A 65-year-old male presented with an eight-week history of a left-sided neck mass. The patient described a mass, which was initially the size of a golf ball, but grew rapidly. By the second month, it caused significant pain, dysphagia, and dyspnea, prompting an urgent visit to the hospital. Pertinent physical examination findings at presentation included a firm, large 20 cm mass which occupied the entire left neck. The mass crossed the midline, displacing the trachea to the right side.\nThe patient was admitted and a computed-tomography (CT) scan was performed (Figure ) revealing a large (9.5 cm x 12.9 cm x 21 cm), trans-spatial, infiltrative, mixed solid and cystic mass involving the left carotid, parotid, masticator, parapharygeal, and prevertebral spaces. The tumor extended through the left thyrohyoid membrane and involved the submucosal surface of the oropharynx, hypopharynx, and larynx and crossed the midline via the retropharyngeal space, insinuating between the right thyroid lobe and cricoid cartilage and extended inferiorly into the anterior and superior mediastinum. There was also severe narrowing of the supraglottic airway and rightward deviation of the entire laryngeal axis. No obvious erosion of adjacent osseous structures, hyoid bone, laryngeal cartilage, or tracheal cartilaginous rings was appreciated.\nA tracheostomy tube and a percutaneous feeding tube were placed. Additionally, a biopsy of the mass was performed. This revealed a myxoid sarcoma composed of cords, clusters, and strands of cohesive epithelioid cells within a large amount of myxoid matrix. Pale vesicular nuclei and mitotic figures were readily identified within the cells. Immunohistochemistry (IHC) stains were negative for brachyury, cytokeratin, smooth muscle actin, and S100. Based on the pathologic appearance of the cells and IHC features, the diagnosis of extraskeletal myxoid chondrosarcoma with prominent cytoplasmic vacuolization was established (Figure ).\nThe tumor was considered to be unresectable due to its locally advanced nature. The patient was recommended to receive concurrent chemotherapy and radiation. Informed patient consent was obtained. He was treated with eight weekly cycles of doxorubicin (10 mg/m2) with concurrent image-guided, intensity modulated radiotherapy to a dose of 7000 centigray (cGy) in 35 fractions given over the course of seven weeks (Figure ).\nDuring treatment, he was noted to have a drastic response to therapy, necessitating re-simulation and re-planning of radiotherapy after 4400 cGy (Figure ). At the completion of the concurrent therapy, the patient was noted to have a remarkable clinical response with a significant improvement in speech and swallowing and in tumor size and pain.\nHe tolerated therapy fairly well and completed the planned treatment with no breaks. He experienced the expected side effects of grade 3 mucositis and grade 3 radiation dermatitis (Common Terminology Criteria for Adverse Events version 4.0), which were managed with opioid analgesics, topical ointments, and silver sulfadiazine cream. Near the end of the therapy, he was suspected to have superimposed cellulitis that was successfully treated with antibiotics.\nDuring the course of the concurrent chemoradiotherapy, molecular profiling (Caris Life Sciences, Phoenix, AZ) was performed on the patient’s tumor for biomarkers of drug response. High expression of TLE3 and negative IHC staining for PGP and TUBB3 suggested a potential benefit for docetaxel, paclitaxel, or nab-paclitaxel. IHC staining was negative for ERCC1, suggesting a potential benefit for carboplatin, cisplatin, or oxaliplatin.\nOne month after the completion of therapy, the patient is recovering as expected from the acute toxicity of therapy and is demonstrating a complete clinical response and an excellent radiographic response (Figure ). Further chemotherapy with carboplatin and paclitaxel is planned given the results of molecular profiling.
A 47-year-old Iranian male patient referred to our outpatient clinic in the Imam Khomeini Hospital, Sari, with a palpable and painless mass in his left shoulder.\nThe patient did not complain about pain or weakness in this location. On examination, the mass was small, mobile, firm, and nontender located in the soft tissue of left shoulder. The skin covering the mass was intact, and there was no ecchymosis and even erythema (). The shoulder range of motion had no functional impairment, and the patient was able to do the routine tasks with no neurovascular disturbance. Medically speaking, important clinical signs and symptoms such as fever, weight loss, and respiratory symptoms were not detected.\nThere was no visible fluctuation in this field, and it was estimated 0.5-1 cm in length and width. Tumor consistency was not similar to bone, and it seemed to be a soft tissue mass. The patient denied any history of trauma and also no significant family history was identified. Interestingly, the patient was operated by general surgeon 6 times because of relapses in a way the last one was 1.5 years ago. At the first time, the patient was referred to the outpatient clinic because of gradual mass enlargement. The patient was operated by wrong method as transverse approach in the lateral border of the shoulder ().\nIn the recent recurrence, chest X-ray showed no obvious lung or bone involvement ( and ). Magnetic resonance imaging (MRI) illustrated a small extraskeletal nodular lesion with ring enhancement and no bone involvement at subcutaneous fat on lateral aspect of shoulder measuring about 13 mm which could be due to a recurrence of the previous tumor ( and ). Unfortunately, no previous documentation such as MRI was found. Thus, this caused difficulty for interpretation of the course of the main disease.\nFirst, we decided to operate the tumor as an incisional biopsy because we were not sure about the nature of the lesion. An important note in this approach was limited access to the lesion because of an incorrect previous approach. Pathologic specimen confirmed the diagnosis of chondrosarcoma. Then, he underwent a wide resection of the lesion in the beach-chair position. In surgery, the five dense fibrotic and firm masses measuring from 0.5 to 2 cm and the middle part of deltoid muscle (because of involvement) were excised and sent to pathology ward. Microscopic examination showed infiltrative hypercellular islands of pleomorphic chondrocytes composed of enlarged cells with irregular hyperchromatic nuclei and inconspicuous cytoplasm which was compatible with the diagnosis of chondrosarcoma with free surgical margins ( and ). We should say that the diagnosis was based on the characteristic clinical, radiologic, and pathological criteria; moreover, it was confirmed by immunohistochemistry. Hence, after consulting with the oncologist, radiation therapy was done. We have followed the patient for 6 months after operation, and until now, no recurrence is recognized.