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.
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.