Massive Simultaneous Hepatic and Renal Perivascular Epithelioid Cell Tumor Benefitted from Surgery and Everolimus Treatment: A Case Report

Overview

Journal World J Gastrointest Surg

Publisher Baishideng Publishing Group

Specialty Gastroenterology

Date 2024 Nov 22

PMID 39575269

Authors

Han-Teng Yang

Fu-Rong Wang

Na He

Yuan-Hua She

Yong-Yue Du

Wen-Gui Shi

Jing Yang

Gang Chen

Shu-Ze Zhang

Feng Cui

Bo Long

Ze-Yuan Yu

Jun-Min Zhu

Geng-Yuan Zhang

Affiliations

Soon will be listed here.

Abstract

Background: Perivascular epithelioid cell tumor (PEComa) is a rare mesenchymal neoplasm that predominantly affects the kidney and uterus. The occurrence of this tumor in the liver, particularly with simultaneous involvement of the liver and kidney, is exceedingly uncommon. Pathological diagnosis is the gold standard. PEComas usually show positive immunohistochemical staining for melanocytic (HMB-45, Melan-A) and myoid (SMA, muscle-specific actin) markers.

Case Summary: We presented a noteworthy case of malignant PEComa affecting both the liver and kidney in a 53-year-old man with tuberous sclerosis complex (TSC). FAT2 and TP73 mutations in the kidney were identified and positive expression of diagnostic markers including HMB-45, Melan A, and TFE3 were detected. In addition, we demonstrated that hepatic artery perfusion chemotherapy was ineffective for hepatic PEComa, while surgery remained the most effective approach. Everolimus showed an excellent efficacy in the postoperative treatment of the tumor.

Conclusion: Surgical treatment is preferred for malignant PEComa affecting liver and kidney, especially with TSC; everolimus is effective postoperatively.

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