall scars: Multimodality imaging findings, pathologic correlation, and radiologic mimics.<em> RadioGraphics</em>. 2012; 32(7): 2031-2043. doi:10.1148/rg.327125024.</li> <li>Patel, N. Scar endometriosis: A sequel of caesarean section. <em>J Clin Diagn Res</em>. 2014; 8(4), 135-140. doi:10.7860/jcdr/2014/7554.4267.</li> <li>Francica G. Reliable clinical and sonographic findings in the diagnosis of abdominal wall endometriosis near cesarean section scar. <em>World J Radiol.</em> 2012;4(4): 135-140. doi:10.4329/wjr.v4.i4.135.</li> <li>Ozel L, Sagiroglu J, Unal A, et al. Abdominal wall endometriosis in the cesarean section surgical scar: A potential diagnostic pitfall. J<em> Obstet Gynaecol Res</em>. 2012 Mar;38(3):526-530. doi: 10.1111/j.1447-0756.2011.01739.x.</li> <li>Fawzy M, Amer T. Efficacy of transabdominal sonoelastography in the diagnosis of caesarean section scar endometrioma: A pilot study. <em>Journal of Obstet Gynaecol</em>. 2015;35(8):832-834. doi:10.3109/01443615.2015.1011107.</li> </ol>10106
Congenital malignant melanoma of the dura
2018-10-16T16:30:04-04:002018-10-16T16:30:04-04:00Dana Sylvestre; Charles Brown, MD; and Andreas Schilling, MD <h2>CASE SUMMARY</h2> <p>A male neonate presented at birth with a subcutaneous mass in the parieto-occipital region. He had an unremarkable birth history. Brain MR imaging at 4 days&rsquo; old revealed a large mass. The mass was resected. The mass was identified as dural-based malignant melanoma in the parieto-occipital region. The lesion extended between the sutures into subcutaneous tissue. The tissue was red-brown with scattered cytoplasmic pigment and extracellular pigment accumulation. Neoplastic cells were positive for S100, HMB-45, and vimentin. The BRAF mutation was not detected. The patient developed numerous hyperpigmented 2- to 3-mm nodular lesions, which were histopathologically identified as malignant melanoma. After resection, the patient began chemotherapy at 3 mo old. Postoperative brain MRI and CT demonstrated no evidence of intracranial mass, hemorrhage, or hydrocephalus. PET/CT revealed no evidence of metastases. The postoperative midline occipital defect was repaired with cranioplasty. He has met all developmental milestones with no residual neurological deficits.</p> <h2>IMAGING FINDINGS</h2> <p>Brain MRI demonstrates a mass with intracranial T1 hyperintensity/T2 hypointensity and extracranial heterogenous T1 hyperintensity/T2 hypointensity with mass effect on the patent superior sagittal sinus and brain parenchyma (Figures 1,2). Computed tomography demonstrates posterior cortical irregularity with posterior scalp edema (Figure 3).</p> <h2>DIAGNOSIS</h2> <p>Congenital malignant melanoma of the dura</p> <h2>DISCUSSION</h2> <p>This case demonstrates congenital malignant melanoma of the dura mater with extracranial extension through the occipital bone. Magnetic resonance imaging (MRI) revealed mostly T1 hyperintensity and T2 hypointensity. Computed tomography revealed extracranial extension of the mass through the occipital bone.</p> <p>Congenital brain tumors (CBTs) are uncommon diagnoses which account for less than 2% of pediatric brain tumors.<sup>1,2</sup> There are many different types of CBTs, such as teratoma, astrocytoma, glioblastoma multiforme, PNET, and choroid plexus tumor.<sup>1,2</sup> Teratoma is the most frequently diagnosed CBT.<sup>1,2</sup> The CBT site is more commonly supratentorial than infratentorial.<sup>1,2</sup> There are limited guidelines available for diagnosing and managing a CBT.<sup>2</sup> The prognosis is guarded due to the large size of the tumor.<sup>1</sup> Imaging plays a vital role in the detection and surgical treatment of CBT.<sup>1</sup></p> <p>Congenital malignant melanoma is very rare. The origin of this condition can be classified as <em>de novo</em>, transplacental, or from giant congenital malignant nevus.<sup>3</sup> In a review of 20 reported cases, Scalvenzi et al found that the diagnosis has a variable outcome and is more common in males.<sup>3</sup> It is likely that the tumor arose <em>de novo</em>, as there was no cutaneous GCMN present or maternal diagnosis of malignant melanoma. The immunohistochemistry of the intracranial mass and cutaneous lesions was consistent with malignant melanoma. Neoplastic cells were positive for S100, HMB-45, and vimentin. S-100 is the most sensitive for detection of melanocytic lesions, whereas HMB-45, MART-1/Melan-A, tyrosinase, and MITF are more specific and less sensitive.<sup>4</sup></p> <p>Meningeal melanoma is more likely to develop from leptomeninges (pia, arachnoid) than the pachymeninges (dura).<sup>5</sup> According to Hayward, a central nervous system (CNS) malignant melanoma should be diagnosed as primary if there is &ldquo;no malignant tumour outside the CNS, involvement of the leptomeninges, intramedullary spinal lesions, hydrocephalus, tumour in the pituitary or pineal gland, or a single intracerebral lesion.&rdquo;<sup>6</sup> This patient&rsquo;s cutaneous lesions developed after the dural mass was resected. No other CNS masses were identified. These findings are consistent with a dural-based primary lesion.</p> <p>Computed tomography and brain MRI were beneficial in narrowing the differential diagnosis. The CT scan demonstrated the intracranial mass with extracranial extension and posterior scalp swelling. Preoperative T1-weighted MRI demonstrated intracranial and extracranial hyperintensity, and T2-weighted MRI demonstrated mostly intracranial and extracranial hypointensity. Sinus pericranii, lymphangioma, and meningoencephalocele were considered unlikely. The MRI did not exclude hemangioma or hematoma. Brain MRI was consistent with the typical melanotic pattern of malignant melanoma with hyperintensity on T1-weighted images and hypointensity on T2-weighted images.<sup>7,8</sup> Tumors with &gt;10% melanin-containing cells are more likely to demonstrate the melanotic pattern.<sup>7</sup> However, this pattern on T1- and T2-weighted MRI is not uniformly found in all malignant melanoma cases.<sup>7</sup></p> <h2>CONCLUSION</h2> <p>This case describes dural based intracranial and extracranial congenital malignant melanoma, which is is an exceptionally rare diagnosis. Brain MRI of the tumor demonstrated the typical melanotic pattern of predominantly T1 hyperintensity and T2 hypointensity. Computed tomography revealed cortical irregularity and posterior scalp swelling. Neuroimaging with CT and MR was essential in guiding management and monitoring for recurrence of this condition.</p> <h2>REFERENCES</h2> <ol> <li>Buetow P, Smirniotopoulos J, Done S. Congenital brain tumors: A review of 45 cases. <em>Am J Neuroradiol</em>. 1990; 11:793-799.</li> <li>Sugimoto M, Kurishima C, Masutani S, et al. Congenital brain tumor within the first 2 months of life. <em>Pediatr and Neonatol</em>. 2015; 56:369-375.</li> <li>Scalvenzi M, Palmisano F, Cacciapuoti S, et al. Giant congenital melanocytic naevus with proliferative nodules mimicking congenital malignant melanoma: A case report and review of the literature of congenital melanoma<em>. Case Rep Derm Med</em>. 2013;2013: Article ID 473635, 6 pages.</li> <li>Ohsie S, Sarantopoulos G, Cochran A, et al. Immunohistochemical characteristics of melanoma. <em>J Cutan Path</em>. 2008; 35:433-444.</li> <li>Escott E. A variety of appearances of malignant melanoma in the head: a review. <em>Radiographics</em>. 2001; 21:625-639.</li> <li>Hayward R. Malignant melanoma and the central nervous system. <em>J Neuro Neurosurg Psychiatry</em>.1976; 39:526-530.</li> <li>Isiklar I, Leeds N, Fuller G, et al. Intracranial metastatic melanoma: correlation between MR imaging characteristics and melanin content<em>. Am J Roentgenol</em>. 1995; 165:1503-1512.</li> <li>Smith A, Horkanyne-Szakaly I, Schroeder J. From the radiologic pathology archives: Mass lesions of the dura: beyond meningioma&mdash;radiologic-pathologic correlation. <em>Radiographics</em>. 2014;34: 295-312.</li> </ol>10104
Primary localized ureteral amyloidosis mimicking urothelial carcinoma
2018-10-16T16:24:26-04:002018-10-16T16:24:26-04:00Syed T. Ali, MD; Shetal Shah, MD; and Christopher Przybycin, MD <h2>CASE SUMMARY</h2> <p>In 2005, a 74-year-old Indian male with a history of coronary artery disease, hyperlipidemia, and diabetes presented with left-sided flank pain without hematuria. The patient was a non-smoker and was on aspirin 81mg daily at home. Except for a mildly elevated blood glucose, his labs including comprehensive metabolic panel, complete blood count and coagulation studies (PT, PTT, and INR) were within normal limits. Urine cytology was negative for malignant cells. A noncontrast CT scan was ordered to evaluate for renal calculi but had revealed a left-sided, short segmental, mid-ureteral thickening with a punctate calcification and mild hydroureteronephrosis (Figure 1).</p> <h2>IMAGING FINDINGS</h2> <p>On a visit in 2013, the patient was evaluated with CT urogram without and with IV contrast (150cc IV iohexol 300, Figure 2). CT urogram without contrast demonstrated wall thickening with circumferential calcification of the middle left ureter with mild luminal narrowing without hydronephrosis. Corticomedullary phase did not depict an enhancing renal or ureteral mass. Excretory phase depicted a mild irregular wall thickening with no intraluminal filling defect. Cystoureteroscopic biopsy was subsequently performed and revealed abundant amorphous eosinophilic material consistent with amyloid. The presence of amyloid was confirmed by Congo red staining, showing apple-green birefringence under polarized light, as well as by a Thioflavin S stain under fluorescence microscopy, which was positive (Figure 3).</p> <h2>DIAGNOSIS</h2> <p>Primary localized ureteral amyloidosis mimicking urothelial carcinoma</p> <h2>DISCUSSION</h2> <p>Primary localized amyloidosis of the ureter is a rare disorder that is clinically and radiographically difficult to distinguish from urothelial carcinoma. Amyloidosis comprises a variety of protein-folding disorders that result in an extracellular deposition of protein aggregates, or amyloid, in tissues and organs, and may be systemic or localized, with the systemic form being classified as primary or secondary disease.<sup>1</sup> Primary amyloidosis is associated with immune dyscrasias, such as multiple myeloma and Waldenstr&ouml;m&rsquo;s macroglobulinemia,<sup>2,3 </sup>while secondary amyloidosis is associated with chronic inflammatory conditions, such as rheumatoid arthritis, Crohn&rsquo;s disease and tuberculosis.<sup>4</sup> Localized amyloidosis is much less common than systemic amyloidosis and occurs in about 10-20% of amyloidosis cases.