Please use this identifier to cite or link to this item: http://hdl.handle.net/2440/59689
Citations
Scopus Web of Science® Altmetric
?
?
Type: Journal article
Title: Primary orbital liposarcoma
Author: Madge, S.
Tumuluri, K.
Strianese, D.
Bonavolonta, P.
Wilcsek, G.
Dodd, T.
Selva-Nayagam, D.
Citation: Ophthalmology, 2010; 117(3):606-614
Publisher: Elsevier Science Inc
Issue Date: 2010
ISSN: 0161-6420
1549-4713
Statement of
Responsibility: 
Simon N. Madge, Krishna Tumuluri, Diego Strianese, Paola Bonavolonta, Geoff Wilcsek, Thomas J. Dodd and Dinesh Selva
Abstract: PURPOSE: To describe 6 new cases of primary orbital liposarcoma and provide a review of the relevant literature. DESIGN: Noncomparative consecutive case series and literature review. PARTICIPANTS: Six patients with primary orbital liposarcoma. METHODS: Review of patient charts, imaging, and histopathology; literature review. MAIN OUTCOME MEASURES: Patient demographics; clinical presentations; results of radiologic imaging; histopathology; surgical techniques used and their complications; other treatment modalities; outcomes and recurrences. RESULTS: 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. CONCLUSIONS: 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.
Keywords: Humans; Liposarcoma; Orbital Neoplasms; Neoplasm Recurrence, Local; Tomography, X-Ray Computed; Magnetic Resonance Imaging; Orbit Evisceration; Adult; Female; Male; Young Adult
Rights: Copyright © 2010 American Academy of Ophthalmology Published by Elsevier Inc.
RMID: 0020100030
DOI: 10.1016/j.ophtha.2009.08.017
Description (link): http://www.elsevier.com/wps/find/journaldescription.cws_home/620418/description#description
Appears in Collections:Opthalmology & Visual Sciences publications

Files in This Item:
There are no files associated with this item.


Items in DSpace are protected by copyright, with all rights reserved, unless otherwise indicated.