Tinnitus

Tinnitus. MRI Head and Internal Auditory Canal Without and With IV Contrast MRI head and IAC without and with IV contrast can be considered for retrocochlear processes such as vestibular schwannoma or other mass lesions in patients with NPT [71,77]. Tinnitus may occur among patients with vestibular schwannomas (63%-75%) and intralabyrinthine schwannomas [78]. These are readily diagnosed by MRI. Rarely, meningiomas in or around the IAC and cerebellopontine angle cistern can also cause tinnitus and are accurately diagnosed on MRI [79,80]. Vestibular schwannomas cause NPT more commonly than PT [49]. Mass lesions in the IAC and posterior fossa such as schwannomas, meningiomas, and endolymphatic sac tumors are readily diagnosed by MRI. A study of 218 patients with NPT found that 91.8% of patients had an unremarkable MRI [81].


Tinnitus

Tinnitus. Spontaneous intracranial hypotension can cause tinnitus, probably due to venous engorgement in the IAC [82,83]. Endolymphatic hydrops has an association with Meniere disease, which presents with episodic hearing loss, vertigo, and tinnitus [84-87] and requires special sequences for diagnosis, including heavily T2-weighted 3-D FLAIR (fluid- attenuated inversion recovery) and 3-D real inversion recovery performed 4 hours after injection of the IV contrast [88], best interpreted alongside a routine 3-D heavily T2-weighted sequence.


Tinnitus

Tinnitus. MRV Head With IV Contrast There is no relevant literature to support the use of MRV head with IV contrast for evaluation of unilateral NPT.


Tinnitus

Tinnitus. MRV Head Without and With IV Contrast There is no relevant literature to support the use of MRV head without and with IV contrast for evaluation of unilateral NPT.


Tinnitus

Tinnitus. MRV Head Without IV Contrast There is no relevant literature to support the use of MRV head without IV contrast for evaluation of unilateral NPT.


Tinnitus

Tinnitus. Tinnitus US Duplex Doppler Carotid Artery There is no relevant literature to support the use of US duplex Doppler carotid artery for evaluation of unilateral NPT.


Tinnitus

Tinnitus. US Duplex Doppler Transcranial There is no relevant literature to support the use of US duplex Doppler transcranial for evaluation of unilateral NPT.


Tinnitus

Tinnitus. US Head There is no relevant literature to support the use of US head for evaluation of unilateral NPT.


Tinnitus

Tinnitus. Variant 4: Nonpulsatile tinnitus, bilateral; no hearing loss, and no neurologic deficit, and no trauma. Initial imaging. Imaging is not helpful in cases of bilateral NPT related to age-related hearing loss, prior exposure to noise, acoustic trauma, ototoxic medications, and chronic bilateral hearing loss [92]. Most cases of bilateral NPT are associated with sensorineural hearing loss, and imaging is typically unrevealing in such cases [34,93,94]. The AAO-HNS guidelines make a strong recommendation against any imaging studies of the head and neck for the subset of patients in whom tinnitus does not localize to 1 ear, is nonpulsatile, and is not associated with focal neurological abnormalities or an asymmetric hearing loss [6,7].


Tinnitus

Tinnitus. Arteriography Cervicocerebral There is no relevant literature to support the use of conventional arteriography for evaluation of bilateral NPT.


Tinnitus

Tinnitus. CT Head With IV Contrast There is no relevant literature to support the use of CT head with IV contrast for evaluation of bilateral NPT.


Tinnitus

Tinnitus. CT Head Without and With IV Contrast There is no relevant literature to support the use of CT head without and with IV contrast for evaluation of bilateral NPT.


Tinnitus

Tinnitus. CT Head Without IV Contrast There is no relevant literature to support the use of CT head without IV contrast for evaluation of bilateral NPT.


Tinnitus

Tinnitus. CT Temporal Bone With IV Contrast There is no relevant literature to support the use of CT temporal bone with IV contrast for evaluation of bilateral NPT.


Tinnitus

Tinnitus. CT Temporal Bone Without and With IV Contrast There is no relevant literature to support the use of CT temporal bone without and with IV contrast for evaluation of bilateral NPT.


Tinnitus

Tinnitus. CT Temporal Bone Without IV Contrast There is no relevant literature to support the use of CT temporal bone without IV contrast for evaluation of bilateral NPT.


Tinnitus

Tinnitus. CTA Head and Neck With IV Contrast There is no relevant literature to support the use of CTA head and neck with IV contrast for evaluation of bilateral NPT.


Tinnitus

Tinnitus. CTA Head With IV Contrast There is no relevant literature to support the use of CTA head with IV contrast for evaluation of bilateral NPT.


Tinnitus

Tinnitus. CTV Head With IV Contrast There is no relevant literature to support the use of CTV head with iv contrast for evaluation of bilateral NPT.


Tinnitus

Tinnitus. MRA Head With IV Contrast There is no relevant literature to support the use of MRA head with IV contrast for evaluation of bilateral NPT.


Tinnitus

Tinnitus. Tinnitus MRA Head Without and With IV Contrast There is no relevant literature to support the use of MRA head without and with IV contrast for evaluation of bilateral NPT.


Tinnitus

Tinnitus. MRA Head Without IV Contrast There is no relevant literature to support the use of MRA head without IV contrast for evaluation of bilateral NPT.


Tinnitus

Tinnitus. MRI Head and Internal Auditory Canal With IV Contrast There is no relevant literature to support the use of MRI head and IAC with IV contrast for evaluation of bilateral NPT.


Tinnitus

Tinnitus. MRI Head and Internal Auditory Canal Without and With IV Contrast There is no relevant literature to support the use of MRI head and IAC without and with IV contrast for evaluation of bilateral NPT.


Tinnitus

Tinnitus. MRI Head and Internal Auditory Canal Without IV Contrast There is no relevant literature to support the use of MRI head and IAC without IV contrast for evaluation of bilateral NPT.


Tinnitus

Tinnitus. MRV Head With IV Contrast There is no relevant literature to support the use of MRV head with IV contrast for evaluation of bilateral NPT.


Tinnitus

Tinnitus. MRV Head Without and With IV Contrast There is no relevant literature to support the use of MRV head without and with IV contrast for evaluation of bilateral NPT.


Tinnitus

Tinnitus. MRV Head Without IV Contrast There is no relevant literature to support the use of MRV head without IV contrast for evaluation of bilateral NPT.


Tinnitus

Tinnitus. US Duplex Doppler Carotid Artery There is no relevant literature to support the use of US duplex Doppler carotid artery for evaluation of bilateral NPT.


Tinnitus

Tinnitus. US Duplex Doppler Transcranial There is no relevant literature to support the use of US duplex Doppler transcranial for evaluation of bilateral NPT.


Tinnitus

Tinnitus

Tinnitus. Tinnitus


Tinnitus

Tinnitus. Supporting Documents The evidence table, literature search, and appendix for this topic are available at https://acsearch.acr.org/list. The appendix includes the strength of evidence assessment and the final rating round tabulations for each recommendation.


Tinnitus

Tinnitus. For additional information on the Appropriateness Criteria methodology and other supporting documents go to www.acr.org/ac.


Post Treatment Surveillance of Bladder Cancer. Introduction/Background Urothelial carcinoma (UC), previously known as transitional cell carcinoma, accounts for >90% of all urinary bladder cancers in the United States. In the genitourinary tract, UC is the second most common cancer and cause of cancer death [1]. The American Cancer Society estimated that there will be 81,400 new cases of bladder cancer and 17,980 deaths related to bladder cancer in 2020 [1]. Bladder cancer staging is based on the American Joint Committee on Cancer Tumor, Node, Metastasis system, and T-stage (depth of invasion) is used to differentiate patients into 2 groups: nonmuscle invasive bladder cancer (NMIBC) and muscle-invasive bladder cancer (MIBC) [2]. NMIBC accounts for 75% of bladder cancers and consists of a heterogeneous group of tumors that includes superficial papillary tumors (Ta), carcinoma in situ (Tis), and tumors invading the lamina propria (T1), all with different rates of recurrence and progression [2]. MIBC consists of tumors that invade the muscularis propria (T2) and beyond, and these tumors have a significantly higher rate of recurrence and progression after treatment. The 5- year survival rate for all stages of UC of the urinary bladder combined is 78% [1]. For NMIBC stages 0 and I, the 5-year survival rates are 95% and 75%, respectively; 5-year survival rates drop to 70%, 35%, and 5% for MIBC at stages II, III, and IV, respectively [1].


Post Treatment Surveillance of Bladder Cancer. Reprint requests to: publications@acr.org


Post Treatment Surveillance of Bladder Cancer. Post-Treatment Surveillance of Bladder Cancer The AUA/SUO and National Comprehensive Cancer Network (NCCN) guidelines differ slightly in imaging recommendations following treatment for NMIBC. The NCCN guidelines recommend upper-tract surveillance imaging for patients at low or intermediate risk, as clinically indicated, and scheduled upper-tract imaging every 1 to 2 years for patients at high risk [17]. The AUA/SUO guidelines recommend upper-tract surveillance imaging patients at both intermediate and high risk at 1 to 2 year intervals [4]. For the purposes of this manuscript, NMIBC has been divided into 2 categories: NMIBC without symptoms or risk factors (low-risk patients) and NMIBC with symptoms or risk factors (intermediate- and high-risk patients). Local practice patterns (NCCN versus AUA/SUO) should determine whether upper-tract surveillance should be considered in patients with intermediate risk and no symptoms. Special Imaging Considerations Cystoscopic and Virtual Cystoscopic Surveillance Patients with NMIBC undergo routine surveillance cystoscopy to assess for recurrence and progression to MIBC. As cystoscopy is a relatively invasive procedure, there was previous interest in developing virtual cystoscopic or cystographic techniques using CT or MRI, particularly for problem solving and for cases in which conventional cystoscopy is difficult, such as for the evaluation of narrow-necked diverticula. CT cystography, following the instillation of air, saline, or water-soluble contrast into the urinary bladder via a Foley catheter, and MRI evaluation of the urinary bladder with virtual cystoscopy (3-D fly through) and cystography (T2-weighted turbo spin-echo imaging) are not commonly performed and do not eliminate the need for conventional cystoscopy.