<sup>3,5</sup> This condition can occur in the lungs, trachea, larynx, tongue, skin, nervous system, gastrointestinal system and genitourinary system.<sup>3,6</sup></p> <p>Localized amyloidosis of the urinary tract is of clinical importance because it mimics urothelial carcinoma. Depending on the degree of disease involvement, patients may present with painless hematuria, irritative urinary symptoms, and obstructive symptoms.<sup>3,7</sup> Although the cause of localized amyloidosis of the urinary tract is unknown, local monoclonal plasma cell infiltrates and local amyloid synthesis have been suggested as causes.<sup>3,7</sup> Within the genitourinary tract, localized amyloidosis is commonly found in the prostate and seminal vesicles, but can also occur in the bladder, renal pelvis, ureter and urethra.<sup>5,9</sup> Ultrasound, CT scans and MRI are often used as initial methods to evaluate patients with urinary tract amyloidosis.</p> <p>Primary localized amyloidosis of the ureter has been reported to occur more often in females, with the average age of presentation across both genders to be 58.<sup>4</sup> It is a rare disorder and occurs less frequently than amyloidosis of the prostate, seminal vesicles and bladder.<sup>5,9</sup> In a previous study, amyloidosis is typically unilateral and most commonly affects the distal third of the ureter.<sup>9 </sup> Patients can present with features of ureteral stricture and hydronephrosis.<sup>10</sup> Ultrasound and intravenous pyelography can reveal hydronephrosis.<sup>4 </sup>Computed tomography may identify nonspecific diffuse thickening of ureteral walls and calcifications, which may also be seen with urothelial carcinoma, tuberculosis, schistosomiasis or hematoma.<sup>4,7,8,10,11</sup> Localized amyloidosis has previously been described to present on MRI T2 weighted imaging as a hypointense lesion without obvious mass-effect, a characteristic that may distinguish amyloidosis from carcinoma.<sup>4,8,9,10</sup></p> <p>Urine cytology is insufficient for distinguishing amyloidosis from carcinoma because of the subendothelial location of many amyloid deposits and because of the limited (50%) sensitivity for urothelial carcinoma.<sup>6</sup> Definitive diagnosis of amyloidosis is achieved with biopsy. Congo red staining of amyloid under light microscopy with polarized light classically produces the appearance of apple-green birefringence. Treatment of the amyloid sample with potassium permanganate may help distinguish the type of amyloid protein.<sup>4 </sup>Immunohistochemistry or mass spectrometry may provide further information about protein components of the particular amyloid.<sup>4</sup></p> <p>Localized amyloidosis of the ureter usually is benign and typically carries a good prognosis.<sup>4,6,12</sup> Systemic amyloidosis must be ruled out prior to treatment, as certain diseases, including multiple myeloma, Hodgkin lymphoma, and renal cell carcinoma can manifest with systemic amyloidosis.<sup>13</sup> Systemic amyloidosis can be excluded by utilizing serum and urine electrophoresis, chest radiography and if appropriate, rectal biopsy or fine needle aspiration of subcutaneous fat.<sup>2 </sup>Once localized amyloidosis of the urinary tract is confirmed, various treatment options may be considered. Patients with non-obstructing amyloidosis can be observed and followed with serial imaging to assess disease status.<sup>12</sup> Cases of ureteral amyloidosis in which malignancy could not be excluded preoperatively or intraoperatively, or cases in which ureteral involvement precluded renal preservation have been treated with nephroureterectomy.<sup>4 </sup>Alternative surgical treatments may be utilized in patients in whom biopsy can confirm amyloidosis prior to surgery, and include ureteroneocystostomy, resection with end anastomosis of the ureter, and autotransplantation.<sup>4,8</sup> Medical management of ureteral amyloidosis with oral colchicine and oral or topical dimethyl sulphoxide have been reported.<sup>4,8,9</sup></p> <h2>CONCLUSION</h2> <p>Localized amyloidosis of the urinary tract is rare and is difficult to differentiate from malignancy based on clinical and radiographic features alone. MRI findings of T2 weighted hypointense lesions may help distinguish amyloidosis from malignancy. Additional studies would help characterize the sensitivity and specificity for this study. Radiographic features of the other imaging modalities may be nonspecific, but it is important for clinicians and radiologists to be cognizant of this disease so proper management and treatment may be provided.</p> <h2>REFERENCES</h2> <ol> <li>Chitale S, Morsey M,Peat D,Webb R.Amyloidosis of lower genitourinary tract: a review. <em>EAU-EBU Update Series.</em> 2007;5:70-76.</li> <li>Merrimen JL, Alkhudair WK, Gupta R. Localized amyloidosis of the urinary tract: case series of nine patients. <em>Urology.</em> 2006;67(5):904&ndash;909</li> <li>DeSouza MA, Rekhi B, Thyavihally YB, et al. Localized amyloidosis of the urinary bladder, clinically masquerading as bladder cancer. <em>Indian J Pathol Microbiol.</em> 2008;51:415-417.</li> <li>Ding X, Yan X, Ma X, et al. Localized amyloidosis of the ureter: A case report and literature review. <em>Can Urol Assoc J.</em> 2013;7(11-12):E764-E767. doi:10.5489/cuaj.576.</li> <li>Kawashima A, Alleman WG, Takahashi N, et al. Imaging evaluation of amyloidosis of the urinary tract and retroperitoneum. <em>Radiographics.</em> 2011;31:1569-1582.</li> <li>Zhou F, Lee P, Zhou M, Melamed J, Deng F-M. Primary localized amyloidosis of the urinary tract frequently mimics neoplasia: a clinicopathologic analysis of 11 cases. <em>Am J Clin Exp Urol.</em> 2014;2(1):71-75.</li> <li>Domiciano DS, De Carvalho JF. Primary localized amyloidosis of the ureter. <em>Isr Med Assoc J.</em> 2008;10:237-238.</li> <li>Tsujioka Y, Jinzaki M, Tanimoto A, et al.. Radiological findings of primary localized amyloidosis of the ureter. <em>J Magn Reson Imaging.</em> 2012;35:431-435.</li> <li>Singh SK, Wadhwa P, Nada R, Mohan VC, Singh P, et al. Localized primary amyloidosis of the prostate, bladder and ureters. <em>Int Urol Nephrol. </em>2005;37:495&ndash;497</li> <li>Weiwei Z, Yi H, Jinsong Z. Primary localized amyloidosis of the ureter. <em>Abdom Imaging.</em> 2011;36:609-611.</li> <li>Paidy S, Unold D, Catanzano TM. AIRP best cases in radiologic-pathologic correlation: localized amyloidosis of the renal pelvis. <em>Radiographics.</em> 2012;32:2025-2030.</li> <li>Borza T, Shah RB, Faerber GJ, Wolf JS. Localized amyloidosis of the upper urinary tract: a case series of three patients managed with reconstructive surgery or surveillance. <em>J Endourol.</em> 2010;24(4): 641&ndash;644.</li> <li>Raja K, Ahmed E, Mubarak M, et al. Primary Localized Amyloidosis of Urinary Bladder: A Case Report and Review of Literature. <em>Nephrourol Mon.</em> 2013;5(5):994-996.</li> </ol>10102
Interhemispheric cyst with associated dysplasia of the corpus callosum
2018-10-16T16:19:05-04:002018-10-16T16:19:05-04:00Ryan Crete, MD; Scott A. Jorgensen, MD; Alexander J. Towbin, MD; and Richard Towbin, MD <!-- ><p><a href="/Communities/pediatric-imaging-community" target="_self"><img src="http://cdn.agilitycms.com/applied-radiology/PDFs/Pediatric/AR_featured-pediatric-case_4article.jpg" alt="" width="300" height="36" /></a></p><--> <h2>CASE SUMMARY</h2> <p>A 12-year-old girl presented with a first time seizure. A CT scan was interpreted as interhemispheric arachnoid cyst. MRI revealed an interhemispheric cyst with left frontal lobe gliosis and cortical dysplasia, as well as aplasia of the rostrum and left genu of the corpus callosum. It was later elucidated that the patient exhibited mild cognitive delay.</p> <h2>IMAGING FINDINGS</h2> <p>Axial nonenhanced CT [NECT, Figure 1) shows an extra-axial, left parasagittal mass associated with the anterior falx cerebri. The mass demonstrates homogenous fluid density and displaces the anterior left frontal lobe laterally. No calcification, parenchymal edema or hydrocephalus is present.</p> <p>MRI shows the mass to exhibit CSF signal on all pulse sequences. Axial ADC map (Figure 2) shows no restricted diffusion. Sagittal T2WI (Figure 3) shows the CSF signal intensity mass associated with aplasia of the rostrum and portion of the genu of the corpus callosum (CC). The remainder of the CC is well formed. No evidence of communication with the ventricular system. Axial FLAIR images (Figure 4) show homogenous fluid signal suppression with absence of normal grey matter bordering the cyst in the medial left frontal lobe. There is also increased signal indicating gliosis and/or dysplasia seen posterolateral to the mass with a single focus of cystic change and no evidence of communication with the ventricular system in the axial plane. Axial T1WI (Figure 5) shows CSF signal mass with absence of normal grey matter in the displaced medial frontal lobe. The anterolateral left frontal lobe grey matter is thinned and dysplastic.</p> <h2>DIAGNOSIS</h2> <p>Interhemispheric cyst associated with partial agenesis of the corpus callosum. Differential diagnosis includes arachnoid cyst, porencephalic cyst, dermoid/epidermoid cyst, low grade cystic neoplasm (eg, ganglioglioma, DNET), and parasitic cyst (eg, hydatid disease).</p> <h2>DISCUSSION</h2> <p>Callosal dysgenesis/agenesis and intracranial cysts are two common intracranial malformative lesions and their association has been well documented.<sup>1-7</sup> Patients with that combination of anomalies are frequently identified in utero by sonography.<sup>1</sup> Older infants may present with macrocephaly, neurodevelopmental disorders and seizure.<sup>2,3</sup> The callosal deformity may range from complete agenesis to subtle segmental dysgenesis.<sup>1,2</sup> How the cyst develops is controversial, but it is clear that cysts may not become apparent by imaging until late in gestation.<sup>1,4 </sup> Two basic types of cysts have been described.<sup>2,3</sup> Type 1 cysts are likened to diverticula and communicate with the lateral or third ventricles. Type 2 cysts do not communicate with the ventricles. Several subtypes have been described based on associated anomalies such as hydrocephalus and dysplastic/heterotopic grey matter.<sup>2,3</sup> There is evidence that type 1 cysts may form secondarily due to communicating hydrocephalus, and these cysts are primarily ependymal-lined.<sup>3</sup> Pathologic evaluation for type 2 cysts may reveal arachnoid or glial-ependymal cells. Based on the classification scheme proposed by Barkovich, the case presented is a type 2c interhemispheric cyst.