Orbital mesenchymal chondrosarcoma: Report of 3 cases

Purpose: To describe three cases of extras! cictal mcscnchymal chondrosarcoma (EMC). Melh_ods: All patients with the diagnosis of EMC were selected from the NYEEI surgical pathologs files (1989-I996). Medical records, pathologic specimens, and diagnostic images were reviewed Results: Case 1 was an 11 yr old Caucasian female who presented with an orbital apex mass found incidentally on ST after orbital trauma The patient underwent excisional biopsy, followed by radiation and chemotherapy. She died of recalcitrant pneiimonitis. Case 2 was a 25 yr old Asiai female presenting with cpistaxis, hypcrptyalism and palpable swelling of the medial orbit. CT and MRI studies revealed a mass of (he right orbit, extending into the ethmoid sinuses, left orbit and anterior cranial fossa. The patient underwent bifrontal craniotomy and cranio.-'acial resection of the tumor followed by radiation therapy She shows no recurrence 1 5 yr <ftcr treatment. Case 3 was a 15 yr old Hispanic male who picscnted with proptosis and gobe displacement CT scans revealed a partialK calcified superopostcrior mass. Excisional >iopsy was performed, radiation therapy followed. The patient has no sign of recurrence <fter 1 year Conclu s ions Orbital mesenchymal cliondrosarcoma is an exceedingly rare n alignant neoplasm, previously reported to occur predominantly in young females (19-34 yn). Histologically, the tumor displays a bimorphic appearance, and the differential diagnosis includes hemangiopenc\loma, chondro-sarcoma. meningioma. soft tissue round cell sarcomis. neunnoma, calcified granulomatous infections and métastases The predominant pattern is the spindle shaped cell mesenchymal pattern; scattered regions of cartilaginous stroma in which are embedded islands of malignant chondrocytcs allow the diagnosis of EMC, These cells exhibit S-100 protein by immiinoliistochcinistry Two of our cases studied by i m mnnohi stochern is try exhibited this sunning The pathologist should be aware f this malignant spindle cell neoplasm of the orbit which often disph\s minimal chondroid dif eroitiation Long term tblow-up may be essential due to the protracted clinical course seen in patient with EMC from other sites.