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 10-year-old boy presented with bloody rhinorrhea of one month duration from the right side of the nasal cavity. On nasal endoscopy, a large, polypoid mass was observed to fill the right nasal cavity causing deviation of the lateral nasal wall to the right. CT showed a lobulated, low attenuating mass with ring and arc calcifications in the right nasal cavity and nasal septum, measuring about 4.9 ×3.3 ×2.9 cm (). The mass extended beyond the nasal septum into the right ethmoid sinus and had caused pressure erosion of the adjacent bony margins of the cribriform plate and medial orbital wall. However, no definite bony destruction was demonstrated. After intravenous infusion of contrast medium, subtle internal septal enhancement of the mass was observed. Coronal and axial T2-weighted MR images (TR/TE, 4128/100) revealed more accurately the anatomical relationships of the mass (), which was located in the right nasal cavity and nasal septum, and extending into the right ethmoid sinus. The mass had displaced and compressed the right medial orbital wall laterally and the cribriform plate superiorly, and mass was homogeneously hypointense on T1-weighted images () and heterogeneously hyperintense on T2-weighted images (). It showed mainly peripheral and septal-like enhancement on contrast-enhanced T1-weighted images and regional inhomogeneously strong enhancement at its anterosuperior portion (). Areas of low signal intensity were seen on T2-weighted MR images (), which corresponded to matrix mineralization on CT images (). An endoscopic biopsy was performed in the nasal cavity, and the preliminary pathologic diagnosis was of myxoid chondrosarcoma or chordoma, due to the presence of a cord-like cellular arrangement with a myxoid background. Whole body PET-CT with a low-dose CT protocol was performed at the 17th day after biopsy. PET-CT revealed a mild F18-fluorodeoxyglucose (FDG) uptake area (pSUV, peak standardized uptake value = 2.7) in the lesion without a definite metastatic lesion ().\nThe patient underwent subtotal resection of the mass; some residual mass remained in the ethmoid sinus and nasal septum. The removed tumor was a pale gray, soft to firm, polypoid mass measuring 4.7 ×3.0 ×3.0 cm. Histologically, there were scattered pleomorphic chondroblasts with thin anastomosing strands surrounded by a myxoid stroma; cellular areas were arranged in cords (). The presence of a characteristic cord-like cellular arrangement resembling chordoma and strong positivity for S-100 protein and vimentin led to a final diagnosis of myxoid chondrosarcoma. The low cellular atypia, few mitoses, and low cellularity observed represented a low grade tumor. We could not prove the presence of the specific t (9; 26) translocation which is regarded as another pathognomonic feature of myxoid chondrosarcoma because the RT-PCR technique and primer required were unavailable.\nAfter surgery, the patient underwent radiation therapy on the remaining primary tumor, and subsequently the remaining tumor showed a decreased size and enhancement on follow up CT.