<sup>2,3</sup> Type 2c associations classically present in childhood with seizures and developmental delay, and are associated with subcortical heterotopia.</p> <p>Ultrasound is commonly used as a prenatal screening tool, and both callosal dysgenesis and intracranial cysts may be identified. If callosal dysgenesis is identified prenatally, repeat sonographic evaluation is indicated prior to delivery to evaluate for macrocephaly and hydrocephalus.<sup>1,6</sup> CT is a useful screening examination in the setting of seizure, but as in the presented case, often will not fully characterize associated abnormalities. MRI is the preferred modality for complete characterization of the congenital anomalies. In this case, the CT finding of a midline cyst was originally thought to represent an isolated arachnoid cyst, but MRI demonstrated the callosal dysgenesis and associated grey matter dysplasia with gliosis in the adjacent white matter.</p> <p>With MRI Type 1 cysts typically appear isointense to CSF on all pulse sequences, while type 2 cysts are often hyperintense to CSF on both T1 and T2WI.<sup>3</sup> Type 2 cysts may also be multiloculated, and communication between locules and ventricles may be difficult to visualize and exclude.<sup>2,3 </sup>The imaging triad AVID (Asymmetric Ventriculomegaly, Interhemispheric cyst, and Dysgenesis of the corpus callosum) has been proposed to aid with distinction from aqueductal stenosis and porencephaly.<sup>6</sup> Associated subependymal and subcortical heterotopia, polymicrogyria, deficient falx, fused thalami and hydrocephalus may also be associated.<sup>2</sup></p> <p>Developmental delay and focal neurologic deficits are now shown to vary significantly in both type 1 and 2 cysts, ranging from severe delay and medically refractive seizures to average aptitude, normal social interactions and no clinical findings.<sup>1,2</sup> Our patient was seen for a first-time seizure and exhibited mild developmental delay. Overall, seizures are seen in about half of patients, and lifelong epilepsy is common. Cyst fenestration or shunting may be required, and may result in significant clinical improvement in appropriately selected individuals.<sup>2</sup></p> <h2>CONCLUSION</h2> <p>Interhemispheric cysts should not be dismissed as arachnoid cysts without complete characterization of the corpus callosum, adjacent cortex and involved white matter given the common association between these anomalies.</p> <h2>REFERENCES</h2> <ol> <li>Pavone P, Barone R, Baieli S, Parano E, Incorpora G, Ruggieri M. Callosal anomalies with interhemispheric cyst: expanding the phenotype. <em>Acta Paediatr</em>. 2005; 94(8): 1066-72.</li> <li>Barkovich A, Raybaud C. Pediatric Neuroimaging, 5th ed. Philadelphia, PA: Lippincott Williams &amp; Wilkins; 2011.</li> <li>Barkovich A, Simon E, Walsh C. Callosal agenesis with a cyst: a better understanding and new classification. <em>Neurology</em>. 2001; 56(2): 220-7.</li> <li>Stroustrup S, Levine D. Appearance of an interhemispheric cyst associated with agenesis of the corpus callosum. <em>AJNR</em>. 2004; 25: 1037-40.</li> <li>Tange Y, Aoki A, Mori K, Niijima S, Maeda M. Interhemispheric glioependymal cyst associated with agenesis of the corpus callosum. <em>Neurol Med Chir (Tokyo).</em> 2000; 40: 536-42.</li> <li>Oh KY, Kennedy AM, Selden NR, McLean L, Sohaey R. Asymmetric ventriculomegaly, interhemispheric cyst, and dysgenesis of the corpus callosum (AVID): an imaging triad. <em>J Ultrasound Med</em>. 2012; 31(11): 1811-20.</li> <li>Probst FP. Agenesis of the corpus callosum. <em>Acta Radiol</em>. 1973; 331(Suppl): 1&ndash;152. Comments</li> <li>Barkovich A, Raybaud C (2011) Pediatric neuroimaging, 5th edn. Lippincott Williams &amp; Wilkins, Philadelphia, PA</li> <li>Stroustrup S, Levine D (2004) Appearance of an interhemispheric cyst associated with agenesis of the corpus callosum. AJNR 25:1037-1040</li> <li>Barkovich A, Simon E, Walsh C (2001) Callosal agenesis with a cyst: a better understanding and new classification. Neurology 56(2):220-7</li> <li>Pavone P, et al (2005) Callosal anomalies with interhemispheric cyst: expanding the phenotype. Acta Paediatr 94(8):1066-72</li> <li>Tange Y, Aoki A et al (2000) Interhemispheric glioependymal cyst associated with agenesis of the corpus callosum. Neurol Med Chir (Tokyo) 40:536-42</li> <li>Oh KY et al (2012) Asymmetric ventriculomegaly, interhemispheric cyst, and dysgenesis of the corpus callosum (AVID): an imaging triad. J 14. Ultrasound Med 31(11):1811-20</li> <li>Probst FP (1973) Agenesis of the corpus callosum. Acta Radiol 331(Suppl):1&ndash;152</li> </ol>10100
Percutaneous thrombin and coil occlusion of right gastroepiploic artery pseudoaneurysm
2018-10-16T16:14:14-04:002018-10-16T16:14:14-04:00Allan Zhang, DO; Charan Singh, MD; Dipesh Patel, MD; and Pat Svrcek, MD <h2>CASE SUMMARY</h2> <p>A 76-year-old woman from a skilled nursing facility who was nonverbal at baseline secondary to an anoxic brain injury sustained twenty years previous presented to the emergency department after her care providers noticed bright red blood in her stool. Patient was reported to be taking a proton pump inhibitor for stress ulcer prophylaxis and low-dose aspirin daily. Her initial hemoglobin and hematocrit on arrival was 13.5g/dL and 38.8%, respectively, both within normal limits for her age.</p> <h2>IMAGING FINDINGS</h2> <p>Abdominal and pelvic CT angiography was performed per institutional protocol for gastrointestinal bleed. The exam demonstrated a 5.4 &times; 3.7 &times; 4.4 cm (TRV by AP by CC) hematoma within the first portion of the duodenum. On the postcontrast images, the majority of the hematoma demonstrated peripheral thrombus formation with a central area of active contrast extravasation measuring approximately 2.3 cm, concerning for a pseudoaneurysm (Figure 1).</p> <p>Under local anesthesia (Lidocaine 2%) and using the Seldinger technique, a selective celiac axis catheterization (Cobra Glidecath, 5F, 65-cm-long) with a guidewire (Radifocus guidewire, 0.035&rdquo;, 150-cm-long) confirmed the large right gastroepiploic artery pseudoaneurysm (Figure 2). Despite multiple efforts using a Simmons 2 catheter and a 3 French Progreat microcathether, attempts to reach the pseudoaneurysm via endovascular approach were unsuccessful due to vessel tortuosity.</p> <p>At this point, percutaneous thrombin with coil occlusion was suggested in light of the patient&rsquo;s high-risk clinical condition. Under ultrasound guidance, a 22-gauge needle was advanced into the pseudoaneurysm and a A 4 French vascular sheath placed. Three-thousand units of thrombin was injected, resulting in instant thrombosis of the pseudoaneurysm. An 8 mm coil was also inserted for additional hemostasis; however, due to thromboses, the coil stayed within the margin of the pseudoaneurysm (Figure 3). A follow-up common hepatic angiogram showed no significant opacification of the pseudoaneurysm.</p> <p>A follow-up ultrasound examination performed one month later showed a smaller and thrombosed pseudoaneurysm with no internal color flow (Figure 4).</p> <h2>DIAGNOSIS</h2> <p>Right gastroepiploic artery pseudoaneurysm</p> <h2>DISCUSSION</h2> <p>Visceral pseudoaneurysms are rare vascular conditions that are often found incidentally because patients present with no specific clinical signs or symptoms. As such, it is not uncommon to discover visceral pseudoaneurysms after they have ruptured, with resultant high mortality rates.<sup>1</sup> Management of bleeding pseudoaneurysms remains debatable.<sup>2</sup></p> <p>Generally, transcatheter-based approaches are used in cases such as these with embolization of the afferent and efferent arteries feeding the pseudoaneurysm.<sup>3</sup> With the advent of microcatheters, this embolization can be achieved with coils, particles, or glue. In our case, due to the significant collateral circulation in the celiac arterial system (gastroepiploic, short gastric, and splenic arteries), the parent artery involved can also be sacrificed with low risk for local ischemia. Either endovascular or percutaneous approaches are used in these cases.</p> <p>Due to the tortuosity of the vessels and difficulty in reaching feeding branches of pseudoaneurysms, the endovascular approach may be limited in utility, as in our case. With respect to potential complications, they can be characterized at the injection site (eg, bleeding, thrombosis, fistulation, nerve damage), intervention site (eg, rupture, dissection, improper target or trajectory), and postembolization (eg, secondary infection, post-embolization syndrome).<sup>3, 4, 5</sup></p> <p>The percutaneous approach is an alternative with localized injection of thrombin. This variant was first pioneered by Cope and Zeit for the treatment of iatrogenic femoral artery pseudoaneurysm and subsequently used in limited case studies for other instances of successful repair.<sup>6</sup> To our knowledge this successful visceral artery pseudoaneurysm repair with percutaneous thrombin injection is the first for ruptured right gastroepiploic artery pseudoaneurysm.</p> <h2>CONCLUSION</h2> <p>Visceral pseudoaneurysms are rare entities that can result in catastrophic events if not recognized early and treated appropriately. While the initial management remains debatable, interventional embolization has continued to be at the forefront of treatment management. It is important to recognize that in cases of failed endovascular approach due to vessel tortuosity, percutaneous embolization under ultrasound guidance can achieve successful results.</p> <h2>REFERENCES</h2> <ol> <li>Pasha SF, Gloviczki P, Stanson AW, Kamath PS. Splanchnic Artery Aneurysms. Mayo Clin <em>Proc.</em> 2007;82(4):472-479.</li> <li>Bergert H, Hinterseher I, Kersting S, Leonhardt J, Bloomenthal A, Saeger HD. Management and outcome of haemorrhage due to arterial pseudoaneurysms in pancreatitis. <em>Surg</em>. 2005;137:323&ndash;328.</li> <li>Carr SC, Pearce WH, Vogelzang RL, et al. Current management of visceral artery aneurysms. <em>Surg</em>. 1996; 120:627.</li> <li>Ferrero E, Viazzo A, Ferri M, et al.&nbsp;Management and urgent repair of ru<em>ptured visceral artery aneurysms. </em>Ann Vasc Surg. 2011;&nbsp;25: 981.e7-11.</li> <li>Coll DP, Ierardi R, Kerstein MD, Yost S, Wilson A, Matsumoto T. Aneurysms of the pancreaticoduodenal arteries: a change in management. <em>Ann Vasc Surg.</em> 1998; 12 (03) 286-291.</li> <li>Chadha M, Ahuja C. Visceral Artery Aneurysm: Diagnosis and Percutaneous Management. <em>Semin Intervent Radiology</em>. 2009; 26(3): 196-206.</li> </ol>10098
Infantile choriocarcinoma with cutaneous, liver, and lung metastases
