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 56-year-old female was admitted in the Department of Oral Maxillofacial Head and Neck Oncology Surgery with a history of painless mass of the left posterior region of the parotid gland. The swelling was slowly progressive over a period of 2 months and there were no associated systemic symptoms. Physical examination revealed a parotid mass 1.5 cm × 2 cm × 2 cm in size, firm in consistency, and with no palpable cervical lymph nodes. The preoperative computed tomography (CT) scan depicted a 4.5 cm × 3.4 cm × 3.2 cm sized irregular lobular mass involving the deep lobe of the left parotid gland. Margins were clear, uneven texture, and the compression of the left internal jugular vein with the forward displacement was observed. The cervical lymph nodes were not enlarged []. The coronal CT demonstrated a soft tissue mass that extended up to the left temporal bone []. Parotid gland pleomorphism adenoma or facial nerve neurilemmoma was the clinical differential diagnosis. Following discussion and after obtaining consent from the patient, left partial parotidectomy with conservation of facial nerve was performed under general anesthesia. During the course of the surgery, the tumor was identified with an intact capsule and jugular vein, and the carotid artery was palpated [Figure –].