Massive simultaneous hepatic and renal perivascular epithelioid cell tumor benefitted from surgery and everolimus treatment:A case report  

作　　者：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 

机构地区：[1]Department of General Surgery,Lanzhou University Second Hospital,Lanzhou 730030,Gansu Province,China [2]Department of Pathology,Lanzhou University Second Hospital,Lanzhou 730030,Gansu Province,China [3]Oncology Department Ward,Lanzhou University Second Hospital,Lanzhou 730030,Gansu Province,China [4]Cuiying Biomedical Research Center,Lanzhou University Second Hospital,Lanzhou 730030,Gansu Province,China

出　　处：《World Journal of Gastrointestinal Surgery》2024年第10期3334-3342,共9页世界胃肠外科杂志（英文）

基　　金：Supported by Cuiying Scientific and Technological Innovation Program of Lanzhou University Second Hospital,No.CY2022-QN-A13 and No.CY2023-QN-A02;Lanzhou Science and Technology Plan Project,No.2023-4-26;Gansu Provincial Natural Science Foundation Project,No.23JRRA1630 and No.24JRRA331;Gansu Provincial Health Research Program Project,No.GSWSKY-2019-32.

摘　　要：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.

关 键 词：Perivascular epithelioid cell tumor HEPATIC RENAL SURGERY Case report 

分 类 号：R735.7[医药卫生—肿瘤]