2018-10-16T16:02:29-04:002018-10-16T16:02:29-04:00Logan Dance, MD; Apurvi Patel, MD; Mittun Patel, MD; Alexander J. Towbin, MD; and Richard Towbin MD <p><a href="/Communities/pediatric-imaging-community" target="_self"><img src="http://cdn.agilitycms.com/applied-radiology/PDFs/Pediatric/AR_featured-pediatric-case_4article.jpg" alt="" width="300" height="36" /></a></p> <h2>CASE SUMMARY</h2> <p>An otherwise healthy 37-day-old male presented to dermatology with a pedunculated, friable red glabellar mass (centered between the eyes). First noticed as a flat, bluish lesion at infant&rsquo;s 10 days of age, its subsequent rapid growth led to an emergency department visit, where dermatology diagnosed a hemangioma and initiated propranolol treatment. Despite treatment, the mass continued to grow rapidly, encroaching upon the patient&rsquo;s right eye. The patient was admitted for further workup.</p> <p>An elevated beta hCG, anemia (7.4 mg/dL), and thrombocytopenia (92,000) suggested an alternative diagnosis. MRI and ultrasound examinations led to a percutaneous biopsy; pathology was consistent with choriocarcinoma. PET/CT found FDG-avid glabellar, liver and lung lesions. Maternal and placental testing was negative for choriocarcinoma.</p> <p>Choricarcinoma is treated as a germ cell tumor. Therapy was initiated as per Children&rsquo;s Oncology Group protocol AGCT 01P1 (6 cycles of cisplatin, etoposide, and bleomycin). During cycle 1 he deteriorated requiring respiratory support and transfusions in the ICU. After cycle 4 he received a right lobectomy for the lung lesion and after cycle 5 the glabellar lesion was finally removed. PET scan after 6 cycles did not show any FDG activity in the glabellar lesion or the lungs, but mild persistent activity in the liver. It was determined that liver transplant would be his best option, due to the location of the mass.</p> <h2>IMAGING FINDINGS</h2> <p>Ultrasound demonstrated a hypoechoic, hypervascular mass (Figure 1). MRI brain scan demonstrated a cutaneous solid, avidly enhancing glabellar mass (Figure 2). CT scan showed a peripherally enhancing liver mass and a mass-like area of consolidation in the right lung (Figure 3). Initial PET/CT demonstrated FDG avid liver and lung metastases with a small focus of residual activity at the glabella consistent with incomplete resection (Figure 4).</p> <p>Follow-up PET/CT showed rapid re-growth of the glabellar mass and enlargement of the hepatic and pulmonary masses just 12 days later (Figure 5). Three-month follow-up PET/CT showed significantly decreased size and activity of the metastases consistent with a treatment response (Figure 6).</p> <h2>DIAGNOSIS</h2> <p>Infantile choriocarcinoma with cutaneous, liver, and lung metastases.</p> <h2>DISCUSSION</h2> <p>The unusual predilection of infantile malignancies for cutaneous metastases allows their early discovery and creates vivid clinical stigmata. While relatively common in infancy, such presentations are exceedingly rare in children and adults. The location of the cutaneous metastasis in our case hastened the diagnosis and life-saving treatment.</p> <p>In a series of 208 infants with cutaneous metastases, numerous bluish skin nodules created the so-called &ldquo;blueberry muffin&rdquo; baby appearance in 82% of cases. The differential diagnosis of lesions presenting with cutaneous involvement (ordered most to least common) include: leukemia, Langerhans cell histiocytosis, neuroblastoma, rhabdoid tumor, rhabdomyosarcoma, primitive neuroectodermal tumor, choriocarcinoma, and adrenocortical carcinoma.<sup>1</sup></p> <p>Choriocarcinoma is notorious for its life-threating hemorrhagic metastases and famous for being the first solid tumor to be cured with chemotherapy alone (methotrexate, 1956).<sup>2</sup> It arises from malignant placental B-hCG secreting trophoblastic cells and typically occurs in the setting of a molar pregnancy. Maternal metastases are rarely seen in normal pregnancy (about 1 in 50,000 live births).<sup>3</sup> Infantile metastases are extremely rare and may occur with or without maternal metastases.</p> <p>The misdiagnosis of infantile choriocarcinoma for infantile hemangioma has occurred as described in a 2014 case report by Brooks and Nolting<sup>4</sup> also with a solitary cutaneous metastasis to the face. The imaging findings of infantile choriocarcinoma include marked, early peripheral enhancement with central necrosis and hemorrhage. These attributes are nonspecific<sup>5</sup> and can mimic an involuting hemangioma or other solid tumors. PET/CT shows significant peripheral FDG avidity. Studies in maternal choriocarcinoma suggest PET/CT is useful in staging and follow-up.<sup>6</sup></p> <p>Considered one of the fastest growing tumors, infantile choriocarcinoma classically presents with hepatomegaly, anemia, failure to thrive, and precocious puberty between 0 days and 5 months of life. Left untreated, the disease is usually fatal within 3 weeks of presentation.<sup>7</sup> Chemotherapy and delayed surgical excision of metastases can be curative with long-term survival rates of around 80%.<sup>8</sup></p> <h2>CONCLUSION</h2> <p>A solitary cutaneous metastasis can be mistaken for infantile hemangioma both clinically and radiographically. Atypical MRI appearance is one important clue that can suggest an alternative diagnosis. Early diagnosis and treatment of infantile choriocarcinoma is critical for survival. PET/CT may be useful for staging and follow-up.</p> <h2>REFERENCES</h2> <ol> <li>Isaacs H Jr. Cutaneous metastases in neonates: A review. <em>Pediatr Dermatol. 2011; Mar-Apr;28(2):85-93. </em></li> <li>Yarris JP, Hunter AJ. Roy Hertz, MD (1909-2002): The cure of choriocarcinoma and its impact on the development of chemotherapy for cancer. <em>Gynecol Oncol. </em>2003; May 89(2):193-198.</li> <li>Tidy JA, Rustin GJ, Newlands ES, et al. Presentation and management of choriocarcinoma after nonmolar pregnancy. <em>Br J Obstet Gynaecol.</em> 1995; Sep 102(9):715-719.</li> <li>Brooks T, Nolting L. Cutaneous Manifestation of Metastatic Infantile Choriocarcinoma. <em>Case Rep Pediatr. </em>2014; 2014:104652. Epub 2014 Aug 12.</li> <li>van der Hoef M1, Niggli FK, Willi UV, Huisman TA. Solitary infantile choriocarcinoma of the liver: MRI findings. <em>Pediatr Radiol.</em> 2004 Oct;34(10):820-823. Epub 2004 Jul 28.</li> <li>Mapelli P, Mangili G, Picchio M, et al. Role of 18F-FDG PET in the management of gestational trophoblastic neoplasia. <em>Eur J Nucl Med Mol Imaging.</em> 2013; Apr;40(4):505-13.</li> <li>Yoon JM, Burns RC, Malogolowkin MH, Mascarenhas L. Treatment of infantile choriocarcinoma of the liver. Pediatr Blood Cancer. 2007 Jul;49(1):99-102.</li> <li>McNally OM, Tran M, Fortune D, Quinn MA. Successful treatment of mother and baby with metastatic choriocarcinoma.<em> Int J Gynecol Cancer. </em>2002 Jul-Aug;12(4):394-398.</li> </ol>10064
Dedifferentiated liposarcoma of the hypolarynx
2018-09-22T17:37:03-04:002018-09-22T17:37:03-04:00Krishna Kumar, BA; Sarah Kurian, MD; and Brian Chin, MD <h2>CASE SUMMARY</h2> <p>A 41-year-old male with 20-pack-year smoking history presented to the ED with 3 weeks of worsening shortness of breath, dysphagia, and coughing, and sudden onset of throat pain after eating and vomiting. On examination, he appeared in distress, with a muffled/hoarse voice and posterior pharyngeal erythema. Initially his symptoms were attributed to a food bolus, but he was unable to tolerate water or his secretions.</p> <p>Otolaryngology was consulted, and the patient was given dexamethasone and racemic epinephrine. A contrast-enhanced CT of the soft tissue of the neck revealed a well-defined, oblong soft tissue 1.7 &times; 2.7 &times; 3.9 cm mass arising from the right aryepiglottic region and narrowing the hypopharynx, (Figures 1-3). The patient underwent an awake tracheostomy placement and direct laryngoscopy with biopsy. Initial pathology was low-grade sarcoma or myxosarcoma. Immunohistochemical (IHC) stains on paraffin sections were reactive to vimentin, focally reactive to desmin, but negative for S100, indicating the mass was mesenchymal and connective tissue in origin, most consistent with a sarcoma.</p> <p>Given the rarity of sarcoma in this location, the patient underwent excisional biopsy of the mass.</p> <h2>IMAGING FINDINGS</h2> <p>Axial CT image (Figure 1) shows a low-attenuation soft tissue mass posterior to the epiglottis that significantly narrows the airway. Sagittal CT (Figure 2) image shows a low-attenuation mass anterior to the cervical vertebrae, causing significant narrowing of the airway. Figure 3 shows a high power (400x) view showing lipoblasts, pleomorphic lipoblasts, and mature adipocytes.</p> <h2>DIAGNOSIS</h2> <p>Dedifferentiated liposarcoma arising in the background of well-differentiated liposarcoma, with MDM2 gene amplification detected on fluorescent in-situ hybridization (FISH) testing.</p> <h2>DISCUSSION</h2> <p>Liposarcoma is a malignant neoplasm of fat cells, most commonly occurring in male adults 40-60 years of age. Even though most commonly found in the retroperitoneum,<sup>1</sup> up to 6% of liposarcomas occur in the head/neck region.<sup>2</sup> The most common head and neck sites are the cheek, anterior neck, larynx, posterior cervical region and pyriform sinus<sup>3</sup>. While lipomas primarily are found in the subcutaneous fat, liposarcomas usually arise in the deep soft tissues and involve intermuscular fascial planes.<sup>4</sup> The incidence of liposarcoma in the hypopharynx is not well-reported in the literature, with less than 40 cases reported, almost all of which are well-differentiated liposarcoma.<sup>5,6</sup></p> <p>As described above, liposarcomas are classified into intermediate graded tumors (well-differentiated) and malignant graded tumors (dedifferentiated, myxoid, pleomorphic, and not otherwise specified).<sup>7</sup> About 90 percent of dedifferentiated liposarcomas arise <em>de novo</em> and 10 percent occur as recurrence of a prior well-differentiated liposarcoma.<sup>8</sup></p> <p>Liposarcoma subtype is based on histologic and genetic findings, although computed tomography (CT) and magnetic resonance imaging (MRI) are important for characterizing and determining tumor extent. The size of the tumor is important to note, as masses greater than 5 centimeters upstage the tumor. Certain CT/MRI features may support a specific grade and/or subtype.</p> <p>Well-differentiated liposarcomas are often circumscribed and lobulated; dedifferentiated liposarcomas, infiltrating and amorphous. A dedifferentiated liposarcoma has signal characteristics similar to muscle and enhances avidly.