Post Treatment Surveillance of Bladder Cancer. CTU CTU CT urography (CTU) is an imaging study that is tailored to improve visualization of both the upper and lower urinary tracts. There is variability in the specific parameters, but it usually involves unenhanced images followed by intravenous (IV) contrast-enhanced images, including nephrographic and excretory phases acquired at least 5 minutes after contrast injection. Alternatively, a split-bolus technique uses an initial loading dose of IV contrast and then obtains a combined nephrographic-excretory phase after a second IV contrast dose; some sites include arterial phase. CTU should use thin-slice acquisition. Reconstruction methods commonly include maximum-intensity projection or 3-D volume rendering. For the purposes of this document, we make a distinction between CTU and CT abdomen and pelvis without and with IV contrast. CT abdomen and pelvis without and with IV contrast is defined as any protocol not specifically tailored for evaluation of the upper and lower urinary tracts and without both the precontrast and excretory phases.


Post Treatment Surveillance of Bladder Cancer. MRU MR urography (MRU) is also tailored to improve imaging of the urinary system. Unenhanced MRU relies upon heavily T2-weighted imaging of the intrinsic high signal intensity from urine for evaluation of the urinary tract. IV contrast is administered to provide additional information regarding obstruction, urothelial thickening, focal lesions, and stones. A contrast-enhanced T1-weighted series should include corticomedullary, nephrographic, and excretory phase. Thin-slice acquisition and multiplanar imaging should be obtained. For the purposes of this document, we make a distinction between MRU and MRI abdomen and pelvis without and with IV contrast. MRI abdomen and pelvis without and with IV contrast is defined as any protocol not specifically tailored for evaluation of the upper and lower urinary tracts, without both the precontrast and excretory phases, and without heavily T2-weighted images of the urinary tract.


Post Treatment Surveillance of Bladder Cancer. Discussion of Procedures by Variant Variant 1: Nonmuscle invasive bladder cancer with no symptoms or risk factors. Post-treatment surveillance. In patients with NMIBC without symptoms or risk factors, metastatic disease is uncommon, thus screening for distant metastatic disease is not recommended. Bladder recurrence is common following treatment for NMIBC. In a study of 190 patients with low-grade Ta disease, bladder cancer recurrence was identified in 43.2% (82 of 190)


Post Treatment Surveillance of Bladder Cancer. Post-Treatment Surveillance of Bladder Cancer


Post Treatment Surveillance of Bladder Cancer. of patients; however, progression to MIBC was seen in only 2 patients [18]. The incidence of upper-tract UC (UTUC) in this patient population is 0.6% to 0.9% [19,20]. Routine surveillance of the upper urinary tract in asymptomatic, low-risk patients is not recommended. Urine cytological analysis and cystoscopy are performed routinely in the setting of NMIBC and are felt to be sufficiently accurate for the diagnosis of bladder cancer recurrent in this patient population [4,21,22].


Post Treatment Surveillance of Bladder Cancer. CT Abdomen and Pelvis In patients with NMIBC without risk factors or symptoms, screening for distant metastatic disease with cross- sectional imaging (CT abdomen and pelvis without or with IV contrast) is not supported.


Post Treatment Surveillance of Bladder Cancer. CT Chest Chest CT is generally not appropriate for patients with NMIBC without symptoms or risk factors.


Post Treatment Surveillance of Bladder Cancer. CTU CTU CTU is a primary imaging test for comprehensive evaluation of the genitourinary tract that can be used to identify metastatic disease and metachronous UC. In the setting of NMIBC without symptoms or risk factors, metastatic disease is uncommon; thus, screening for distant metastatic disease is not supported.


Post Treatment Surveillance of Bladder Cancer. Although bladder recurrence is common, CT is not supported to screen for bladder recurrence, and it is generally felt that urine cytological evaluation and cystoscopy are sufficiently accurate for the diagnosis of bladder recurrence in this patient population.


Post Treatment Surveillance of Bladder Cancer. The incidence of UTUC in this patient population is 0.6% to 0.9% [19,20]. In addition, in a study of 935 patients with history of NMIBC, only 29% (15 of 51) of UTUCs were diagnosed on routine imaging while the remaining UTUCs were diagnosed once patients became symptomatic, for an overall imaging efficacy of 0.49% (15 UTUC out of 3,074 CT examinations) [23]. Routine surveillance of the upper urinary tract in asymptomatic, low-risk patients is not supported.


Post Treatment Surveillance of Bladder Cancer. FDG-PET/CT Skull Base to Mid-Thigh Imaging with PET using the tracer fluorine-18-2-fluoro-2-deoxy-D-glucose (FDG)/CT is generally not appropriate for patients with NMIBC without symptoms or risk factors. The risk of metastatic disease is extremely low. FDG is excreted by the kidneys, and activity obscures evaluation of the upper and lower urinary tract for recurrent disease.


Post Treatment Surveillance of Bladder Cancer. MRI Abdomen and Pelvis In patients with NMIBC without risk factors or symptoms, screening for distant metastatic disease with cross- sectional imaging is not supported.


Post Treatment Surveillance of Bladder Cancer. There has been increasing interest in using MRI for local staging of bladder cancer in the pretreatment setting [24- 28]. However, progression to MIBC in this patient population is rare. MRI has been used to evaluate the urinary bladder following transurethral resection of bladder tumor (TURBT). In a study including 47 patients with recurrent bladder cancer, MRI demonstrated a sensitivity of 67% and 73% and a specificity of 81% and 62% for 2 readers, respectively, and false-negatives included low-grade Ta lesions [29]. In another study, diffusion-weighted imaging (DWI) had a sensitivity of 100% and specificity of 81.8% for recurrent tumor in 11 patients [30].


Post Treatment Surveillance of Bladder Cancer. Despite these results, there is limited data for using MRI as a screening test in patients with previously treated bladder cancer. At this time, it is generally felt that urine cytological evaluation and conventional cystoscopy are sufficiently accurate for the diagnosis of bladder recurrence in this patient population.


Post Treatment Surveillance of Bladder Cancer. MRU MRU can be used as a primary imaging test for comprehensive evaluation of the genitourinary tract that can be used to identify metastatic disease and metachronous UC. Currently, evaluation for metastatic disease and routine surveillance of the upper urinary tract in asymptomatic, low-risk patients is not supported. Although MRU has been shown to have a sensitivity of 63% and specificity of 91% in a small study of 35 patients with suspected UTUC, the incidence of UTUC in this patient population is only between 0.6% and 0.9% [19,20,31].


Post Treatment Surveillance of Bladder Cancer. Radiography Chest Chest radiography is generally not appropriate for patients with NMIBC without symptoms or risk factors.


Post Treatment Surveillance of Bladder Cancer. Radiography Intravenous Urography CTU and, to a lesser extent, MRU have replaced IV urography (IVU) for the evaluation of the upper urinary tract. IVU does not have a current role in surveillance of NMIBC.


Post Treatment Surveillance of Bladder Cancer. Post-Treatment Surveillance of Bladder Cancer US Pelvis (Bladder) Because cystoscopy is relatively invasive and time consuming, there is interest in noninvasive and effective imaging modalities to identify recurrent bladder cancer. In a small prospective study, transabdominal ultrasound (US) was found to have a sensitivity of 78.5% and specificity of 100% for the diagnosis of recurrent UC of the urinary bladder, with cystoscopy as the reference standard [32]. In this study, US accurately diagnosed bladder cancer in 78.6% (11 of 14) of patients, missing 2 tumors <3 mm and 1 lesion located in a diverticulum. In another study, the combination of grayscale US, multiplanar reconstruction, and 3-D virtual US had a sensitivity of 96.4% and specificity of 88.8% compared with conventional cystoscopy [33]. Despite these results, it is generally understood that US has limited ability to identify MIBC in clinical practice and is sparingly used. As cystoscopy allows identification of recurrent neoplasm, concurrent biopsy, and local staging, US has not replaced the need for conventional cystoscopic surveillance for patients with NMIBC.


Post Treatment Surveillance of Bladder Cancer. Variant 2: Nonmuscle invasive bladder cancer with symptoms or risk factors. Post-treatment surveillance. Patients with NMIBC and risk factors require frequent surveillance for recurrent bladder cancer, which is generally done with conventional cystoscopy. In patients at intermediate risk with a history of TURBT and intravesical chemotherapy, recurrent bladder cancer is identified in up to 57% (413 of 724) of patients [8]. In patients at high risk, 59.6% (2,694 of 4,521) of patients develop multiple recurrences within 2 years of initial treatment [34]. In addition, progression to MIBC is seen in 8.6% to 15% of patients with high-risk disease [35-37].


Post Treatment Surveillance of Bladder Cancer. CT Abdomen and Pelvis NMIBC is a heterogeneous group of tumors, and although distant metastatic disease is uncommon in this patient population, cross-sectional imaging may be used to assess for metastatic disease in patients with symptoms or risk factors. There is no relevant literature regarding the use of CT abdomen and pelvis without or with IV contrast for the evaluation of metastatic bladder cancer; however, in the absence of contraindications, IV contrast is generally indicated to improve sensitivity for the identification of metastatic disease. CT abdomen and pelvis without and with IV contrast (excluding CTU) adds little information over CT abdomen and pelvis with IV contrast and does not offer a complete examination of the urinary tract. CTU, however, is a comprehensive examination and can be used to assess for metastatic disease and metachronous upper-tract UC (see below).


Post Treatment Surveillance of Bladder Cancer. CT Chest Chest CT without or with IV contrast is generally not appropriate for patients with NMIBC with symptoms or risk factors, unless an abnormality is identified with chest radiography.


