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A Liver Transplant for Local Control in a Pediatric Patient with Metastatic TFE3-Associated Perivascular Epithelioid Cell Tumor (PEComa) to the Liver. Case Rep Pathol 2021;2021:3924565

Date

10/16/2021

Pubmed ID

34650822

Pubmed Central ID

PMC8510831

DOI

10.1155/2021/3924565

Abstract

Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal tumors with widespread distribution throughout the body and unpredictable clinical behavior. Recently, a subset of these tumors has been reported to harbor Transcription Factor E3 (TFE3) gene rearrangement with distinct morphologic and immunophenotypic features. Although limited, these tumors may represent a separate entity from the conventional PEComas and may require different treatment approaches. Surgery is the main treatment option with no clear consensus on systemic therapy. Here, we present the first case of a malignant pediatric colonic TFE3-associated PEComa with isolated liver metastasis leading to liver transplantation for the local control.

Author List

Lee W, HaDuong J, Sassoon A, Dao T, Nael A

Author

Whayoung Lee MD Assistant Professor in the Pathology department at Medical College of Wisconsin