A Liver Transplant for Local Control in a Pediatric Patient with Metastatic TFE3-Associated Perivascular Epithelioid Cell Tumor (PEComa) to the Liver

Whayoung Lee; Josephine HaDuong; Aaron Sassoon; Tuan Dao; Ali Nael

doi:10.1155/2021/3924565

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 Oct 5:2021:3924565. doi: 10.1155/2021/3924565. eCollection 2021.

Authors

Whayoung Lee¹, Josephine HaDuong², Aaron Sassoon³, Tuan Dao⁴, Ali Nael⁵

Affiliations

¹ Department of Pathology, University of California Irvine Medical Center, 101 The City Drive South, Bldg. 1, Room 3003 Orange, CA 92868, USA.
² Pediatric Oncology Department, Children's Hospital of Orange County (CHOC), 1201 West La Veta Ave. Orange, CA 92868, USA.
³ Pathology Department, Children's Hospital of Orange County (CHOC), 1201 West La Veta Ave. Orange, CA 92868, USA.
⁴ Radiology Department, Children's Hospital of Orange County (CHOC), 1201 West La Veta Ave. Orange, CA 92868, USA.
⁵ Pathology Department, Children's Hospital of Orange County (CHOC) and University of California Irvine Medical Center, 1201 West La Veta Ave. Orange, CA 92868, USA.

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.

Publication types

Case Reports