Post Treatment Surveillance of Bladder Cancer. CTU CTU is a primary imaging test for comprehensive evaluation of the genitourinary tract that can be used to identify metastatic disease and metachronous UC in patients with NMIBC who have symptoms or risk factors. Although CTU has not replaced cystoscopy, CT performs well in identifying recurrent bladder cancer following TURBT. In a study of CTU in 121 patients at risk for urothelial recurrence after TURBT (with symptoms or positive urine cytology), 59 bladder recurrences were identified in 38 patients. The authors found that overall accuracy was better in the urinary bladder during the nephrographic phase compared with the pyelographic/excretory phase (91.7% [354 of 386] versus 83.2% [321 of 386], P = .038) [38]. In another study of patients with a history of UC, CTU had a sensitivity of 77.8% (63 of 81) and specificity of 77.9% (60 of 77) for the detection of bladder cancer [39].


Post Treatment Surveillance of Bladder Cancer. CTU for the evaluation of the upper urinary tract is effective in patients with symptoms, particularly in the setting of a negative cystoscopy. In a study of CTU in 121 patients at risk for urothelial recurrence after TURBT (with symptoms or positive urine cytology), 19 upper-tract recurrences were identified in 13 patients. In this study, accuracy for upper-tract recurrence was better in the nephrographic phase compared with the pyelographic phase (86.7% [260 of 300] versus 80.0% [240 of 300], P = .028) [38].


Post Treatment Surveillance of Bladder Cancer. FDG-PET/CT Skull Base to Mid-Thigh FDG-PET/CT is generally not appropriate for patients with NMIBC. The risk of metastatic disease is extremely low, FDG is excreted by the kidneys, and activity obscures evaluation of the upper and lower urinary tract for recurrent disease.


Post Treatment Surveillance of Bladder Cancer. MRI Abdomen and Pelvis Although distant metastatic disease is uncommon in this patient population, cross-sectional imaging may be used to assess for metastatic disease in patients with symptoms or risk factors. There is no relevant literature regarding the use of MRI abdomen and pelvis without IV contrast in the evaluation of metastatic UC. Given the improved


Post Treatment Surveillance of Bladder Cancer. Post-Treatment Surveillance of Bladder Cancer soft-tissue contrast of MRI compared with CT, MRI of the abdomen and pelvis without IV contrast may be acceptable for the identification of metastatic disease; however, MRI without and with IV contrast is preferred to improve sensitivity. MRU, however, is a comprehensive examination and can be used to assess for metastatic disease and metachronous upper-tract UC (see below).


Post Treatment Surveillance of Bladder Cancer. There has been increasing interest in using MRI for local staging of bladder cancer in the pretreatment setting. Several meta-analyses of MRI for local staging of bladder cancer have been performed. For the differentiation of NMIBC from MIBC, sensitivity ranges from 97% to 92% and specificity ranges from 79% to 88% [24-26]. Vesical Imaging-Reporting and Data System (VI-RADS) using multiparametric MRI with T2-weighted imaging, DWI and dynamic contrast-enhanced imaging has been developed to identify MIBC and standardize reporting. A multireader validation study of VI-RADS for the identification of MIBC demonstrated an intraclass correlation coefficient of 0.85 among 5 readers with a pooled area under the curve of 0.90 [27]. A larger study of 340 patients (255 with NMIBC and 85 with MIBC) concluded that VI-RADS had an accuracy of 94% for identifying MIBC among 2 readers [28].


Post Treatment Surveillance of Bladder Cancer. Despite these results, there are limited data for use of MRI as a screening test in patients with previously treated bladder cancer. At this time, it is generally felt that urine cytological evaluation and conventional cystoscopy are sufficiently accurate for the diagnosis of bladder recurrence in this patient population.


Post Treatment Surveillance of Bladder Cancer. MRU MRU offers a comprehensive evaluation of the genitourinary tract and can be used to evaluate for metastatic disease and metachronous UTUC following treatment of NMIBC. In a study of 91 examinations in 88 patients with suspected UTUC, MRU had a sensitivity of 72.4% to 86.2% and specificity of 97.9% to 99.5% for 2 readers, respectively [40].


Post Treatment Surveillance of Bladder Cancer. Radiography Chest Metastatic disease in patients with NMIBC is uncommon; however, chest radiography may be appropriate in patients with NMIBC with symptoms or risk factors.


Post Treatment Surveillance of Bladder Cancer. Radiography Intravenous Urography CTU and, to a lesser extent, MRU have replaced IVU for the evaluation of the upper urinary tract. IVU does not have a current role in surveillance of NMIBC.


Post Treatment Surveillance of Bladder Cancer. US Pelvis (Bladder) In a small prospective study, transabdominal US was found to have a sensitivity of 78.6% and specificity of 100% for the diagnosis of recurrent UC of the urinary bladder, with cystoscopy as the reference standard [32]. In this study, US accurately diagnosed bladder cancer in 78.6% (11 of 14) of patients, missing 2 tumors <3 mm and 1 lesion located in a diverticulum. In another study, the combination of grayscale US, multiplanar reconstruction, and 3-D virtual US had a sensitivity of 96.4% and specificity of 88.8% compared with conventional cystoscopy [33]. Despite these results, US has limited ability to identify MIBC or nodal metastatic disease. As cystoscopy allows identification of recurrent neoplasm, concurrent biopsy, and local staging, US has not replaced the need for cystoscopic surveillance for patients with NMIBC.


Post Treatment Surveillance of Bladder Cancer. Variant 3: Muscle-invasive bladder cancer with or without cystectomy. Post-treatment surveillance. Following radical cystectomy for MIBC, 5-year recurrence-free survival is approximately 58%; risk factors for recurrence include advanced tumor stage, lymph node involvement, lymphovascular invasion, high tumor grade, and positive surgical margins [10,41-43]. Recurrences can be described as pelvic relapse; surgical bed recurrence; internal and external iliac and obturator lymph node involvement or distant metastatic disease; and osseous, pulmonary, hepatic, extrapelvic lymphadenopathy, peritoneal, and brain metastases. Most recurrences occur within the first 2 years following cystectomy, and most recurrences are distant metastatic disease [44]. Pelvic relapse is seen in 34% of patients, and the 2-year risk of local failure exceeds 30% [45].


Post Treatment Surveillance of Bladder Cancer. Post-Treatment Surveillance of Bladder Cancer In a study of 1,110 patients following radical cystectomy, recurrences were identified in 29.2% (324 of 1,110) of patients, and 61.7% (200 of 324) of recurrences were single-site recurrences with 43 local (22 cystectomy bed and 21 pelvic lymph node) and 138 distant (36 lung, 19 liver, 52 bone, 17 extrapelvic lymph node, 7 peritoneal, 4 brain, and 3 other) [46]. In a smaller study of 343 patients, 46% (158) of patients developed recurrence; 30% (104) were distant, 6% (21) were distant and local, and 10% (33) were only local. Eighty-four percent of recurrences were identified within 2 years. Following cystectomy, patients are also at risk of developing UTUC, which is found in up to 3.7% of patients [47,48]. As recurrence can involve the entire urinary tract, the urethra also needs to be screened, often with urethral wash cytology, although urethral recurrence may occasionally be identified on cross- sectional imaging. The risk of urethral recurrence is 2.7% to 3.8%, and risk factors include prostatic involvement of the MIBC [47-49].


Post Treatment Surveillance of Bladder Cancer. CT Abdomen and Pelvis As described earlier, recurrences can be described as pelvic relapse; surgical bed recurrence; internal and external iliac and obturator lymph node involvement or distant metastatic disease; and osseous, pulmonary, hepatic, extrapelvic lymphadenopathy, peritoneal, and brain metastases.


Post Treatment Surveillance of Bladder Cancer. There is no relevant literature regarding the use of CT abdomen and pelvis without or with IV contrast for the evaluation of metastatic bladder cancer; however, in the absence of contraindications, IV contrast is generally indicated to improve sensitivity for the identification of metastatic disease. CT of the abdomen and pelvis without and with IV contrast (excluding CTU) adds little over CT abdomen and pelvis with IV contrast and does not offer a complete examination of the urinary tract.


Post Treatment Surveillance of Bladder Cancer. CT Chest All patients with MIBC require imaging of the thorax. In the setting of bladder cancer, there is a lack of data comparing the utility of chest radiography and chest CT. Chest radiography is an effective screening examination and should be performed at regular intervals. Any abnormality identified at radiography should be followed up with a CT examination for improved characterization. There is no relevant literature regarding the use of CT chest without or with and without IV contrast in the evaluation of bladder cancer metastases to the thorax; however, CT chest is often performed as a component of the imaging follow-up of patients with MIBC.


Post Treatment Surveillance of Bladder Cancer. CTU CTU CTU is a primary imaging test for comprehensive evaluation of the genitourinary tract and can be used to identify distant metastatic disease and metachronous UTUC in this patient population. In one study, accuracy of CTU for UTUC was better in the nephrographic phase compared with the pyelographic phase for upper-tract recurrences (86.7% [260 of 300] versus 80.0% [240 of 300], P = .028), although the 2 phases are complementary [38].


Post Treatment Surveillance of Bladder Cancer. FDG-PET/CT Skull Base to Mid-Thigh FDG-PET/CT in the setting of MIBC is typically used to resolve equivocal findings identified on other imaging tests, but there is increasing evidence that FDG-PET/CT alters patient management and has prognostic value compared with other staging examinations.


Post Treatment Surveillance of Bladder Cancer. Kibel et al [50] evaluated FDG-PET/CT prior to cystectomy for MIBC and found that FDG-PET/CT had a sensitivity of 70% (7 of 10) and specificity of 94% (30 of 32) for metastatic disease. However, occult metastatic disease was found in 7 of 42 patients with FDG-PET/CT compared with CT alone. In another study of 44 patients with MIBC, FDG-PET/CT demonstrated a sensitivity of 57% for pelvic lymph node involvement compared with 33% for CT, and FDG-PET/CT identified all bone lesions that were detected by scintigraphy [51]. A more recent study demonstrated a sensitivity of 62% to 79% for nodal metastases based on standardized uptake values [52]. A meta-analysis for nodal metastatic disease demonstrated a pooled sensitivity of 57% and specificity of 92% [53]. Given FDG activity in excreted urine, pelvic staging may be difficult. One group of authors found that with diuretics and oral hydration there was improved assessment of locally recurrent disease [54].


