A 47-year-old man presented to our referral center in November 2017, with a history of EMC of the right shoulder improperly excised with unplanned microscopic positive surgical margins (R1). The patient underwent debulking of the primary mass in September 2017 in a non-referral center, without a preoperative core-needle biopsy. The histological examination diagnosed EMC. No post-operative treatments were provided. At our first evaluation, the patient did not show macroscopic residual disease or distant metastasis both at the clinical and the radiological assessment. The pathological review of the excised specimen confirmed the diagnosis of EMC. After MTB discussion a “watch and wait” approach was adopted. After a 4-months follow-up, the clinical examination showed an elastic soft painless mass in the right shoulder. A Magnetic Resonance Imaging (MRI) of the shoulder was performed, showing a 35 × 15 mm intramuscular solid mass (Fig. ) within the right deltoid muscle. Computed Tomography (CT) did not reveal distant metastasis. The case was discussed in our MTB where the decision was made for preoperative RT, with a total dose of 50 Gy (fractionated in 200 cGy/die) which was a administered with Rapidarc technique. Post radiotherapy MRI performed 3 weeks later showed a partial radiological response, with a mass measuring 16 × 6 mm (Fig. ).\nIn July 2018, a wide surgical resection including the right anterior deltoid muscle and the lateral margin of the right pectoralis major muscle was performed, with a planned macroscopic margin of minimum 3 cm and the complete excision of the scar and the field of the previous surgery. The defect was reconstructed by right latissimus dorsi myocutaneous flap and polypropylene prosthesis (Figs. and ).\nThe size of the excised specimen was 23.5 × 10 × 3.5. Pathological examination was negative for viable tumor cells, showing only a fibrous subcutaneous scar. The patient had a complete response to preoperative RT. After MTB discussion, no further adjuvant treatments were provided. The patient did not show postoperative complications and was discharged on postoperative day 5. Neither relapse or systemic progression occurred in 24-months post-operative follow up. After the surgery the patient wore a shoulder brace for 15 days to avoid damage to the flap. Then a rehabilitation program consisting of 24 sessions (three times a week, lasting 1 h a session) was performed to improve the range of motion of the shoulder and motor performance of the shoulder girdle. No functional deficits were observed 6 months after surgery and at the 24-month follow up. Currently, after 2 years from surgery, at the clinical evaluation, the patient does not show differences in the flexion and abduction of the shoulder between right and left side (Fig. and ) and he confirms that he is leading a normal life without experiencing functional deficit or limitations in his daily life and his professional activities.
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.