<sup>9,10</sup> On MRI, the presence of juxtaposed focal mass-like nonlipomatous tissue with prolonged T1 and T2 relaxation times and a tissue attenuation similar to skeletal muscle on CT supports a dedifferentiated liposarcoma. Often in a well-differentiated liposarcoma, the nonlipomatous tissue appeared as thickened irregular septa, rather than a focal masslike area.<sup>10</sup></p> <p>Myxoid liposarcomas have an inhomogeneous appearance on CT and MRI, especially under contrast, and may even appear cystic on MRI. Myxoid and pleomorphic liposarcomas have a moderate to very inhomogenous appearance on CT and MRI, containing areas of necrosis, and enhancement is irregular. Fat is difficult to detect in these tumors, as it often accounts for &lt;25% of the volume.<sup>2</sup></p> <p>Surgical excision is the mainstay in treatment of liposarcoma, with the objective of wide resection with negative margins. In a study done by McCulloch et al,<sup>11</sup> there was an 80% recurrence after incomplete surgical resection of head and neck liposarcomas, but around 20% after complete surgical resection. However, given the amount of vital neurovascular structures located in this area, complete resection can often be difficult. Induction chemotherapy is often used in these areas in order to shrink the lesion prior to resection. Additionally, adjuvant intensity-modulated conformal radiotherapy (IMRT) or systemic therapy is increasingly used in areas where wide resection of the tumor margins cannot be performed. A study done by Eeles et al observed that adjuvant therapy decreased the rate of local recurrence of head and neck sarcomas.<sup>12</sup> Palbociclib, a potent CDK4/CDK6 inhibitor, has shown activity in well-differentiated and dedifferentiated liposarcoma by targeting these regulatory enzymes to halt disease progression. Another targeted therapy in dedifferentiated liposarcoma subtype is the significant presence of the MDM2 gene. MDM2 is a negative regulator of the p53 tumor suppressor gene and was found to be implicated in liposarcomas, as in this case report. RG7112 is an MDM2 antagonist that may be a future treatment option, but the drug is undergoing further study.<sup>13</sup></p> <p>Prognosis depends on many factors, including grade, subtype and extent at presentation. Well-differentiated and myxoid tumors rarely develop distant metastases, and five-year survival rates for these tumors are 83-100%.<sup>8</sup> Dedifferentiated and pleomorphic tumors are considered more malignant with greater potential for metastases. In a study done by Tirumani et al the lung was the most common site of metastases of dedifferentiated liposarcoma, with high tumor grade and local recurrence the greatest predictors of metastasis.<sup>14</sup> Dedifferentiated tumors have a five-year survival rate around 20 percent, and pleomorphic tumors around 56 percent.<sup>8</sup> Studies by Golledge et al and Gerry et al found that the overall prognosis for head and neck liposarcomas are better than other locations, especially when compared to the retroperitoneum. They reported a five-year survival rate at 67 percent for head and neck liposarcomas<sup>15</sup> and a higher disease-specific survival and overall survival rate.<sup>16</sup></p> <h2>CONCLUSION</h2> <p>&gt;As evidenced by the literature and our case, when presented with a soft-tissue mass in the head/neck, it is important to consider liposarcoma, although it is rare. Imaging with MRI and/or CT is important in localizing the mass and determining its size and potential involvement of adjacent neurovascular structures prior to resection. Subtle findings in these modalities can even help to differentiate between subtypes of liposarcoma; however, pathology is required for a final diagnosis. Surgery continues to be a mainstay of treatment. Newer adjuvant therapies are being investigated, which is important, as complete resection of cancer in this area can be difficult when trying to preserve satisfactory postoperative function.</p> <h2>REFERENCES</h2> <ol> <li>Gaillard F, and Di Muzio B. &ldquo;Liposarcoma | Radiology Reference Article.&rdquo; <em>Radiopaedia.org</em>. Radiopaedia, 2017. 10 Mar. 2017.</li> <li>Munk, PL, Lee MJ, Janzen DL, et al. &ldquo;Lipoma and Liposarcoma: Evaluation Using CT and MR Imaging.&rdquo; <em>American Journal of Roentgenology</em> 169.2 1997; 589-594.</li> <li>Yueh B, Bassewitz HL, and Eisele DW. &ldquo;Retropharyngeal Liposarcoma.&rdquo; <em>American Journal of Otolaryngology</em>. 1995; 16(5): 331-340.</li> <li>Nouri H, Hassani R, Aderdour L, and Raji A. &ldquo;The Well-differentiated Liposarcoma of the Hypopharynx.&rdquo; <em>European Annals of Otorhinolaryngology, Head and Neck Diseases</em>. 2011;128(3): 143-145.</li> <li>Zhu H, Sun J, Wei S, et al. &ldquo;Well-Differentiated Laryngeal/Hypopharyngeal Liposarcoma in the MDM2 Era Report of Three Cases and Literature Review.&rdquo; <em>Head and Neck Pathology</em> (2016): n. pag. <em>SpringerLink</em>.</li> <li>Riva G, Sensini M, Corvino, Garzaro AM, and Pecorari G. &ldquo;Liposarcoma of Hypopharynx and Esophagus: A Unique Entity?&rdquo; <em>Journal of Gastrointestinal Cancer</em> 47.2 (2016): 135-42.</li> <li>Doyle, Leona A. &ldquo;Sarcoma Classification: An Update Based on the 2013 World Health Organization Classification of Tumors of Soft Tissue and Bone.&rdquo; <em>Cancer</em> 120.12 (2014): 1763-774.</li> <li>Kikuchi, N., T. Nakashima, J. Fukushima, K. Nariyama, and S. Komune. &ldquo;Well-differentiated Liposarcoma Arising in the Parapharyngeal Space: A Case Report and Review of the Literature.&rdquo; <em>The Journal of Laryngology &amp; Otology</em> 129.S2, S86-S90.3 (2015).</li> <li>Vella, O., A. Bequignon, F. Comoz, and E. Babin. &ldquo;Retropharyngeal Liposarcoma: A Rare Cause of Dysphagia.&rdquo; <em>European Annals of Otorhinolaryngology, Head and Neck </em><em>Diseases</em> 133.6 (2016): 429-430.</li> <li>Kransdorf, M. J., J. M. Meis, and J. S. Jelinek. &ldquo;Dedifferentiated Liposarcoma of the Extremities: Imaging Findings in Four Patients.&rdquo; <em>American Journal of Roentgenology</em> 161.1 (1993): 127-130.</li> <li>Mcculloch, T. M., K. H. Makielski, and M. A. Mcnutt. &ldquo;Head and Neck Liposarcoma: A Histopathologic Reevaluation of Reported Cases.&rdquo; <em>Archives of Otolaryngology - Head </em><em>and Neck Surgery</em> 118.10 (1992): 1045-1049.</li> <li>Eeles, R. A. et al. &ldquo;Head and Neck Sarcomas: Prognostic Factors and Implications for Treatment.&rdquo; <em>British Journal of Cancer</em> 68.1 (1993): 201&ndash;207.</li> <li>Nassif NA, Tseng W, Borges C et al. Recent advances in the management of liposarcoma. F1000Research 2016, 5(F1000 Faculty Rev):2907.</li> <li>Tirumani, S.h., H. Tirumani, J.p. Jagannathan, A.b. Shinagare, J.l. Hornick, N.h. Ramaiya, and A.j. Wagner. &ldquo;Metastasis in Dedifferentiated Liposarcoma: Predictors and Outcome in 148 Patients.&rdquo; <em>European Journal of Surgical Oncology (EJSO)</em> 41.7 (2015): 899-904.</li> <li>Golledge, Jonathan, Cyril Fisher, and Peter H. Rhys-Evans. &ldquo;Head and Neck Liposarcoma.&rdquo; <em>Cancer</em> 76.6 (1995): 1051-1058.</li> <li>Gerry, Daniel, Nyssa F. Fox, Laura S. Spruill, and Eric J. Lentsch. &ldquo;Liposarcoma of the Head and Neck: Analysis of 318 Cases with Comparison to Non-head and Neck Sites.&rdquo; <em>Head &amp; Neck</em> 36.3 (2013): 393-400.</li> </ol>10062
Giant solitary fibrous tumor of the pleura
2018-09-22T17:32:12-04:002018-09-22T17:32:12-04:00Erica Rego and Brian A. Solomon, MD <h2>CASE SUMMARY</h2> <p>A 54-year-old man presented to the ER with chronic shortness of breath and wheezing. He had no significant medical or surgical history. The patient denied a history of tobacco use and asbestos exposure, and had no family history of lung cancer or pulmonary disease. On physical examination, he was afebrile with a normal cardiac exam. He had decreased breath sounds throughout the entire right hemithorax. All laboratory values were within normal limits.</p> <h2>IMAGING FINDINGS</h2> <p>Initial PA and lateral chest X-rays demonstrated apparent elevation of the right hemidiaphragm (Figure 1). A subsequent contrast-enhanced CT scan of the chest demonstrated a heterogeneous, lobulated, hypervascular mass in the right lower hemithorax measuring approximately 18 x 18 x 16 cm (CC, AP, transverse). There was no apparent chest wall or mediastinal invasion and no associated pleural effusion or mediastinal adenopathy was seen. Also, there was no evidence of necrosis or calcification. The mass had a significant systemic arterial supply, primarily from the right inferior phrenic artery. Mass effect resulted in shift of the heart and mediastium to the left (Figures 2). A follow up PET/CT scan demonstrated mild heterogeneous FDG activity within the mass with an SUV of 5.18. No FDG-avid mediastinal or hilar adenopathy was seen and there was no evidence of FDG avid metastasis. Also, there was no evidence of direct chest wall invasion (Figure 3).</p> <p>Subsequently, the patient underwent a CT-guided transthoracic biopsy followed by complete surgical resection.</p> <h2>DIAGNOSIS</h2> <p>Giant solitary fibrous tumor of the pleura</p> <h2>DISCUSSION</h2> <p>Solitary fibrous tumors of the pleura (SFTP) are rare, slow-growing neoplasms, initially described in 1870 by Wagner and further characterized by Klemperer and Rabin in 1931.<sup>1,2</sup></p> <p>The tumor is of unknown etiology and there is no known association with asbestos exposure.3 The incidence is approximately 2.8/100,000 with 800 reported cases to date.<sup>2</sup> The peak incidence is in the 5th to 8th decades, with no sex predilection. The giant type of fibrous pleural tumors are defined as occupying greater than 40% of the hemithorax and are especially rare, accounting for less than 5% of pleural neoplasms.<sup>4</sup></p> <p>These tumors are typically benign, slow growing lesions, with malignancy occurring in less than 20% of cases. Previously thought to arise from the mesenchymal of the submesothelial cells of the parietal pleura,<sup>5</sup> giant solitary fibrous tumors have now been reclassified by the World Health Organization Classification of Tumors. They are now considered part of a broader class of soft tissue neoplasms of fibroblastic and myofibroblastic origin and can arise from virtually any part of the body.<sup>6</sup> The lesions that arise from the pleura are often encapsulated within a thin membrane, which consists of collagen covered by normal mesothelial cells. The tumor is attached to the pleura by a highly vascular pedicle in 38-42% of cases. The base of the tumor is on the visceral pleura in about three quarters of the patients.<sup>5</sup> Malignant tumors are often necrotic with hemorrhage, and they may also result in pleural effusion and adjacent structural invasion.