Post Treatment Surveillance of Bladder Cancer. In a study that included 41 patients with suspected recurrent bladder cancer after primary treatment that underwent FDG-PET/CT, authors found that FDG-PET/CT had a sensitivity of 87% and specificity of 94% for recurrent/metastatic bladder cancer following treatment [55]. In this study, metastatic disease was found in abdominal and pelvic lymph nodes, including suprarenal lymph nodes; pulmonary and osseous metastatic disease was also identified. Perhaps more importantly, the results of the FDG-PET/CT changed the treatment decision in 40% of patients and had prognostic value in determining overall survival and progression-free survival. In another study of the National Oncologic PET Registry, authors found that FDG-PET/CT changed management in approximately 35% of patients and had a large impact on chemotherapy monitoring [56]. In addition, there is


Post Treatment Surveillance of Bladder Cancer. Post-Treatment Surveillance of Bladder Cancer


Post Treatment Surveillance of Bladder Cancer. increasing evidence that FDG-PET/CT can be used to assess for treatment response after neoadjuvant or induction chemotherapy [57-59]. Although not widely available, there is increasing interest in 11C-choline-PET. In a study of 27 patients with either MIBC or recurrent NMIBC that failed TURBT and intravesical therapy, the presence of residual bladder cancer was detected in 84% (21 of 25) of patients with CT and 96% (24 of 25) of patients with 11C-choline PET, and lymph node involvement was identified correctly in 50% (4 of 8) of patients with CT and 62% (5 of 8) of patients with PET [60].


Post Treatment Surveillance of Bladder Cancer. Fluoroscopy Abdomen Loopogram Abdominal radiography can be useful in the early postoperative setting to evaluate for ureteral stent location and to evaluate patients with abdominal distention and postoperative ileus. A fluoroscopic loopogram, in which water- soluble contrast is instilled into an ileal conduit in a retrograde fashion, can be used to evaluate for leak in the early postoperative period and to confirm patent ureteral anastomoses in the setting of hydronephrosis and declining renal function following urinary diversion. Abdominal radiography and fluoroscopic examinations are not useful for detection of tumor recurrence.


Post Treatment Surveillance of Bladder Cancer. MRI Abdomen and Pelvis MRI performs well for identifying metastatic disease within the abdomen and pelvis; however, nodal disease is largely based on size criteria. A recent meta-analysis evaluating nodal metastatic disease in the setting of bladder or prostate cancer demonstrated a pooled per-patient sensitivity of 56% and specificity of 94% [61]. There is no relevant literature regarding the use of MRI of the abdomen and pelvis without IV contrast in the evaluation of metastatic UC. Given the improved soft-tissue contrast of MRI compared with CT, MRI of the abdomen and pelvis without IV contrast may be acceptable for the identification of metastatic disease; however, MRI without and with IV contrast is preferred to improve sensitivity.


Post Treatment Surveillance of Bladder Cancer. Although MRI can be used for local staging of bladder cancer, the presence of inflammation and fibrosis affects the accuracy of MRI following neoadjuvant chemoradiation, when accuracy drops to only 30% [62]. However, DWI may help distinguish inflammation and fibrosis from tumor; in a small study of 20 patients who underwent low- dose neoadjuvant chemoradiation, MRI had an accuracy rate of 44% in determining pathologic response for T2- weighted imaging alone, 33% for dynamic contrast-enhanced imaging, and 80% for DWI [63].


Post Treatment Surveillance of Bladder Cancer. MRU MRU is a primary imaging test for the comprehensive evaluation of the genitourinary tract and can be used to assess for metastatic disease and metachronous UTUC. In a study of 91 examinations in 88 patients with suspected UTUC, MRU had a sensitivity of 72.4% to 86.2% and specificity of 97.9% to 99.5% for UTUC for 2 readers, respectively [40].


Post Treatment Surveillance of Bladder Cancer. Radiography Chest All patients with MIBC require imaging of the thorax. Chest radiographs are an effective screening examination and should be performed at regular intervals. Any abnormality identified on radiography should be followed with a CT examination for improved characterization.


Post Treatment Surveillance of Bladder Cancer. Radiography Intravenous Urography CTU and, to a lesser extent, MRU have replaced IVU for the evaluation of the upper urinary tract. In a study of 128 patients at high risk for UTUC, in whom 46 patients were diagnosed with UTUC, excretory urography had a per- patient sensitivity of 80.4% (37 of 46) and a specificity of 81.0% (47 of 58), whereas CTU had a sensitivity of 93.5% (43 of 46) and a specificity of 94.8% (55 of 58) [64]. IVU is not recommended for detection of tumor recurrence. However, IVU could be used to assess for ureteral anastomotic patency if reflux cannot be demonstrated on a loopogram.


Post Treatment Surveillance of Bladder Cancer. US Pelvis (Bladder) Following cystectomy, the acoustic window is limited, and US is of little clinical use for the identification of local recurrence or nodal metastatic disease. Given the high incidence of recurrent disease (up to 46% of patients) following cystectomy for MIBC, surveillance imaging with CT or MRI is recommended [43]. US may be useful to assess the kidneys for hydronephrosis in the setting of declining renal function, regardless of whether the urinary bladder has been resected or not.


Post Treatment Surveillance of Bladder Cancer. Supporting Documents The evidence table, literature search, and appendix for this topic are available at https://acsearch.acr.org/list. The appendix includes the strength of evidence assessment and the final rating round tabulations for each recommendation.


Post Treatment Surveillance of Bladder Cancer. For additional information on the Appropriateness Criteria methodology and other supporting documents go to www.acr.org/ac.


Post Treatment Surveillance of Bladder Cancer. Appropriateness Category Names and Definitions Relative Radiation Level Information Potential adverse health effects associated with radiation exposure are an important factor to consider when selecting the appropriate imaging procedure. Because there is a wide range of radiation exposures associated with different diagnostic procedures, a relative radiation level (RRL) indication has been included for each imaging examination. The RRLs are based on effective dose, which is a radiation dose quantity that is used to estimate population total radiation risk associated with an imaging procedure. Patients in the pediatric age group are at


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. Introduction/Background Soft tissue vascular anomalies (VAs) may be diagnosed prenatally or at any time during life [1-3]. Anomalies of the soft tissues may be located in the extremities, face, scalp, neck, airway, thoracoabdominal wall, mediastinum, lungs, and abdomen (mesentery, retroperitoneum, and viscera). They may be associated with syndromes and may signal the presence of internal vascular lesions. Soft tissue VAs are subdivided into broad categories of vascular malformations (VMs) and vascular tumors (VTs). VTs are true neoplasms with increased mitotic activity and endothelial cell turnover; VMs are composed of abnormal or defective formation of vascular tissue [4] (see Appendix 1).


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. The prevalence of VMs varies by type: venous malformations (70%), lymphatic malformations (12%), arteriovenous malformations (AVMs) (8%), combined malformation syndromes (6%), and capillary malformations (4%) [5]. They may be divided into simple (further divided into low-flow or fast-flow VMs) or combined. Low- flow, simple VMs often contain 1 type of low-flow vessel: capillary, lymphatic, or venous vessel. AVMs or arteriovenous fistulas (AVFs) are fast-flow, simple VMs. Combined VMs are composed of more than 2 types of simple VM components and may be named for a major vessel [4,6]. Complex anomalies may be associated with overgrowth syndromes, which are often composed of infiltrative venous and lymphatic tissues through thickened subcutaneous fat affecting the trunk and/or limbs [7-10] (see Appendix 2).


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. VTs are divided into masses that behave in a benign, locally aggressive, borderline, or malignant manner. The most common benign VT is infantile hemangioma, which presents in the newborn period, whereas other VTs, malignant and other aggressive vascular lesions, may be diagnosed at any age. Most benign lesions are observed or treated in a noninvasive manner. Locally aggressive and borderline VTs present shortly after birth and may present with thrombocytopenia and/or a consumptive coagulopathy, which can complicate treatment. Malignant VTs are rapidly growing masses found in children of all ages, are often more aggressive than similar tumors in adults, and may be difficult to accurately diagnose due to poorly differentiated cell type [4,6].


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. Initial Imaging Definition Initial imaging is defined as imaging at the beginning of the care episode for the medical condition defined by the variant. More than one procedure can be considered usually appropriate in the initial imaging evaluation when:


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. The American College of Radiology seeks and encourages collaboration with other organizations on the development of the ACR Appropriateness Criteria through representation of such organizations on expert panels. Participation on the expert panel does not necessarily imply endorsement of the final document by individual contributors or their respective organization.


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. Reprint requests to: publications@acr.org


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. OR Discussion of Procedures by Variant Variant 1: Infant. Clinical signs or symptoms of infantile hemangioma. Initial imaging. Infantile hemangiomas are the most common benign neoplasm of infancy, with a prevalence of 4% to 5% [13]. Infantile hemangiomas, distinct from VMs, are a true neoplasm rather than an abnormality of embryonic development of vascular tissue (see Appendix 1). They become clinically evident within the first few weeks of life and progress through phases of latency, growth, and plateau, predictably by the first year of life; in rare cases, growth continues through 24 months of age. With either complete or partial involution, lesion regression is completed by 4 years of age in 90% of cases but may continue through to 8 years of age [13,14]. Risk factors for having infantile hemangiomas include prematurity, White race (3%-10%), and female sex (female:male ratio range 1.4:1 to 3:1) [15].


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. A rare, but important, location of infantile hemangiomas is in the airway, because rapid proliferation of the VT can obstruct the airway. Infantile hemangiomas of the airway are most commonly localized in the subglottic airway but may be more diffuse, in a beard-like distribution throughout the soft tissues overlying the mandible and neck [18]. Subglottic infantile hemangiomas may also extend from the neck into the mediastinum.


