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

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“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 compile and analyze cases of PEComa-NOS in an effort to better define their natural history. Des...

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Malignant Perivascular Epithelioid Cell Neoplasm of the Mediastinum and the Lung

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Malignant TFE3-rearranged perivascular epithelioid cell neoplasm (PEComa) presenting as a subcutaneous mass.

Perivascular epithelioid cell neoplasms (PEComas) are a group of mesenchymal tumours with concurrent melanocytic and myogenic differentiation. Although many cases are sporadic, PEComas can be associated with tuberous sclerosis. A distinct subset of deep-seated PEComas has been shown to carry TFE3 fusions. To our knowledge, this is the first reported case of primary subcutaneous malignant PEComa...

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Malignant Perivascular Epithelioid Cell Neoplasm (PEComa) of the Pelvis: A Case Report

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ژورنال

عنوان ژورنال: Sarcoma

سال: 2012

ISSN: 1357-714X,1369-1643

DOI: 10.1155/2012/541626