Document Detail


Perivascular epithelioid cell tumor of the orbit: a case report and review of the literature.
MedLine Citation:
PMID:  15803216     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
We present a unique case of a perivascular epithelioid cell tumor (PEComa) in the orbit of a 9-year-old female patient. The entity of PEComas has been described only recently. Characteristic histologic features and an immunohistochemical profile of negativity for epithelial markers and positivity for melanogenesis-related markers define the tumors. In children and young adults, this tumor has a predilection for the falciform ligament and ligamentum teres of the liver. It is associated with, but not exclusive to, tuberous sclerosis. To the best of our knowledge, this is the first reported case of a PEComa of the orbit in a child or adult. The main differential diagnoses for this melanin pigment-producing lesion include melanoma and pigmented paraganglioma. The histologic features, immunohistochemical profile, ultrastructural studies, and molecular studies led us to favor a diagnosis of PEComa. The prognosis of this entity is undetermined due largely to the small number of reported cases.
Authors:
Pratibha Iyengar; Dan D Deangelis; Mark Greenberg; Glenn Taylor
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Publication Detail:
Type:  Case Reports; Journal Article     Date:  2004-12-06
Journal Detail:
Title:  Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society     Volume:  8     ISSN:  1093-5266     ISO Abbreviation:  Pediatr. Dev. Pathol.     Publication Date:    2005 Jan-Feb
Date Detail:
Created Date:  2005-04-01     Completed Date:  2005-06-28     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  9809673     Medline TA:  Pediatr Dev Pathol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  98-104     Citation Subset:  IM    
Affiliation:
Department of Pathology, Hospital for Sick Children, 555 University Avenue, Toronto, ON M5G 1X8, Canada.
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MeSH Terms
Descriptor/Qualifier:
Antigens, Neoplasm
Carcinoma / chemistry,  pathology*,  surgery
Child
Disease-Free Survival
Epithelioid Cells / chemistry,  pathology*
Female
Humans
Immunoenzyme Techniques
Neoplasm Proteins / analysis
Orbital Neoplasms / chemistry,  pathology*,  surgery
Treatment Outcome
Tumor Markers, Biological / analysis
Chemical
Reg. No./Substance:
0/Antigens, Neoplasm; 0/MART1 antigen; 0/Neoplasm Proteins; 0/Tumor Markers, Biological; 0/melanoma-specific antigens

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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