<sup>7</sup></p> <p>SFTP are often asymptomatic and tend to present secondary to mass effect on adjacent structures, resulting in nonspecific thoracic symptoms including chest pain, dyspnea, cough, and more rarely hemoptysis. Extrathoracic symptoms have also been reported; these include weakness, fever, weight loss, nocturnal sweating, chills, digital clubbing or hypertrophic osteoarthropathy.<sup>3</sup> Hypoglycemia has also been reported related to the production by the tumor of insulin-like growth factor II (IGF-II), which causes an increased glucose utilization and an impaired growth hormone counter-regulatory response to hypoglycemia.<sup>8,9 </sup></p> <p>Treatment is based on complete surgical resection, which is curative in nearly all benign lesions and approximately half of malignant ones. Pedunculated tumors can be safely resected with a wedge resection of the lung, but large sessile tumors often require lobectomy or pneumonectomy. Surgical resection, however, is often complicated by the extensive bronchial and systemic arterial supply. In cases involving systemic arterial supply to the tumor, catheter directed embolization may be helpful in reducing the blood supply and risk of perioperative hemorrhage.<sup>5 </sup>In many cases, the large tumor burden can also result in chronic compressive atelectasis and, therefore, post- operative expansion edema is a known complication.<sup>9</sup></p> <p>The CT appearance of an SFTP is often that of a well-circumscribed lobular homogenous mass often demonstrating marked heterogeneity and necrosis as well as atelectasis and compression/displacement of adjacent structures.<sup>4 </sup>Pleural effusions are relatively rare, occurring is less than 10% of patients.<sup>10</sup></p> <h2>CONCLUSION</h2> <p>Giant solitary fibrous tumors of the pleura are part of a broad class of fibroblastic lesions that can occur nearly anywhere in the body. These relatively rare tumors are often asymptomatic, eventually presenting with symptoms related to mass effect on adjacent structures or occasionally from paraneoplastic phenomena. The imaging features are typical of benign lesions: well circumscribed, without necrosis or local invasion and often have a significant systemic blood supply. This case also highlighted the PET scan findings of mild FDG activity. They are rarely malignant and complete surgical resection is usually curative.</p> <h2>REFERENCES</h2> <ol> <li>Wagner E. Das tuberkel&auml;hnliche Lymphadenom (der cytogene oder reticulirte Tuberkel). <em>Arch Heilk (Leipzig)</em> 1870; 11:497.</li> <li>Okike N, Bernatz PE, Woolner LB. Localized mesothelioma of the pleura: benign and malignant variants. <em>J Thorac Cardiovasc Surg</em> 1978; 75:363&ndash;372.</li> <li>Suter M et al, Localized Fibrous Tumours of the Pleura: 15 New Cases and Review of the Literature. <em>Eur J Cardiothorac Surg</em>. 1998;14(5), 453-459.</li> <li>Rosado-de-Christenson ML, Abbott GF., Page McAdams H, et al. Solitary fibrous tumors of the pleura. Radiographics. 2003; 23:759&ndash;783. doi: 10.1148/rg.233025165.</li> <li>England DM, Hochholzer L, McCarthy MJ. Solitary benign and malignant fibrous tumors of the pleura. <em>Am J Surg Pathol</em>. 1989; 13:640&ndash;658. doi: 10.1097/00000478-198908000-00003.</li> <li>Galateau-Salle F, Churg A, Roggli V, Travis WD; World Health Organization Committee for Tumors of the Pleura The 2015 World Health Organization Classification of Tumors of the Pleura: Advances since the 2004 Classification. <em>J Thorac Oncol</em>. 2016 Feb;11(2):142-54. doi: 10.1016/j.jtho.2015.11.005.</li> <li>de Perrot M, Fischer S, Br&uuml;ndler MA, Sekine Y, Keshavjee S. Solitary fibrous tumors of the pleura. <em>Ann Thorac Surg</em> 2002; 74:285&ndash;293.</li> <li>Masson EA, Mac Farlane IA, Graham D, Foy P. Spontaneous hypo-glycemia due to a pleural fibroma: role of insulin-like growth factors. <em>Thorax.</em> 1991; 46:930&ndash;931.</li> <li>Juntang Guo, Xiangyang Chu, Yu-e Sun, et al. <em>World J Surg</em>. 2010 Nov; 34(11): 2553&ndash;2557.Published online 2010 Jul 14. doi:&nbsp; 10.1007/s00268-010-0715-x).</li> <li>Robinson LA, Reilly RB. Localized pleural mesothelioma. The clinical spectrum. <em>Chest</em>. 1994;106:1611&ndash;1615.</li> </ol>10060
2018-09-22T17:25:40-04:002018-09-22T17:25:40-04:00Tarik Nurkic, MD; Sarah Castillo-Jorge, MD; and Ilona Schmalfuss, MD <h2>CASE SUMMARY</h2> <p>A 67-year-old African-American male presented to our emergency department with facial drooling and concern for a possible cerebrovascular event. On further inquiry, the patient revealed sensation of a mass for over 20 years without attributable symptoms. Physical examination demonstrated submucosal fullness of the left retromolar triangle with intact mucosa. No associated facial weakness, soft-tissue swelling or adenopathy was reported. Routine laboratory values were unremarkable.</p> <h2>IMAGING FINDINGS</h2> <p>A non-contrasted head CT (Figure 1) performed in the ED to evaluate for stroke incidentally noted a complex mass in the left masticator space with focal areas of low attenuation suggestive of fluid. There was regressive remodeling of the adjacent posterior maxillary sinus wall (Figure 2) consistent with a longstanding process rather than a destructive malignant lesion.</p> <p>MR of the face without and with intravenous Gadolinium was performed for further evaluation and revealed a corresponding dumbbell shaped, complex mass containing lobulations. The mass was primarily isointense to muscle on T1-weighted images (Figure 3) with heterogeneous enhancement following gadolinium administration (Figure 4). Focal areas of hyperintensity were seen on T2 weighted images consistent with internal cysts (Figure 5). There was preservation of the cortex of the maxillary sinus and maxillary alveolus (Figures 2, 3 and 5) as well as preserved integrity of the gingival mucosa which was best demonstrated on coronal T2 images (Figure 5) and confirmed on clinical examination. Marked hyperintensity was present on DWI with an average apparent diffusion coefficient of 0.9 &times; 10<sup>-3</sup> mm<sup>2</sup>/s (Figure 6).</p> <h2>DIAGNOSIS</h2> <p>Extra-gingival peripheral ameloblastoma. Differential diagnoses: neurofibroma or schwannoma of the buccal branch of the left trigeminal nerve, keratocystic odontogenic tumor or solitary fibrous tumor.</p> <h2>DISCUSSION</h2> <p>Peripheral ameloblastomas (PA) are exceedingly rare tumors, constituting about 1% of all ameloblastomas. To our knowledge, six cases of extra-gingival ameloblastomas have been reported in the literature. Clinical presentations of ameloblastomas vary with location. The most common symptom is painless tumefaction, as seen in our patient. They can be divided into the following subtypes:</p> <p>Intraosseous solid or multi-cystic, the most common type, representing 86% of maxillary ameloblastomas.</p> <p>Intraosseous unicystic, which account for 13% of maxillary ameloblastomas.</p> <p>Extraosseous or peripheral, which constitute about 5% of all ameloblastomas. PA occurs at a significant higher age than the intraosseous ameloblastoma, with a reported maximum incidence between the fifth and sixth decades of life.<sup>1 </sup></p> <p>Malignant ameloblastomas, which are very rare and manifest with metastases, most commonly to the lungs, regional lymph nodes, pleura, vertebrae, skull, diaphragm, liver, parotid glands, and small intestine.</p> <p>Peripheral ameloblastomas are typically found in the retromolar aspect of the mandible. Other less common regions include the posterior tuberosity of the maxilla, the buccal mucosa, or the floor of the mouth. The majority of ameloblastomas derive from the odontogenic apparatus (ie, epithelium, enamel organ and its derivatives or remnants), or less commonly from the lining of a dentigerous cyst.<sup>2</sup> These tumors are thought to arise from either extraosseous remnants of dental lamina or the basal cell layer of surface epithelium, which is believed to have odontogenic potential.<sup>1,3</sup> Additional theories implicate stratified squamous epithelium or pluripotent cells of minor salivary glands as their origin.<sup>4 </sup>It is interesting that the reported cases of extra-gingival ameloblastoma all developed around the orifices of either the Stensen&rsquo;s or Wharton&rsquo;s ducts and could thus represent tumors of salivary gland origin.<sup>4</sup></p> <p>Peripheral ameloblastoma is rarely the initial preoperative diagnosis derived from clinical examination or imaging. The typical radiographic appearance of an ameloblastoma is that of a multi-loculated mass with well-defined cystic spaces of varying size with signs of local invasion. However, perineural tumor spread has not been reported with PA.<sup>5 </sup>The imaging appearance of these tumors is not pathognomonic but it can be characteristic with a &ldquo;soap bubble&rdquo; or &ldquo;honeycomb&rdquo; appearance with often associated resorption of the adjacent tooth root best seen on CT. It is sometimes indistinguishable from a dentigerous cyst.<sup>6 </sup>Often, avid contrast enhancement is seen in the solid portions of the tumor with marked hyperintensity on T2-weighted sequences related to the cystic nature of the lesions. Calcifications are unusual.<sup>2</sup> CT and MR are crucial in their contribution to preoperative planning by delineating the anatomic extent. Treatment of PA consists of complete surgical excision. Amelobastomas are slow-growing with a tendency to spread into marrow spaces with pseudopods without concomitant resorption of trabecular bone. As a result, their margins are not clearly evident radiographically or grossly during surgery.<sup>6 </sup>Therefore, they have a high propensity to recur.</p> <p>The histological diagnostic criteria of PAs include origin from the overlying epithelium, presence of odontogenic epithelium islands in the lesion, and lack of potential to bone infiltration.<sup>3</sup> Histopathologic subtypes include plexiform, follicular, unicystic, basal cell, granular cell, clear cell, acanthomatous, vascular, and desmoplastic patterns. An interesting characteristic of the histology of a PA is that it shares many of the features of basal cell carcinoma. Both are characterized by a proliferation of basal cells, often arranged in nests and intermixed with a fibrous stroma. However, PA can be differentiated by nuclei located in the upper part of the cytoplasm. In addition, immunohistochemical analysis can be used to distinguish between the two.