Primary dedifferentiated liposarcoma of the orbit, a rare entity: Case report and review of literature

Abstract

Head and neck liposarcomas are rare entities accounting for less than 5% of all liposarcomas. The primary orbital location is even rarer, with about 40 cases described in the English literature. According with the widely accepted classification of Enzinger and Weis there are 5 histologic variants of liposarcomas: well differentiated, myxoid, dedifferentiated, round cell and pleomorphic. The first two are considered low-grade and display a favourable prognosis (>90% 5-year DSS and OS), whereas the dedifferentiated, round cell, and pleomorphic are defined high-grade and burdened with poorer prognosis (5-year DSS ranging 45-73%). Dedifferentiated liposarcomas (DDL) of the head and neck region are exceedingly rare, therefore there are scattered and contrasting data regarding their clinical history, treatment modality, and prognosis. We presented a case of DDL arising in the the left orbit (fourth case of primary orbital DDL described), free from disease after 5-year follow-up. Clinical history, treatment, and characteristics of the presented case were described and discussed in the light of how reported in the literature, in the attempt to bring further insight in the nature and management of this rare pathological entity.