Document Detail


Primary orbital liposarcoma.
MedLine Citation:
PMID:  20022639     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
Simon N Madge; Krishna Tumuluri; Diego Strianese; Paola Bonavolonta; Geoff Wilcsek; Thomas J Dodd; Dinesh Selva
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Publication Detail:
Type:  Case Reports; Journal Article     Date:  2009-12-22
Journal Detail:
Title:  Ophthalmology     Volume:  117     ISSN:  1549-4713     ISO Abbreviation:  Ophthalmology     Publication Date:  2010 Mar 
Date Detail:
Created Date:  2010-03-01     Completed Date:  2010-03-19     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  7802443     Medline TA:  Ophthalmology     Country:  United States    
Other Details:
Languages:  eng     Pagination:  606-14     Citation Subset:  IM    
Copyright Information:
Copyright 2010 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.
Affiliation:
Discipline of Ophthalmology and Visual Sciences, University of Adelaide, South Australia.
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MeSH Terms
Descriptor/Qualifier:
Adult
Female
Humans
Liposarcoma / pathology*
Magnetic Resonance Imaging
Male
Neoplasm Recurrence, Local / diagnosis*
Orbit Evisceration
Orbital Neoplasms / pathology*
Tomography, X-Ray Computed
Young Adult

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