“Malignant” Perivascular Epithelioid Cell Neoplasm : Risk Stratification and Treatment Strategies

Purpose. Perivascular epithelioid cell tumors (PEComas) are a rare collection of tumors characterized by a myomelanocytic phenotype, and PEComas occurring in “nonclassic” anatomic distributions are known as perivascular epithelioid cell tumor not otherwise specified (PEComa-NOS). This review aims to...

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Bibliographic Details
Published in:Sarcoma 2012, Vol.2012 (2012), p.1-12
Main Authors: Bleeker, Jonathan S., Quevedo, J. Fernando, Folpe, Andrew L.
Format: Article
Language:English
Online Access:Get full text
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Summary:Purpose. Perivascular epithelioid cell tumors (PEComas) are a rare collection of tumors characterized by a myomelanocytic phenotype, and PEComas occurring in “nonclassic” anatomic distributions are known as perivascular epithelioid cell tumor not otherwise specified (PEComa-NOS). This review aims to compile and analyze cases of PEComa-NOS in an effort to better define their natural history. Design. We evaluated all 234 cases of PEComa-NOS reported in the English literature, extracting information regarding diagnostic features, treatment approaches, and outcomes. Multivariate analysis of a number of variables evaluable on pathologic review was performed to refine preexisting risk stratification criteria. Outcomes for patients receiving nonsurgical treatment are also reported. Results. Primary tumor size ≥5 cm (P=0.02) and a high (1/50 HPF) mitotic rate (P
ISSN:1357-714X
1369-1643