<sup>1 </sup>PA is positive for CK19 and negative for Ber-EP4, whereas the opposite is true for BCC. In a study done by Kato et al, all cases were negative for Ber-EP4, suggesting that both peripheral and intraosseous ameloblastomas are tumors with a common origin, and that PA is derived from odontogenic epithelial remnants, rather than from basal cells of the oral epithelium.<sup>7</sup></p> <p>In regard to differential diagnostic considerations, neurofibroma is a reasonable choice; however, the MR images do not demonstrate the expected target sign commonly seen with these tumors. Schwannoma was another possibility, as these tend to show cystic degeneration, in particular when larger in size. Schwannomas, however, typically reveal free diffusion in the center of the lesion with a ring of peripheral restriction, which was not the case in the presented patient.<sup>8,9</sup> Keratocystic odontogenic tumors could also be included in the differential diagnosis of partially cystic masses in this region demonstrating similar T2 characteristics. However, these lesions frequently contain perakeratin or orthokeratin contents and characteristically experience focal areas of hyperintensity on T1 weighted images.<sup>10 </sup>Additionally, ameloblastomas tend to be more cellular and generally demonstrate a greater degree of diffusion restriction than keratocystic odontogenic tumors. Solitary fibrous tumor can also occur in the masticator space but these usually demonstrate a T2-hyperintense core with free diffusion.<sup>8,9</sup></p> <h2>CONCLUSION</h2> <p>Peripheral ameloblastomas are exceedingly rare tumors, making up about 1% of all ameloblastomas. The imaging appearance of these tumors is not pathognomonic but often displays a characteristic &ldquo;soap bubble&rdquo; or &ldquo;honeycomb&rdquo; appearance with resorption of the adjacent tooth root, particularly when seen in the retromolar aspect of the mandible.</p> <h2>REFERENCES</h2> <ol> <li>Vanoven BJ, Petruzelli GJ. Peripheral ameloblastoma of the maxilla: a case report and literature review. American Journal of Otolaryngology. 2008;29:357-360.</li> <li>Weissman JL, Snyderman CH, Yousem SA, Curtin HD. Ameloblastoma of the maxilla: CT and MR appearance. American Journal of Neuroradiology. 1993;14:223-226.</li> <li>Martelli-Junior HD, Souza LN, Nogueira SLA, Melo-Filho MR, De Paula, AMB. Peripheral ameloblastoma. Oral Surgery Oral Medicine Oral Pathology Oral Radiology and Endodontology. 2005;99:E31-3.</li> <li>Philipsen HP, et al. Peripheral ameloblastoma: biological profile based on 160 cases from the literature. <em>Oral Oncol</em>. 2001; 37:17-27.</li> <li>Garcia RMIM, Blanco, RJA; Garcia CM; Verrier HA. Ameloblastomas maxilares: a proposito de dos casos clinicos. <em>Acta Otorrinolaringolo Esp. </em>2001;52:261-265.</li> <li>Iordanidis SM, Dimitrakopoulos J, Kariki H. Ameloblastoma of the maxilla. Case report. <em>Australian Dental Journal</em>. 1999;44:51-55.</li> <li>Surya V et al. A case of peripheral ameloblastoma of retromolar trigone: Histopathological and immunohistochemical profile.<em> Contemporary Clinical Dentistry</em>. 2015;6(4):564-566.</li> <li>Das A. Benign neck masses showing restricted diffusion: Is there a histological basis for discordant behavior? <em>World J Radiol</em>. 2016;8(2):174-182.</li> <li>Das A. Diffusion-weighted imaging in extracranial head and neck schwannomas: A distinctive appearance. <em>Indian J Radiol Imaging, </em>26(2): 231-236.</li> <li>Mosier KM. Magnetic resonance imaging of the maxilla and mandible: signal characteristics and features in the differential diagnosis of common lesions. <em>Top Magn Reson Imag</em><em></em><em>ing. </em>2015;(24)1: 23-37.</li> </ol>10058
2018-09-22T17:19:19-04:002018-09-22T17:19:19-04:00Mark Reddick, MD; Prakash Gajera, MD; Patrick Sutphin, MD, PhD; Harold Park, MD; and Sanjeeva P. Kalva, MD  CASE SUMMARY   A 58-year-old man presented with altered mental status. He had hepatitis C and alcohol-related cirrhosis with a Model for End Stage Liver Disease score of 17, Child-Pugh score of A6, and Westhaven grade 2 encephalopahy with hyperammonemia (240 mcmol/L) despite standard medical therapy.   IMAGING FINDINGS   A CT scan of the abdomen demonstrated cirrhosis, chronic portal vein thrombosis, and a porto-systemic (PS) shunt from the IMV to right IIV measuring up to 18 mm (Figure 1A). A mesenteric arteriogram to evaluate flow dynamics demonstrated chronic PVT with flow primarily through the PS shunt (Figure 1B). Conventional TIPS was unsuccessful due to chronic PVT.   DIAGNOSIS   Hepatic encephalopathy   DISCUSSION   The splenic vein was accessed via transsplenic US-guided needle puncture and a 5Fr-10cm vascular sheath was placed. The right portal vein was then accessed with a hydrophilic 0.035” wire and 5Fr angiographic catheter. This was exchanged for a 5mm balloon catheter, which was insufflated with air so as to be visible on intravascular ultrasound (IVUS) that was positioned in the IVC from a right femoral vein approach. A Colapinto needle was advanced from the middle hepatic vein via transjugular access to puncture the echogenic balloon under IVUS guidance. A guidewire was advanced through the Colapinto needle into the main portal vein and the balloon was removed through the splenic access. A 10mm × 80mm × 20mm Viatorr stent graft was placed and dilated to 8mm. Post-TIPS portal venography demonstrated persistent filling of the IMV-IIV shunt (Figure 1C). The shunt was occluded with an 18mm vascular plug. Post-embolization portal venography demonstrated a patent TIPS and occlusion of the IMV-IIV PS shunt (Figure 1D). The splenic access site was occluded with a 5 mm vascular plug.   One day postprocedure, the patient’s serum ammonia level decreased to 86mcmol/L. At discharge one week later, he was without encephalopathy.   CONCLUSION   One etiology for refractory hepatic encephalopathy (HE) is the presence of a portosystemic (PS) shunt. Laleman et al demonstrated safety and efficacy of PS shunt embolization for treatment of refractory HE but excluded patients with portal vein thrombosis (PVT). 1  Intagliata et al demonstrated successful PS shunt occlusion for treatment of HE in a patient with PVT after anticoagulation and de novo recanalization. 2  Kallini et al demonstrated the technique of trans-splenic portal vein recanalization and TIPS creation in the setting of noncirrhotic chronic portal vein thrombosis. 3  This case demonstrates that refractory HE from a large PS shunt can be treated in the setting of chronic portal vein thrombosis using a trans-splenic approach to facilitate TIPS creation followed by shunt occlusion and can result in at least early improvement of encephalopathy.   REFERENCES     Laleman W, Simon-Talero M, Maleux G, et al. Embolization of large spontaneous portosystemic shunts for refractory hepatic encephalopathy: a multicenter survey on safety and efficacy.  Hepatology.  2013; 57:2448-2457.   Intagliata NM, Saad WE, Caldwell SH. Effects of restoring portal flow with anticoagulation and partial splenorenal shunt embolization.  Hepatology.  2015; 61:1088-1090.   Kallini JR, Gabr A, Kulik L. Noncirrhotic complete obliterative portal vein thrombosis: Novel management using trans-splenic transjugular intrahepatic portosystemic shunt with portal vein recanalization.  Hepatology.  2016; 63:1387-1390.   10056
Chronic intestinal perforation by a wooden toothpick, complicated by abscess formation
2018-09-22T17:13:49-04:002018-09-22T17:13:49-04:00Julia Gosis, MD; Michael Wright, MD; and Christopher Kuzminski, MD <h2>CASE SUMMARY</h2> <p>A 40-year-old Hispanic male presented with a 2-day history of sharp right lower quadrant pain and subjective fevers. On physical exam, he was afebrile with normal vital signs. He was moderately tender in the right lower quadrant without guarding or rebound. Labs were notable for a leukocytosis of 17.7.</p> <h2>IMAGING FINDINGS</h2> <p>The patient had a 6-month history of similar episodes of intermittent pain, with one requiring admission. A contrast-enhanced CT scan at that time showed inflammation in the right lower quadrant centered about a loop of thick-walled distal ileum (Figure 1A). The appendix was notably normal (not shown). Findings were thought most consistent with an infectious enteritis, with the differential including inflammatory bowel disease and Meckel&rsquo;s diverticulitis. He improved with intravenous antibiotics, but re-presented to the emergency department 1 month later with similar symptoms, with CT showing persistent but improved inflammatory changes in the right lower quadrant (Figure 1B).</p> <p>Now presenting a third time with recurrent symptoms, he again underwent imaging with intravenous contrast-enhanced CT. This demonstrated significant worsening of inflammation in the right lower quadrant, now with a 3.0 x 2.3 cm rim-enhancing fluid collection consistent with an abscess (Figures 1C and 2). There was no free air. Within the inflamed loop of distal ileum that had been seen on the two prior exams, there was a thin hyperdense linear structure impacted in the wall, with one end piercing the wall and sitting free within the peritoneal cavity (Figure 1C). Retrospectively, this linear structure was present on the two prior exams but obscured by adjacent enhancing bowel wall and inflammation (Figures 1A and 1B). This now had penetrated further through the wall and into the peritoneal cavity, thereby making itself for more apparent. Findings were most consistent with chronic foreign body perforation, now complicated by a small abscess.</p> <p>The patient was taken for diagnostic laparoscopy with intraoperative findings concordant with those seen on CT. The inflamed segment of small bowel was approximately 15 cm away from the terminal ileum and was resected. Within this segment of bowel was a wooden toothpick (Figure 3). The patient had an uneventful postoperative course.</p> <h2>DIAGNOSIS</h2> <p>Chronic intestinal perforation by a wooden toothpick, complicated by abscess formation</p> <h2>DISCUSSION</h2> <p>While foreign body ingestion is common, the vast majority of foreign bodies pass through the GI tract without complication. It is estimated that less than 1% of cases result in foreign body perforation, most commonly seen with sharp or elongated dietary objects, such as fish bones, chicken bones, and toothpicks. Ingested foreign bodies have a propensity to lodge in regions of anatomic angulation, including the duodenal C- loop, the ligament of Treitz, the ileocecal and rectosigmoid junctions, or in acquired strictures. Risk factors for foreign body ingestion include rapid eating, mental disorders, use of dentures and alcohol and drug consumption.<sup>1-3</sup></p> <p>Clinical presentation is quite variable, and can be acute, subacute, chronic, or even asymptomatic.