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    
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.
Pratibha Iyengar; Dan D Deangelis; Mark Greenberg; Glenn Taylor
Related Documents :
20221596 - Intramuscular castleman's disease of the deltoid: a case report and review of the liter...
6703196 - Ependymoma of the broad ligament. a report of two cases.
7885566 - Carcinoid tumor of the sacrum: case report.
10562366 - Dermatofibrosarcoma protuberans metastasizing to lymph nodes: a case report and review ...
15699086 - Epidemiology of chronic fatigue syndrome.
2682616 - Thyroid neoplasms containing mature fat: a report of two cases and review of the litera...
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    
Department of Pathology, Hospital for Sick Children, 555 University Avenue, Toronto, ON M5G 1X8, Canada.
Export Citation:
APA/MLA Format     Download EndNote     Download BibTex
MeSH Terms
Antigens, Neoplasm
Carcinoma / chemistry,  pathology*,  surgery
Disease-Free Survival
Epithelioid Cells / chemistry,  pathology*
Immunoenzyme Techniques
Neoplasm Proteins / analysis
Orbital Neoplasms / chemistry,  pathology*,  surgery
Treatment Outcome
Tumor Markers, Biological / analysis
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

Previous Document:  Testicular mixed germ cell tumor with polyembryoma component in brothers.
Next Document:  "Proximal-type" and classic epithelioid sarcomas represent a clinicopathologic continuum: case repor...