Primary orbital liposarcoma:Clinicopathologic case report and review of the literature

A primary orbital liposarcoma was diagnosed in a 15-year old girl who complained of a rapidly progressing proptosis. An excisional biopsy of the tumor by neurosurgical approach was followed by radiation therapy and sequential chemotherapy during 18 months. A recurrence then occurred leading to orbital exenteration and again radiotherapy. The patient died of widespread metastases less than 30 months after the onset of the disease.Histologically, the tumor was composed of spindle-shaped prelipoblastic and lipo-blastic cells, round vacuolated pre-adipocytic cells and large differentiated adipocytic cells. These features, associated with an edematous stroma, led to the diagnosis of myxoid liposarcoma. Electron microscopy disclosed basement membrane formation, pinocytic vesicles and intracytoplasmic lipidic inclusions without peripheral membrane.No more than 15 cases of orbital liposarcomas were reported in the literature during the past 20 years. Primary orbital liposarcomas differ from extraorbital liposarco...