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. Infantile hemangioma may also be associated with VAs of other organs. PHACE, an acronym for posterior fossa malformations, hemangioma, arterial anomalies, coarctation of the aorta/cardiac defects, and eye abnormalities, is predominantly a neurovascular malformation syndrome [19]. Imaging of the non-CNS lesions in PHACE syndrome, although not specifically addressed, are included in the recommendations of Variant 1. Imaging recommendations of hepatic hemangiomas found in patients with multiple cutaneous infantile hemangiomas are presented in Variant 2.


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. Arteriography Area of Interest There is no relevant literature to support the use of arteriography of the area of interest as the initial imaging modality in infants with infantile hemangiomas.


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. CT Area of Interest With IV Contrast When optimal imaging of the airway is required, as with hemangiomas involving the supra- or infraglottic airway or when in a beard-like distribution over the face and neck, CT with intravenous (IV) contrast may be useful. In a study of 11 children with hemangiomas of the upper airway who underwent CT, Koplewitz et al [20] found CT to have an improved definition of the airway lesion, presence, localization, and complete extent of the lesion and a more accurate size assessment compared with bronchoscopy.


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. CT Area of Interest Without and With IV Contrast There is no relevant literature to support the use of CT area of interest without and with IV contrast as the initial imaging modality in infants with infantile hemangiomas. In the study by Koplewitz et al [20], noncontrast and contrast-enhanced CT scans were performed. It was found that the lesions appeared larger after IV contrast was given and that the complete extent and localization of the lesion could be best defined.


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. Soft Tissue Vascular Anomalies-Child CT Area of Interest Without IV Contrast There is no relevant literature to support the use of CT area of interest without IV contrast as the initial imaging modality in infants with infantile hemangiomas.


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. CTA and CTV Area of Interest With IV Contrast There is no relevant literature to support the use of CT angiography (CTA) or CT venography (CTV) area of interest with IV contrast as the initial imaging modality in infants with infantile hemangiomas.


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. MRA and MRV Area of Interest Without and With IV Contrast Dynamic MR angiography (MRA) and MR venography (MRV) with IV contrast of untreated infantile hemangiomas is capable of showing supplying arterial and draining venous vessels [15].


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. MRI Area of Interest Without and With IV Contrast MRI of the area of interest without and with IV contrast in patients with infantile hemangiomas may be useful when clinically determining the complete extent of the lesion is not possible, such as when the infantile hemangiomas of the face and deep facial structures or periorbital and intraorbital extent must be defined, when lumbosacral region infantile hemangiomas are present and underlying tethering or other spinal cord anomaly may be present, and there are beard-type infantile hemangiomas that occupy the pharyngeal region and may affect the oropharyngeal airway. MRI may also be useful in patients with infantile hemangiomas in anatomic locations, when the presence or growth of the lesion may be disfiguring or interfere with sight or hearing, the face, airway, ears, or breast [17].


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. MRI Area of Interest Without IV Contrast There is no relevant literature to support the use of MRI of the area of interest without IV contrast as the initial imaging modality for infants with infantile hemangiomas.


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. Radiography Area of Interest There is no relevant literature to support the use of radiography area of interest as the initial imaging modality in infants with infantile hemangiomas.


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. US Area of Interest Ultrasound (US) of the area of interest is useful to distinguish imaging features of infantile hemangiomas from VMs. Paltiel et al [21] studied 49 lesions and Ding et al [14] studied 66 lesions, describing US imaging characteristics of superficial and deep infantile hemangiomas from well-circumscribed mixed echogenicity solid masses with central and peripheral vessels on grayscale US. Both groups showed that US is useful in distinguishing infantile hemangiomas from VMs and for identifying infantile hemangiomas, which may be combined with other VA components [14,21].


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. US Area of Interest With IV Contrast There is no relevant literature to support the use of US area of interest with IV contrast as the initial imaging modality in infants with infantile hemangiomas.


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. US Duplex Doppler Area of Interest US with duplex Doppler is most useful to assess and confirm diagnosis of infantile hemangiomas. Paltiel et al [21] studied 49 lesions and Ding et al [14] studied 66 lesions, showing that a combination of arterial and venous waveforms on US duplex Doppler enables distinguishing infantile hemangiomas from low-flow VMs.


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. A multicenter prospective study of 1,656 infants with infantile hemangiomas confirmed that patients with higher numbers of cutaneous infantile hemangiomas have a greater incidence of infantile hepatic hemangioma, 8.3% in patients with 5 to 9 lesions compared with 0.4% in patients with <5 cutaneous lesions. Based on the results of this study, screening liver imaging examination is indicated in patients with 5 cutaneous infantile hemangiomas and for patients up to 9 months of age [23].


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. Soft Tissue Vascular Anomalies-Child In an analysis of 121 children with hepatic hemangioma in the Liver Hemangioma Registry, Kulungowski et al [24] found that 88 children had multiple cutaneous infantile hemangiomas lesions, 68 (77%) of which were multifocal and 20 (23%) of which were diffuse type infantile hepatic hemangioma.


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. Arteriography Abdomen There is no relevant literature to support the use of arteriography of the abdomen as the initial imaging modality when screening for hepatic hemangiomas in infants with multiple cutaneous infantile hemangiomas.


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. CT Abdomen With IV Contrast There is no relevant literature to support the use of CT of the abdomen with IV contrast as the initial imaging modality when screening for hepatic hemangiomas in infants with multiple cutaneous infantile hemangiomas.


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. CT Abdomen Without and With IV Contrast There is no relevant literature to support the use of CT of the abdomen without and with IV contrast as the initial imaging modality when screening for hepatic hemangiomas in infants with multiple cutaneous infantile hemangiomas.


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. CT Abdomen Without IV Contrast There is no relevant literature to support the use of CT of the abdomen without IV contrast as the initial imaging modality when screening for hepatic hemangiomas in infants with multiple cutaneous infantile hemangiomas.


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. CTA and CTV Abdomen With IV Contrast There is no relevant literature to support the use of CTA and CTV of the abdomen with IV contrast as the initial imaging modality when screening for hepatic hemangiomas in infants with multiple cutaneous infantile hemangiomas.


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. MRA and MRV Abdomen Without and With IV Contrast There is no relevant literature to support the use of MRA and MRV of the abdomen without and with IV contrast as the initial imaging modality when screening for hepatic hemangiomas in infants with multiple cutaneous infantile hemangiomas.


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. MRI Abdomen Without and With IV Contrast There is no relevant literature to support the use of MRI of the abdomen without and with IV contrast as the initial imaging modality when screening for hepatic hemangiomas in infants with multiple cutaneous infantile hemangiomas. A recent guidance document of the American Society of Pediatric Hematology Oncology Vascular Anomalies Special Interest Group recommends contrast-enhanced MRI of the liver including dynamic sequences if the diagnosis is unclear following Doppler US [25].


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. MRI Abdomen and Pelvis Without IV Contrast There is no relevant literature to support the use of MRI of the abdomen and pelvis without IV contrast as the initial imaging modality when screening for hepatic hemangiomas in infants with multiple cutaneous infantile hemangiomas.


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. Radiography Abdomen There is no relevant literature to support the use of radiography of the abdomen as the initial imaging modality when screening for hepatic hemangiomas in infants with multiple cutaneous infantile hemangiomas.


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. US Abdomen With IV Contrast US of the abdomen with IV contrast, specifically for evaluation of the liver, for the presence of hepatic hemangiomas in infants with multiple cutaneous infantile hemangiomas may be used to increase the sensitivity and diagnostic confidence, particularly for focal lesions that are seen in congenital hepatic hemangiomas rather than the diffuse and multifocal hepatic hemangiomas seen in infantile hemangiomas [26,27]. The addition of IV contrast for


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. Soft Tissue Vascular Anomalies-Child US of the abdomen has been shown by El-Ali et al [27] to differentiate infantile hepatic hemangioma in infants from congenital hemangioma of the liver in 5 infants based on the pattern of early and late arterial phase and delayed washout of IV contrast (P = .0016). The IV contrast enhancement pattern on US was similar to the manner in which the lesions are known to enhance with IV contrast on CT and MRI examinations [28].


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. US Duplex Doppler Abdomen A recent guidance document of the American Society of Pediatric Hematology Oncology Vascular Anomalies Special Interest Group recommends Doppler US of the liver as the preferred initial imaging study. On US examination imaging features such as multifocal and diffuse patterns are more specific to infantile hepatic hemangiomas and may be used to help differentiate them from congenital hemangiomas of the liver [25]. Solitary lesions may be larger in diameter and of heterogeneous echogenicity, with more prominent peripheral vascular components [26].


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. Variant 3: Child. Clinical signs or symptoms of vascular anomaly (tumor or malformation) not suggesting infantile hemangioma. Initial imaging. VAs are a diverse group of lesions including tumors with benign, locally aggressive, or malignant behaviors and malformations, which may involve low-flow (venous or lymphatic) or fast-flow (arterial) blood flow [4] (see Appendix 1). Many VAs are fully formed before birth and do not clinically present in a fashion typical of infantile hemangiomas. Imaging characterization of the lesion and delineation of its extent are warranted.


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. Arteriography Area of Interest There is no relevant literature to support the use of arteriography area of interest as the initial imaging modality for vascular lesion such as tumor or malformation.


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. CT Area of Interest With IV Contrast There is no relevant literature to support the use of CT area of interest with IV contrast as the initial imaging modality for vascular lesion such as tumor or malformation.


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. CT Area of Interest Without and With IV Contrast There is no relevant literature to support the use of CT area of interest without and with IV contrast as the initial imaging modality for vascular lesion such as tumor or malformation.


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. CT Area of Interest Without IV Contrast There is no relevant literature to support the use of CT area of interest without IV contrast as the initial imaging modality for vascular lesion such as tumor or malformation.


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. CTA and CTV Area of Interest With IV Contrast There is no relevant literature to support the use of CTA and CTV area of interest with IV contrast as the initial imaging modality for vascular lesion such as tumor or malformation.


