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Orbital Atypical Lipomatous Tumor/Well-Differentiated Liposarcoma Masquerading as Pleomorphic Lipoma

A Diagnostic Challenge

McKey, Kyle, M.D.*; Zhang, Paul J. L., M.D.; Shields, Carol L., M.D.*,‡; Lally, Sara E., M.D.*,‡; Eagle, Ralph C. Jr, M.D.*,§; Milman, Tatyana, M.D.*,§

Ophthalmic Plastic & Reconstructive Surgery: May/June 2019 - Volume 35 - Issue 3 - p e76–e80
doi: 10.1097/IOP.0000000000001378
Case Reports
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Atypical lipomatous tumor/well-differentiated liposarcoma is a common neoplasm of the superficial and deep soft tissues of the extremities, trunk, and retroperitoneum. Atypical lipomatous tumor/well-differentiated liposarcoma is very rare in the orbit, with only 19 previously reported cases. The authors describe a 22-year-old woman who presented with an 8-month history of diplopia and was found to have an orbital mass on MRI. The excised tumor initially was interpreted as spindle cell/pleomorphic lipoma based on its morphologic and immunohistochemical features. Nine years later, the patient returned with a recurrence that required surgical debulking. Histopathologic and molecular cytogenetic evaluation of both primary and recurrent lesions disclosed Atypical lipomatous tumor/well-differentiated liposarcoma. This case highlights the diagnostic challenges and the importance of molecular genetic studies in evaluation of fatty orbital tumors.

This report describes a 22-year-old woman with an atypical lipomatous tumor of the orbit, initially diagnosed as spindle cell lipoma, and discusses the role of molecular genetics in distinguishing between these lesions.

*Department of Ophthalmology, Wills Eye Hospital, Thomas Jefferson University

Department of Pathology and Laboratory Medicine, Hospital of the University of Pennsylvania, Perelman School of Medicine

Ocular Oncology Service

§Pathology Department, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania, U.S.A.

Accepted for publication March 1, 2019.

The authors have no financial or conflicts of interest to disclose.

Ethics: The Institution Ethics Review Committee approval was waved for this retrospective case report study. The study was performed in compliance with the tenets of Declaration of Helsinki.

Address correspondence and reprint requests to Tatyana Milman, M.D., Departments of Ophthalmology and Pathology, Wills Eye Hospital and Thomas Jefferson University Hospital, 840 Walnut Street, Suite 1410, Philadelphia, PA 19107. E-mail: tmilman@willseye.org

© 2019 by The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc., All rights reserved.