RESUMEN
BACKGROUND: Mesenchymal chondrosarcoma (MC) is a subtype of chondrosarcoma, with an incidence varying from 1 to 8% of all chondrosarcomas. It is an aggressive neoplasm with a high tendency for late recurrence and occasional delayed distant metastasis. Orbital MC is very rare, and only approximately 30 cases have been described in the literature. We describe here one case of primary orbital MC. CASE REPORT: A 14-year-old boy without a past medical history presented with a 1-month history of progressive proptosis on the right eye. Computed tomography (CT) scans of the orbit revealed a right intraconic lesion, with areas of calcification. The lesion was excised. Histopathological analysis revealed that the tumor had a biphasic pattern, showing a combination of small cell malignancy and well-differentiated cartilage. Immunohistochemistry examination revealed a diffuse membrane expression of CD99 on the small cell malignancy; S-100 was positive only within the cartilage component. The patient received chemotherapy, and no metastatic disease was found at the 2-month follow-up. CONCLUSION: Although rare, MC should be considered in the differential diagnosis of a well-circumscribed orbital lesion in young adults, especially when CT scans reveal areas of calcification within the tumor.