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. MRA and MRV Area of Interest Without and With IV Contrast Using contrast-enhanced MRA and MRV patterns of high or low signal intensity can help distinguish between low- flow and fast-flow VMs. In a study by van Rijswijk et al [29], they describe a 95% specificity and 83% sensitivity in differentiating venous and nonvenous malformations using dynamic contrast-enhanced MRA. Dynamic 4-D MRA with IV contrast may be used to detect the presence of arteriovenous microshunts in VMs, which have been found to be associated with the presence of phleboliths [30].


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. Subtraction MRA techniques subtract MR signal from noncontrast-enhanced MR images from a contrast-enhanced MRA, resulting in improved visualization of vascular structures as soft tissue signal is removed [31]. There may also be value in the use of noncontrast MRA as an anatomic survey before performing contrast-enhanced MRA to facilitate planning of field of view and contrast bolus timing [32].


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. MRA Area of Interest Without IV Contrast Numerous noncontrast-enhanced MRA sequences are also available, relying on the speed of blood flow (flow- dependent) and subject to signal loss in vessels with slow blood flow. Flow-independent noncontrast-enhanced MRA sequences produce bright blood or dark blood pool images and enable imaging a larger field of view. Noncontrast MRA images may incorporate longer scan times and therefore produce higher spatial resolution images


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. Soft Tissue Vascular Anomalies-Child [33]. Noncontrast MRA does not provide dynamic flow information provided by contrast-enhanced MRA. Furthermore, although time-of-flight cannot be used to characterize soft tissue components, it does show the feeding and draining vessels of a fast-flow VM [34].


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. MRI Area of Interest Without and With IV Contrast MRI area of interest without and with IV contrast as the initial imaging modality for vascular lesion such as tumor or malformation may be helpful as the initial imaging examination and may be performed contemporaneously with US to investigate a soft tissue mass or skin discoloration.


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. MRI findings typically show a lobulated and often infiltrative soft tissue mass with T1 hypointense and T2 hyperintense signal, variable vascular flow voids, variable patterns of enhancement, and possibly phleboliths, depending upon the type of lesion. The addition of MRA may be beneficial to making a definitive diagnosis [31].


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. MRI Area of Interest Without IV Contrast MRI area of interest without IV contrast as the initial imaging modality for vascular lesion such as tumor or malformation may be helpful as the initial imaging examination and may be performed contemporaneously with US to investigate a soft tissue mass or skin discoloration.


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. MRI without IV contrast typically shows a lobulated and often infiltrative soft tissue mass with T1 hypointense and T2 hyperintense signal, variable vascular flow voids, and possibly phleboliths, depending upon the type of lesion. The lack of contrast will limit the ability to characterize the type of vessel and characterization of flow through the lesion (high- versus low-flow) and, therefore, the type of VM being evaluated. The addition of IV contrast and possibly MRA may be beneficial to making a more definitive, accurate characterization of the lesion and eventually the diagnosis [31].


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. Radiography Area of Interest There is no relevant literature to support the use of radiography area of interest as the initial imaging modality for vascular lesions such as tumor or malformation [35]. Radiographs may reveal calcifications within a soft tissue mass, indicating the diagnosis of VM, but is not typically the initial imaging study when a VA is suspected. Phleboliths occur at the site of microshunts in VMs.


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. US Area of Interest US may be used to distinguish characteristic features of VTs and to differentiate between low-flow and fast-flow VMs. Aside from infantile hemangioma, VTs reveal well-defined solid tissue components with variable echotexture [21].


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. Venous VMs can be partially characterized with grayscale US, and certain features such as multiple anechoic spaces, echogenic phleboliths, and expanded soft tissue spaces that are compressible (muscle, subcutaneous fat, dermis layers) can be diagnostic. Lymphatic VMs are also able to be at least partially characterized by grayscale US when multiple anechoic spaces with cysts, which may contain fluid-fluid levels in the event there has been prior infection or hemorrhage into the lesion, that are noncompressible are visualized. Venolymphatic VMs show a combination of the above features. Fast-flow VM-AVMs and AVFs show a cluster of vessels without an associated solid tissue mass [36,37] in contradistinction to hemangiomas.


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. Congenital hepatic hemangiomas are focal lesions of the liver associated with multiple cutaneous infantile hemangiomas.


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. US Area of Interest With IV Contrast US of the abdomen and pelvis with IV contrast, specifically for evaluation of the liver, for the presence of hepatic hemangiomas in infants with multiple cutaneous infantile hemangiomas may be used to increase sensitivity and diagnostic confidence, particularly for focal lesions, which are seen in congenital hepatic hemangiomas rather than the diffuse and multifocal hepatic hemangiomas seen in infantile hemangiomas [26,27]. The addition of IV contrast for US of the abdomen and pelvis has been shown by El-Ali et al [27] to differentiate infantile hepatic hemangioma in infants from congenital hemangioma of the liver in 5 infants based on the pattern of early and late arterial phase and delayed washout of IV contrast (P = .0016). The IV contrast enhancement pattern on US was similar to the manner in which the lesions are known to enhance with IV contrast on CT and MRI examinations [28].


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. US Duplex Doppler Area of Interest US with duplex Doppler may be used to distinguish characteristic features of VTs, verify arterial waveforms in fast- flow VMs (arterialization of draining veins), and differentiate between low-flow and fast-flow VMs. Aside from


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. Soft Tissue Vascular Anomalies-Child infantile hemangioma, VTs typically demonstrate both arterial and venous waveforms. Venous malformations show multiple anechoic spaces, but the flow in venous VMs may be so slow that it is difficult to perceive on Doppler. Lymphatic VMs are also composed of multiple anechoic spaces with cysts, which may contain fluid-fluid levels in the event there has been prior infection or hemorrhage but will not have Doppler signal. Venolymphatic malformations show a combination of these features. Fast-flow VM-AVMs and AVFs show a cluster of vessels without an associated solid tissue mass [36] with fast-flow on Doppler US.


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. Variant 4: Child. Ultrasound features raise suspicion for vascular malformation. Next imaging study. If initial US imaging raises suspicion for the diagnosis of VM, further imaging is often helpful to visualize the entire extent of the lesion, assess for the presence of multiple lesions, and evaluate possible involvement of adjacent tissues and organs (including sensitive anatomic regions such as deep facial structures, the airway, orbits, and the spine).


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. Arteriography Area of Interest Digital subtraction angiography provides an excellent definition of AVM anatomy, in particular the AVM nidus and the number and definition of feeding arteries and fistulas in fast-flow (arterial) lesions, with greater sensitivity than MRA [38]. Before an invasive procedure such as digital subtraction angiography is performed, it is best to have a narrowed differential diagnosis (usually requires prior MRI/MRA to suggest the diagnosis of a fast-flow VM). Diagnostic angiography may confirm the suspicion of a fast-flow VM if this remains in question following MRI/MRA but is typically reserved for symptomatic patients when simultaneous treatment is a leading consideration.


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. CT Area of Interest With IV Contrast Contrast-enhanced CT may provide further anatomic definition of a VM after US in other anatomies, aiding in visualizing phleboliths, thrombus, osseous changes such as erosion or findings related to overgrowth syndromes, and soft tissue involvement, especially in defining the deep or infiltrative extent of a VA [39-41].


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. CT Area of Interest Without and With IV Contrast There is insufficient literature reviewing the efficacy of CT area of interest without and with IV contrast in this clinical scenario.


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. CT of the chest without and with IV contrast has been used for diagnosis in patients with pulmonary arteriovenous malformations (PAVMs), which are seen in children with hereditary hemorrhagic telangiectasia (HHT), as a follow- up examination for the depiction of precise anatomy of simple and some complex lesions, after initial screening, which may be performed using transthoracic contrast echocardiography, which confirms the presence of a suspected shunt [42]. Both phases of the CT examination may provide important information in the diagnosis of PAVM [42].


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. Contrast-enhanced CT may provide further anatomic definition of a VM after US in other anatomies, aiding in visualizing phleboliths, thrombus, osseous changes such as erosion or findings related to overgrowth syndromes, and soft tissue involvement, especially in defining the deep or infiltrative extent of a VA [39-41].


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. CT Area of Interest Without IV Contrast There is no relevant literature reviewing the efficacy of noncontrast enhanced CT evaluation of VM. CT without IV contrast is limited in diagnosis of soft tissue and solid organ VMs and VTs because all structures and tissues show homogenous and similar attenuation, which obscures findings or does not allow characterization of the extent of abnormalities.


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. CTA and CTV Area of Interest With IV Contrast CTA and CTV of the upper extremity was shown by Henzler et al [44] to be the superior imaging modality compared with MRI and US because it provides images with high spatial resolution for excellent delineation of the anatomy and extent of VTs and VMs, providing a vascular map of the lesion for use in treatment planning.


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. Soft Tissue Vascular Anomalies-Child CTA of the chest, using a modified pulmonary CTA and CTV protocol, is used to optimally show the feeding artery, nidus, and draining vein components of the PAVM [42].


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. CTA and CTV may provide further anatomic definition of a VM after US in other anatomies, aiding in visualizing phleboliths, thrombus, osseous changes such as erosion or findings related to overgrowth syndromes, and soft tissue involvement, especially in defining the deep or infiltrative extent of a VA [39-41,45].


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. MRA and MRV Area of Interest Without and With IV Contrast MRA of the area of interest without and with IV contrast may be used to define venous and arterial anatomy, as well as better differentiate the tissue types involved in the VM. MRA and MRV of the area of interest without IV contrast, using flow-dependent or flow-independent techniques, can be used as well. For example, Relaxation- Enhanced Angiography without Contrast and Triggering, a flow-independent T2-weighted noncontrast-enhanced MRA sequence, has been shown to be effective for defining the anatomy of major feeding arteries and draining veins and correlation with contrast-enhanced MRA and MRV [32,46].


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. Contrast-enhanced 3-D and 4-D dynamic MRA acquisitions are helpful in distinguishing whether flow through the lesion is slow or fast, arterial and venous anatomy, the location of a nidus in an AVM, and the site of vascular fistula in an AVF. Dynamic MRA combined with contrast-enhanced MRI has been shown to have excellent sensitivity (83%) and specificity (95%) in differentiating low-flow from fast-flow VMs [29,47].


