Primary orbital liposarcoma

Abstract

<h4>Purpose</h4>To describe 6 new cases of primary orbital liposarcoma and provide a review of the relevant literature.<h4>Design</h4>Noncomparative consecutive case series and literature review.<h4>Participants</h4>Six patients with primary orbital liposarcoma.<h4>Methods</h4>Review of patient charts, imaging, and histopathology; literature review.<h4>Main outcome measures</h4>Patient demographics; clinical presentations; results of radiologic imaging; histopathology; surgical techniques used and their complications; other treatment modalities; outcomes and recurrences.<h4>Results</h4>Six cases of primary orbital liposarcoma were identified, 5 of which were primary presentations and 1 of which was a recurrence. In 4 cases, exenteration was deferred, resulting in recurrence of disease in all 4. All cases were exenterated, and 2 cases had local recurrence despite exenteration. Two cases were associated with the Li-Fraumeni syndrome and other malignancies. Literature review identified 34 other cases of primary orbital liposarcoma, which, partly because of its rarity, is frequently initially misdiagnosed. The most common subtype is myxoid (56.8%); other types are pleomorphic (10.8%) and well differentiated (29.7%). Well-differentiated tumors have the best prognosis. Non-exenterating surgery was associated with recurrence, although recurrence post-exenteration also occurred. Although radiotherapy has an established role in the treatment of nonorbital liposarcoma, the role of both radiotherapy and chemotherapy in the management of primary orbital liposarcoma is still unclear.<h4>Conclusions</h4>Orbital liposarcoma remains a diagnostic and surgical challenge. Exenteration remains the treatment of choice, but clinicians must also be aware that liposarcoma may herald the diagnosis of the Li-Fraumeni familial cancer syndrome.