<sup>2,4</sup> Generally, patients with gastric or duodenal perforation present with acute severe pain due to a rapid chemical peritonitis secondary to spillage of erosive intraluminal contents. Similar to our patient, those with a distal ileum or colonic perforation tend to have a slower and more indolent course due to the relatively non-erosive chemical environment within the lower GI tract.<sup>4 </sup>Complications include GI bleeding, abscess formation, fistulas, and migration into adjacent organs.<sup>2,5</sup> Perforation can induce a fibrous reaction and adhesions with subsequent small bowel obstruction.<sup>5</sup></p> <p>While early diagnosis and treatment are imperative for reducing morbidity and mortality associated with intestinal perforation secondary to dietary foreign body ingestion, diagnosis is quite difficult. Most ingestions are accidental and not recalled. There may also be a significant time lag between the ingestion and onset of symptoms.<sup>1,5</sup> Furthermore, the sensitivity of CT for detection of the ingested foreign body is low, with a detection rate of 41.6% for toothpicks and another 71.4% for fish bones.<sup>1,5</sup> Contrast-enhanced scans can make detection more difficult due to the presence of adjacent small contrast-enhanced mesenteric vessels, normally enhancing bowel wall, and high density intraluminal oral contrast. Repeat exam with noncontrast CT may be warranted if there is question of a foreign body on the initial contrast enhanced study.<sup>1,2,5</sup> CT findings include localized inflammatory change with possible abscess formation and a segment of bowel wall thickening. The foreign body may be visualized impacted within the bowel wall and/or extramural.<sup>1,2,5</sup> As in our case, free air is often absent because the gradual nature of the perforation allows the site to seal and become covered by fibrin, omentum, or adjacent bowel, thus preventing an air leak.<sup>1,3</sup></p> <p>A wooden foreign body such as a toothpick will appear as a linear, high-density structure on CT as long as it has resided in the GI tract long enough to absorb water. This appearance is indistinguishable from a fish bone. In the absence of water, however, a wooden foreign body will appear low density due to the presence of air. This occurs when imaging is done shortly after the time of ingestion, generally within 24 hours.<sup>1,5</sup></p> <h2>CONCLUSION</h2> <p>We presented a case of a distal small bowel perforation cause by accidental ingestion of a toothpick. As discussed in the literature, accurate diagnosis was challenging due to the patient&rsquo;s inability to recall the ingestion and the inconspicuity of the thin linear hyperdense toothpick against the surrounding inflammatory change and contrast-enhancing bowel wall. Delayed diagnosis ultimately lead to abscess formation, a common complication. We emphasize the importance of maintaining a high index of suspicion for foreign body perforation in the presence of nonspecific signs of bowel inflammation and localized peritonitis.</p> <h2>REFERENCES</h2> <ol> <li>Goh BK, Tan YM, Lin SE, Chow PK, Cheah FK, Ooi LL, Wong WK. CT in the preoperative diagnosis of fish bone perforation of the gastrointestinal tract. <em>AJR Am J Roentgenol.</em> 2006;187:710&ndash;714.</li> <li>Kuzmich S, Burke CJ, Harvey CJ, et al. Perforation of gastrointestinal tract by poorly conspicuous ingested foreign bodies: radiological diagnosis. <em>Br J Radiol</em>. 2015;88:20150086. doi:&nbsp;10.1259/bjr.20150086</li> <li>Nassani N, El-Charabaty E, Nasr P,et al.. Foreign body penetration through jejunal loops causing renal artery thrombosis and renal infarct. <em>ACG Case Reports Journal.</em> 2017;4. doi:10.14309/crj.2017.12.</li> <li>Kuo CC, Jen TK, Wen CH, et al. Medical treatment for a fish bone-induced ileal micro-perforation: A case report. <em>World J Gastroenterol.</em> 2012; 18: 5994&ndash;5998.</li> <li>Izumi J, Satoh K, Iwasaki W, et al. Small bowel obstruction caused by the ingestion of a wooden toothpick: The CT findings and a literature review. <em>Intern Med</em>. 2017; 56: 657&ndash;660.</li> </ol>10054
2018-09-22T17:02:45-04:002018-09-22T17:02:45-04:00Robbin Z. Thomas, MD, and Ishani B. Dalal, MD <h2>CASE SUMMARY</h2> <p>A 52-year-old woman presented with a 1-year complaint of unexplained/undesired weight loss of 13 lbs. Additional symptoms derived on review of systems included chills, night sweats, fatigue, and pain at the base of her tongue. Standard laboratory values demonstrated slight anemia and increase in serum creatinine. Subsequent biopsy of the base of tongue spot demonstrated amyloid deposition. Urine protein electrophoresis demonstrated elevated free lambda light chain, and bone-marrow biopsy demonstrated elevated levels of clonal plasma cells, consistent with diagnosis of multiple myeloma with associated amyloidosis.</p> <p>The patient then underwent high-dose chemotherapy followed by allogenic stem cell transplantation with stabilization of the disease process. On follow-up examinations after initial therapy, the patient complained of new areas of nodularity involving the tongue, eyelids, and lower abdomen. The left lower abdomen was biopsied and pathologic results demonstrated myeloma/plasmacytoma. Additional involvement of the heart and GI tract was noted during the patient&rsquo;s workup.</p> <h2>IMAGING FINDINGS</h2> <p>PET/CT images (Figures 1-3) demonstrated numerous hazy and nodular opacities within the subcutaneous tissues of the body wall involving the proximal upper extremities, anterior chest/breasts, anterior abdominal wall, and circumferential around the pelvis. The majority of these areas registered below background metabolism (compared to the average liver SUV), with some mildly hypermetabolic, most notably in the right inguinal region. The area of biopsy-proven myeloma/plasmacytoma was obtained from the left lower abdomen.</p> <h2>DIAGNOSIS</h2> <p>Estraosseous multiple myeloma. Differential considerations included a second primary malignancy, infectious process, extramedullary hematopoiesis, and in a different clinical scenario, the possibility of plasmacytoma should be considered.</p> <h2>DISCUSSION</h2> <p>Multiple myeloma may present with nonspecific clinical findings, including bone pain, fatigue, malaise, anemia, renal dysfunction, and increased susceptibility to infection. Advanced age (average age at diagnosis is 60 years) and abnormalities on standard labs may raise suspicion for this disease process.<sup>1</sup> A diagnosis of symptomatic multiple myeloma requires clonal plasma cells &gt;10% on biopsy (usually bone marrow), serum/urine monoclonal protein, and evidence of related end-organ damage.<sup>2</sup> Differentiation from plasmacytoma (localized clonal plasma cell proliferation without bone marrow involvement) is important, as there is vast difference in prognosis and treatment.</p> <p>Skeletal surveys are often performed as part of the standard workup; they can also be used to monitor disease progression and response to therapy. Findings can include lytic lesions, pathologic fractures, and osteopenia.<sup>3</sup> Although not standard practice, additional imaging can include PET/CT or whole-body MRI, both of which are excellent in identifying contiguous spread of disease from osseous structures as well as extraosseous disease.<sup>4, 5</sup> Contiguous spread of disease from osseous structures is more common than non-contiguous extraosseous spread, but this may be due to under-diagnosis, as extra-osseous involvement of myeloma was present in &gt;63% in a post-mortem series.<sup>1,6</sup></p> <p>Examples of extraosseous spread of disease include involvement of the liver, spleen, lymph nodes, skin, lungs, and central nervous system.<sup>1</sup> Determining the presence of extraosseous spread of disease is significant due to its effect on patient prognosis, with shorter progression-free survival and overall survival.<sup>7</sup> The presence of extraosseous multiple myeloma has also been noted to be more common in more aggressive variants of the disease, which typically present at a mean age of 50.<sup>3, 1</sup></p> <p>Common imaging findings for extraosseous myeloma include homogenous soft tissue lesion with corresponding T2 hypointensity due to high cellularity. These lesions do not tend to calcify or demonstrate necrotic change, and will demonstrate mild hypermetabolism on FDG PET.<sup>3</sup></p> <h2>CONCLUSION</h2> <p>Extraosseous involvement of multiple myeloma has increased in the last several decades, possibly due in part to improved imaging detection and increased patient survival. These increased rates are more pronounced in patients that undergo stem-cell transplant, likely as sanctuary sites.<sup>7</sup> Although imaging characteristics for extraosseous myeloma are nonspecific, the presence of extraosseous soft-tissue masses in patients with a known history of multiple myeloma should raise suspicion for extraosseous disease. Tissue biopsy can help to confirm suspicions in equivocal cases. Diagnosing extraosseous metastases is of prognostic importance as their presence correlates with shorter progression-free survival and overall survival.<sup>7</sup></p> <h2>REFERENCES</h2> <ol> <li>Moulopoulos LA, Granfield CA, Dimopoulos MA, et al. Extraosseous multiple myeloma: imaging features. <em>AJR Am J Roentgenol. </em>1993;161 (5): 1083-1087.</li> <li>Kyle RA, Rajkumar SV. Criteria for diagnosis, staging, risk stratification and response assessment of multiple myeloma. <em>Leukemia</em>. 2009;23 (1): 3&ndash;9.</li> <li>Hall MN, Jagannathan JP, Ramaiya NH et-al. Imaging of extraosseous myeloma: CT, PET/CT, and MRI features<em>. AJR Am J Roentgenol. </em>2010;195 (5): 1057-1065.</li> <li>Durie BGM. The role of anatomic and functional staging in myeloma: description of Durie/Salmon plus staging system. <em>Eur J Cancer</em>. 2006; 42:1539&ndash; 1543.</li> <li>Dimopoulos M, Terpos E, Comenzo RL, et al. International Myeloma Working Group. International Myeloma Working Group consensus statement and guidelines regarding the current role of imaging techniques in the diagnosis and monitoring of multiple myeloma<em>. Leukemia. </em>2009; 23:1545&ndash;1556.</li> <li>Oshima K, Kanda Y, Nannya Y, et al. Clinical and pathologic findings in 52 consecutively autopsied cases with multiple myeloma. <em>Am J Hematol.</em> 2001; 67:1&ndash;5.</li> <li>Varettoni M, Corso A, Pica G, et al. Incidence, presenting features and outcome of extraosseous disease in multiple myeloma: a longitudinal study on 1003 consecutive patients. <em>Ann Oncol.</em> 2010; 21:325&ndash;330.</li> </ol>