Perivascular epithelioid cell tumor of the liver coexisting with a gastrointestinal stromal tumor

作     者：Carlos Eduardo Paiva Francisco Alves Moraes Neto Abbas Agaimy Maria Aparecida Custódio Domingues Silvia Regina Rogatto 

作者机构：Oncological and Hemato-oncological Center So Paulo State University Botucatu So Paulo 18610-170 Brazil Department of Pathology Amaral Carvalho Hospital Jaú So Paulo Brazil Institute of Pathology Nuremberg Clinic Center Nuremberg Germany Department of Pathology So Paulo State University Botucatu So Paulo 18610-170 Brazil Department of Urology So Paulo State University Botucatu So Paulo 18610-170 Brazil 

出 版 物：《World Journal of Gastroenterology》 (世界胃肠病学杂志（英文版）)

年 卷 期：2008年第14卷第5期

页      面：800-802页

核心收录：

学科分类：1002[医学-临床医学] 100214[医学-肿瘤学] 10[医学] 

基　　金：Fundao para o Desenvolvimento da UNESP-FUNDUNESP  So Paulo  Brazil 

主　　题：胃肠肿瘤 肝脏疾病 症状 患者 

摘      要：Approximately 10% of patients with gastrointestinal stromal tumors (GIST) develop other neoplasms, either synchronously or metachronously. In this report we describe coexistence of a gastrointestinal stromal tumor and a hepatic perivascular epithelioid cell tumor (PEComa) in a 51-year-old woman with no evidence of tuberous sclerosis. A subcapsular hepatic nodule (0.8 cm in diameter) was found during surgery for symptomatic gastric neoplasm (15 cm in diameter) arising from the lesser curvature. Both tumors revealed histomorphological and immunohistochemical features confirming a diagnosis of a small incidental hepatic PEComa and a high risky extramural gastric GIST, respectively. The patient remained disease-free 25 mo after surgery with no evidence of tumor recurrence or new neoplasms. To our knowledge, this is the first report of PEComa in a patient with GIST. Hepatic lesions detected synchronously or metachronously in patients with GISTs may represent histogenetically distinct lesions and should be sampled to confirm or exclude metastatic GISTs.