Atypical Lipomatous Tumor/Well-Differentiated Liposarcoma of the Orbit: Three Cases and Review of the Literature. (May 2021)

• Record Type: Journal Article
• Authors: Kang, Julia Y.; Kim, Hee Joon; Wojno, Ted H.; Yeung, Aaron M.; Mendoza, Pia R.; Grossniklaus, Hans E.
• Is Part Of: Ophthalmic plastic and reconstructive surgery. Volume 37(2021)Supplement 3S
• Publication Date: 2021-05
• Subjects: Eye -- Surgery -- Periodicals; Ophthalmic plastic surgery -- Periodicals; Reconstructive Surgical Procedures -- Periodicals; Ophthalmologic Surgical Procedures -- Periodicals; Ophthalmology -- Periodicals; Surgery -- Periodicals; 617.70592
• DOI: 10.1097/IOP.0000000000001804
• Languages: English
• ISSNs: 0740-9303

Abstract

Abstract : The authors present 3 patients from this retrospective case series to review the clinical findings, imaging, pathology, and treatment of orbital atypical lipomatous tumor/well-differentiated liposarcoma. Pathology of biopsy specimens ranged from spindle cell proliferations mimicking neurofibroma to proliferations of well-differentiated adipocytes. Immunohistochemical stains were positive for murine double minute 2 in 1 case, and fluorescent in situ hybridization showed amplification of murine double minute 2 in 2 cases. Treatments ranged from serial debulking, proton beam irradiation, and exenteration. None of the patients developed metastases. A literature review supported the low-grade nature of this lesion. Orbital atypical lipomatous tumor/well-differentiated liposarcoma is a low-grade, indolent liposarcoma that may be locally invasive. The histologic diagnosis is enhanced with immunohistochemical staining for murine double minute 2 and fluorescent in situ hybridization analysis for amplification of murine double minute 2. Although treatment may vary according to the individual, conservative therapies may be attempted prior to radical surgery. Abstract : Atypical lipomatous tumor/well-differentiated liposarcoma of the orbit is a low-grade, indolent lipomatous tumor that may be managed with conservative therapies prior to exenteration.