Abstract

The histological features of mesenchymal chondrosarcoma was first described by Lichtenstein and Bernstein (1959), consist of primitive undifferentiated mesenchymal cells in which islands of cartilage are noted. To date, about 100 cases of mesenchymal chandrosarcoma have been described, 67 per cent occured in bone and 33 per cent were extraskeletal. Mesenchymal chondrosarcoma of the orbit is a exceedingly rare tumor. Seven mesenchyma1 chondrosarcoma of the orbit have been described in the literature. It characteristically occurs in young females and presents with proptosis and pain. The diagnosis is made on the distinctive histologic characteristics and not on the clinical findings. The tumor is composed of rich cellular and undifferentiated mesenchymal cells and well-differentiated cartilage. The treatment is exenteration. We report a case of 22-year old female patient with mesenchymal chondrosarcoma of the orbit. The treatment in this case described was exenteration followed by X-ray therapy.