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. Dynamic 4-D MRA with IV contrast may be used to detect presence of arteriovenous microshunts in VMs, which have been found to be associated with presence of phleboliths [30].


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. MRI Area of Interest Without and With IV Contrast MRI of the area of interest without and with IV contrast may be used to define the deep and superficial extent of VM using T1-weighted sequences, and T2-weighted images reveal vascular flow voids as well as fluid filled spaces. Areas of signal loss or flow voids are important to document and can help drive the diagnosis (phleboliths versus fast-flow vessels). A well-defined soft tissue mass is not typically identified in AVM. IV contrast shows intense enhancement of involved soft tissues, cyst walls, and/or vascular structures [31].


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. MRI Area of Interest Without IV Contrast There is no relevant literature to support the use of MRI area of interest without IV contrast as the next imaging modality for vascular lesion such as tumor or malformation.


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. Radiography Area of Interest There is no relevant literature to support the use of radiography area of interest as the next imaging modality for VMs [35].


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. US Area of Interest With IV Contrast There is no relevant literature to support the use of US area of interest with IV contrast as the initial imaging modality for patients with vascular lesions such as tumor or malformation [48].


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. Variant 5: Child. Established diagnosis of vascular malformation presenting with new or persistent signs or symptoms. Initial imaging. VMs are a diverse group of lesions, which are difficult to treat, requiring multiple episodes of interventional embolization/sclerotherapy and or surgical intervention over years of treatment. Before and throughout the treatment course, interval imaging may help for monitoring regression of the lesion and planning approach to the next treatment session.


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. Arteriography Area of Interest There is no relevant literature to support the use of arteriography as the initial diagnostic imaging modality for patients with an established diagnosis of a low-flow VM.


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. In patients with a fast-flow VM, angiography is useful to characterize new or persistent signs or symptoms (recurrent bleeding events, ischemic changes to normal tissues, etc) when simultaneous treatment is planned [49].


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. CT Area of Interest With IV Contrast Although contrast-enhanced CT may provide anatomic definition of a VM if there are persistent anatomical questions after MRI/MRA, there is no relevant literature to support the use of CT area of interest with IV contrast


Soft Tissue Vascular Anomalies-Child

Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. Soft Tissue Vascular Anomalies-Child


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. as the initial imaging modality for patients with an established diagnosis of VM presenting with new or persistent signs and symptoms [39].


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. CT Area of Interest Without and With IV Contrast There is no relevant literature to support the use of CT area of interest without and with IV contrast as the initial imaging modality for patients with an established diagnosis of VM presenting with new or persistent signs and symptoms.


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. CT Area of Interest Without IV Contrast There is no relevant literature to support the use of CT area of interest without IV contrast a as the initial imaging modality for patients with an established diagnosis of VM presenting with new or persistent signs and symptoms.


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. CTA and CTV Area of Interest With IV Contrast There is no relevant literature to support the use of CTA and CTV area of interest with IV contrast as the initial imaging modality for patients with an established diagnosis of VM presenting with new or persistent signs and symptoms.


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. MRA and MRV Area of Interest Without and With IV Contrast MRA and MRV of the area of interest without and with IV contrast may be used to update venous and arterial anatomy as well as better differentiate the tissue types involved in the VM. As embolization and sclerotherapy treatments change the vascular channels, MRA can provide updated information.


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. Contrast-enhanced 3-D and 4-D dynamic MRA acquisitions are helpful in assessing flow through the lesion before and after treatment, the location of a new nidus in an AVM, and a new vascular fistula in an AVF. Dynamic MRA and MRV combined with contrast-enhanced MRI has been shown to have excellent sensitivity (83%) and specificity (95%) in differentiating low-flow from fast-flow VMs [29,47]. Embolization and sclerotherapy are expected to change the shunting within these low-flow VMs.


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. MRI Area of Interest Without and With IV Contrast MRI of the area of interest without and with IV contrast may be used to define the deep and superficial extent of VM with expected treatment changes. IV contrast shows intense enhancement of involved soft tissues, cyst walls, and/or vascular structures [31]. Particular attention may need to be paid to imaging findings that may suggest treatment complications (abscess, tissue necrosis, cellulitis, deep vein thrombosis, etc).


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. MRI Area of Interest Without IV Contrast There is no relevant literature to support the use of MRI area of interest without IV contrast as the initial imaging modality for patients with an established diagnosis of VM presenting with new or persistent signs and symptoms.


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. Radiography Area of Interest Radiographs of the area of interest may reveal calcified phleboliths and embolization material within a previously treated VM; these findings may be helpful in choosing a follow-up imaging study (ie, US for suspected occluded deep vein after sclerotherapy). Radiographs may also better characterize imaging artifacts (embolization coils), which may limit the evaluation of residual VM on MRI/US [35].


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. US Area of Interest US area of interest may be useful as an initial imaging modality in patients with an established diagnosis of VM when presenting with new or persistent signs or symptoms. However, US may be limited if there is extensive embolization material present. VM occlusions directly due to embolization versus associated soft tissue swelling from treatment changes can be visualized on grayscale US.


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. US Area of Interest With IV Contrast There is no relevant literature to support the use of US area of interest with IV contrast as the initial imaging modality for patients with an established diagnosis of VM presenting with new or persistent signs and symptoms.


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. It has been shown that the dynamics of blood flow through VM can be determined using contrast-enhanced US, and that differences in blood flow dynamics as measured using time intensity curve analysis can be shown by comparing pretreatment and post-treatment examinations [50].


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. US Duplex Doppler Area of Interest US with duplex Doppler may be helpful as an initial imaging modality in patients with an established diagnosis of VM when presenting with new or persistent signs or symptoms. Doppler US can help distinguish changes following


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. Soft Tissue Vascular Anomalies-Child enhanced US and that differences in blood flow dynamics as measured using time intensity curve analysis can be shown by comparing pretreatment and post-treatment examinations.


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. Supporting Documents The evidence table, literature search, and appendix for this topic are available at https://acsearch.acr.org/list. The appendix includes the strength of evidence assessment and the final rating round tabulations for each recommendation.


Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. For additional information on the Appropriateness Criteria methodology and other supporting documents go to www.acr.org/ac.


Soft Tissue Vascular Anomalies-Child

Soft Tissue Vascular Anomalies Vascular Malformations and Infantile Vascular Tumors Non CNS Child. Soft Tissue Vascular Anomalies-Child


Monitoring Response to Neoadjuvant Systemic Therapy for Breast Cancer. Introduction/Background Neoadjuvant chemotherapy (NAC) is often given before definitive surgical intervention for locally advanced breast cancer, which is defined as a tumor >5 cm with regional and/or metastatic lymph nodes, skin, or chest wall involvement. NAC is also indicated in T2 tumors (2-5 cm) in which lumpectomy might result in substantial cosmetic defect, triple-negative tumors 2 to 5 cm in size even if node-negative, and human epidermal growth factor receptor 2 (HER2)/neu-positive tumors 2 to 5 cm in size even if node-negative. The primary aims of this approach are to 1) reduce tumor burden, thereby permitting breast conservation rather than mastectomy; 2) promptly treat possible metastatic disease, whether or not it is detectable on preoperative staging; and 3) potentially tailor future chemotherapeutic decisions by monitoring in vivo tumor response [1,2]. Although the overall and disease-free survival for women receiving neoadjuvant versus adjuvant chemotherapy are not substantially different, women who do receive neoadjuvant therapy are less likely to undergo mastectomy and are more likely to be treated with breast conservation [1].


Monitoring Response to Neoadjuvant Systemic Therapy for Breast Cancer. Imaging plays a vital role in managing patients undergoing NAC as treatment decisions rely heavily on accurate assessment of response to therapy. Beyond assessing the primary lesion, imaging is used to stage and monitor patients before, during, and after completion of initial therapy, including the axilla and potential distant metastatic sites. Accurate assessment of tumor burden is critical in determining the best management. Imaging plays an important role as clinical breast examination is challenging for primary tumors that are <2 cm in size, have an irregular shape or ill-defined margins, and show necrosis, fibrosis, or fragmentation with treatment [3]. Axillary imaging is increasingly used before, during, and after therapy to monitor response to treatment and help guide surgical management [4]. Most practices define response per Response Evaluation Criteria in Solid Tumors (RECIST) or RECIST 1, which defines complete response (CR) as disappearance of the tumor in its entirety, partial response (PR) as at least 30% decrease in the longest diameter of the tumor compared with pretreatment baseline, progression of disease as at least 20% increase in longest diameter, and stable disease as no change in the tumor size that would qualify as PR or progression of disease on the basis of longest diameter [5]. Pathologic complete response (pCR) is defined as a surgical specimen free of carcinoma following therapy and represents a surrogate endpoint for treatment with pCR predicting improved disease-free survival [1,6].


Monitoring Response to Neoadjuvant Systemic Therapy for Breast Cancer. Although there is a paucity of published data in men and transgender patients diagnosed with breast cancer, in practice, these patients are managed similarly to women.


Monitoring Response to Neoadjuvant Systemic Therapy for Breast Cancer. Special Imaging Considerations There are several single-institution studies that demonstrate contrast-enhanced mammography has comparable sensitivity and specificity to contrast-enhanced MRI in evaluating for residual disease after NAC [7-10]. Therefore, although not widely used in clinical practice, this may be an option for patients who are unable to undergo MRI [7- 10].


