Case Report
Copyright ©2008 The WJG Press and Baishideng. All rights reserved.
World J Gastroenterol. Feb 7, 2008; 14(5): 800-802
Published online Feb 7, 2008. doi: 10.3748/wjg.14.800
Perivascular epithelioid cell tumor of the liver coexisting with a gastrointestinal stromal tumor
Carlos Eduardo Paiva, Francisco Alves Moraes Neto, Abbas Agaimy, Maria Aparecida Custódio Domingues, Silvia Regina Rogatto
Carlos Eduardo Paiva, Oncological and Hemato-oncological Center, São Paulo State University, Botucatu, São Paulo 18610-170, Brazil
Francisco Alves Moraes Neto, Department of Pathology, Amaral Carvalho Hospital, Jaú, São Paulo, Brazil
Abbas Agaimy, Institute of Pathology, Nuremberg Clinic Center, Nuremberg, Germany
Maria Aparecida Custódio Domingues, Department of Pathology, São Paulo State University, Botucatu, São Paulo 18610-170, Brazil
Silvia Regina Rogatto, Department of Urology, São Paulo State University, Botucatu, São Paulo 18610-170, Brazil
Correspondence to: Dr. Carlos Eduardo Paiva, Oncological and Hemato-oncological Center, São Paulo State University, Rua Antônio Nunes da Silva Sobrinho, 180-Jardim Paraíso II-Botucatu, São Paulo 18610-170, Brazil. cepaiva@fmb.unesp.br
Telephone: +55-14-38143640
Fax: +55-14-38156044
Received: August 15, 2007
Revised: November 29, 2007
Published online: February 7, 2008
Abstract

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.

Keywords: Gastrointestinal stromal tumors; PEComa; Coexistence; Liver; Synchronous