Monitoring Response to Neoadjuvant Systemic Therapy for Breast Cancer. aUniversity of California San Francisco, San Francisco, California. bResearch Author, University of California San Francisco, San Francisco, California. cPanel Chair, New York University Grossman School of Medicine, New York, New York. dPanel Vice-Chair, Perelman School of Medicine of the University of Pennsylvania, Philadelphia, Pennsylvania. eBoston Medical Center, Boston, Massachusetts, Primary care physician. fHackensack University Medical Center, Hackensack, New Jersey; American College of Surgeons. gUniversity of North Carolina Hospital, Chapel Hill, North Carolina. hElizabeth Wende Breast Care, Rochester, New York. iUniversity of Wisconsin School of Medicine & Public Health, Madison, Wisconsin. jSanford Health of Northern Minnesota, Bemidji, Minnesota. kUniversity of Washington, Seattle, Washington. lMayo Clinic, Phoenix, Arizona. mLoyola University Chicago, Stritch School of Medicine, Department of Radiation Oncology, Cardinal Bernardin Cancer Center, Maywood, Illinois. nHoag Family Cancer Institute, Newport Beach, California and University of Southern California, Los Angeles, California; Commission on Nuclear Medicine and Molecular Imaging. oSpecialty Chair, Boston University School of Medicine, Boston, Massachusetts.


Monitoring Response to Neoadjuvant Systemic Therapy for Breast Cancer. The American College of Radiology seeks and encourages collaboration with other organizations on the development of the ACR Appropriateness Criteria through representation of such organizations on expert panels. Participation on the expert panel does not necessarily imply endorsement of the final document by individual contributors or their respective organization.


Monitoring Response to Neoadjuvant Systemic Therapy for Breast Cancer. Reprint requests to: publications@acr.org


Monitoring Response to Neoadjuvant Systemic Therapy for Breast Cancer. OR Discussion of Procedures by Variant Variant 1: Adult female or male or transfeminine (male-to-female) or transmasculine (female-to-male). Known breast cancer. Initial determination of tumor size and extent within the breast prior to neoadjuvant chemotherapy. Initial imaging. Digital Breast Tomosynthesis Diagnostic Mammography, ultrasound (US), and MRI are complementary modalities for assessing primary tumor size before treatment because they are reliable tools to determine tumor size at diagnosis [1,11-15]. Mammography and US are the two main modalities for assessing primary tumor size before treatment because they are reliable tools to determine tumor size at diagnosis [11-15]. Mammography is most accurate for ductal and low-grade malignancies and less accurate for invasive lobular cancers and higher-grade lesions [11-16].


Monitoring Response to Neoadjuvant Systemic Therapy for Breast Cancer. Digital breast tomosynthesis (DBT) addresses some of the limitations encountered with standard mammographic views. In addition to planar images, DBT creates thin-section reconstructed images, which decreases the lesion- masking effect of overlapping normal tissue. In the screening setting, some authors found the advantages of DBT to be especially pronounced in patients <50 years of age [17,18], in patients with dense breasts [17,19], and with lesion types including spiculated masses, [20] asymmetries [21], and architectural distortion [22]. DBT is also useful in the diagnostic setting, improving lesion characterization [22-25] in noncalcified lesions compared with conventional mammography.


Monitoring Response to Neoadjuvant Systemic Therapy for Breast Cancer. A prospective study of 166 patients with breast cancer compared digital mammography (DM) to combined DM plus DBT for accuracy of local tumor staging. They demonstrated better accuracy of DM plus DBT for detecting additional ipsilateral and contralateral disease in patients with nondense breasts [26]. A retrospective study of 222 cancers demonstrated that pathologic response to NAC was less likely with the baseline mammographic finding of spiculation [27].


Monitoring Response to Neoadjuvant Systemic Therapy for Breast Cancer. Because of the presence of dense tissue in up to 50% of patients, obscured margins may limit evaluation of the extent of disease [28]. Therefore, mammography or DBT is most often combined with other modalities, such as US or MRI, to guide clinical management.


Monitoring Response to Neoadjuvant Systemic Therapy for Breast Cancer. FDG-PET/CT Skull Base to Mid-Thigh Fluorine-18-2-fluoro-2-deoxy-D-glucose (FDG)-PET/CT has a low sensitivity for detection of primary breast cancer because of the low spatial resolution of the scanners and the relatively low FDG uptake of both invasive lobular cancers and low-grade malignancies [29,30]. As a result, this modality is not routinely used for pretreatment imaging of the primary breast tumor.


Monitoring Response to Neoadjuvant Systemic Therapy for Breast Cancer. Mammography Diagnostic Mammography, US, and MRI are complementary modalities for assessing primary tumor size before treatment because they are reliable tools to determine tumor size at diagnosis [1,11-15]. Mammography is most accurate for ductal and low-grade malignancies and less accurate for invasive lobular cancers and higher-grade lesions [11-16].


Monitoring Response to Neoadjuvant Systemic Therapy for Breast Cancer. DBT addresses some of the limitations encountered with standard mammographic views. In addition to planar images, DBT creates thin-section reconstructed images, which decreases the lesion-masking effect of overlapping normal tissue. In the screening setting, some authors found the advantages of DBT to be especially pronounced in patients <50 years of age [17,18], in patients with dense breasts [17,19], and with lesion types including spiculated masses [20] and asymmetries [21]. DBT can also be useful in the diagnostic setting, improving lesion characterization [22-25] in noncalcified lesions compared with conventional mammography.


Monitoring Response to Neoadjuvant Systemic Therapy for Breast Cancer. Monitoring Response to Neoadjuvant Chemotherapy A prospective study of 166 patients with breast cancer compared DM to combined DM plus DBT for accuracy of local tumor staging. They demonstrated better accuracy of DM plus DBT for detecting additional ipsilateral and contralateral disease in patients with nondense breasts [26]. A retrospective study of 222 cancers demonstrated that pathologic response to NAC was less likely with the baseline mammographic finding of spiculation [27].


Monitoring Response to Neoadjuvant Systemic Therapy for Breast Cancer. MRI Breast Without and With IV Contrast MRI is complementary to mammography and US for assessing tumor size before treatment. MRI permits evaluation of a viable tumor before and after NAC by detecting changes in tumor vascularity [31]. There is substantial evidence to support the routine use of contrast-enhanced MRI to stage, monitor early response, and assess for residual and recurrent disease given the overall high sensitivity and relatively high specificity of this technique [1].


Monitoring Response to Neoadjuvant Systemic Therapy for Breast Cancer. Dynamic contrast-enhanced MRI is a sensitive tool to determine extent of disease in the breast, especially in young patients (<50 years of age), with sensitivity approaching 90% and specificity ranging between 50% and 97% [1,32]. To accurately evaluate for response to NAC, a pretreatment MRI must be obtained to serve as a baseline for comparison. MRI is particularly useful in the assessment of multifocal and multicentric disease, which is often underestimated on both mammography and US [28]. In fact, multifocal and multicentric disease are detected in up to 16% of patients on staging MRI according to a study by Houssami et al [33].


Monitoring Response to Neoadjuvant Systemic Therapy for Breast Cancer. A prospective study of 216 patients demonstrated that size determination on MRI was superior to clinical examination in predicting pathologic response both before, during, and after completion of NAC [31]. The enhancement pattern on the pretreatment MRI also indicates how reliable this technique will be in evaluating response. Nonmass enhancement on the pretreatment MRI has been shown to reveal a scattered cell pattern more commonly on posttreatment imaging, thereby making assessment of residual disease more difficult [34]. However, when a mass with well-defined margins is seen, MRI can more accurately predict the amount of residual disease on posttreatment imaging [34]. In addition, several studies have shown that MRI is more accurate than mammography and US in defining disease extent for invasive lobular cancer [32,35,36]. MRI can reliably assess the chest wall because pectoral or intercostal muscle enhancement correlates well with invasion [37]. Finally, several studies have shown that up to 3% of patients have unsuspected contralateral disease at the time of initial diagnosis and MRI has been proven effective in detecting such contralateral disease [38].


Monitoring Response to Neoadjuvant Systemic Therapy for Breast Cancer. MRI Breast Without IV Contrast A small study of 71 patients with MRI before and after treatment found no significant difference in lesion size interpretation on unenhanced versus enhanced MRI sequences [39].


Monitoring Response to Neoadjuvant Systemic Therapy for Breast Cancer. However, there is insufficient literature to support the use of MRI without intravenous (IV) contrast in initial imaging evaluation of tumor size and extent in the breast before NAC.


Monitoring Response to Neoadjuvant Systemic Therapy for Breast Cancer. Sestamibi MBI A few institutions routinely image newly diagnosed breast cancer with molecular breast imaging (MBI) using Tc- 99m sestamibi, which is also sometimes referred to as scintimammography. This functional imaging technique reflects cell metabolism by accumulating in active mitochondrial cells.


Monitoring Response to Neoadjuvant Systemic Therapy for Breast Cancer. A prospective study of 90 patients found the longest dimension of the cancer measured on MRI was within 1 cm of that on MBI in 72% of cases and concluded that MBI may be an option for patients with contraindication to MRI [40,41].


Monitoring Response to Neoadjuvant Systemic Therapy for Breast Cancer. However, there is insufficient literature to support the routine use of sestamibi MBI in initial imaging evaluation of tumor size and extent in the breast before NAC.


Monitoring Response to Neoadjuvant Systemic Therapy for Breast Cancer. US Breast Mammography, US, and MRI are complementary modalities for assessing primary tumor size before treatment because they are reliable tools to determine tumor size at diagnosis [1,11-15]. US is more accurate in measuring tumor size than clinical breast examination or mammography. It is most often performed in conjunction with mammography and is more accurate in assessing tumor size [16,42].


Monitoring Response to Neoadjuvant Systemic Therapy for Breast Cancer. Monitoring Response to Neoadjuvant Chemotherapy Variant 2: Adult female or male or transfeminine (male-to-female) or transmasculine (female-to-male). Known breast cancer. Imaging of the breast after initiation or completion of neoadjuvant chemotherapy. Initial imaging. Digital Breast Tomosynthesis Diagnostic Although mammography, DBT, and US are reliable for determining tumor size at diagnosis [11-15], changes within the tumor secondary to NAC may be difficult to evaluate after treatment is initiated. It is well known that tumoral changes related to necrosis, fragmentation, and fibrosis make it difficult for mammography, DBT, and US to accurately determine residual tumor burden [43,44].