title: Perivascular epithelioid cell tumor of the liver coexisting with a gastrointestinal stromal tumor.
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.
authors.affiliation: Oncological and Hemato-oncological Center, Sao Paulo State University, Rua Antonio Nunes da Silva Sobrinho, 180-Jardim Paraiso II-Botucatu, Sao Paulo 18610-170, Brazil. cepaiva@fmb.unesp.br
authors.forename: Carlos Eduardo
authors.initials: CE
authors.lastname: Paiva
authors.forename: Francisco Alves
authors.initials: FA
authors.lastname: Moraes Neto
authors.forename: Abbas
authors.initials: A
authors.lastname: Agaimy
authors.forename: Maria Aparecida
authors.initials: MA
authors.lastname: Custodio Domingues
authors.forename: Silvia Regina
authors.initials: SR
authors.lastname: Rogatto
mesh_terms: D015622:Epithelioid Cells / Q000473:pathology*; D005260:Female; D046152:Gastrointestinal Stromal Tumors / Q000000981:diagnostic imaging / Q000473:pathology*; D006801:Humans; D008113:Liver Neoplasms / Q000000981:diagnostic imaging / Q000473:pathology*; D008875:Middle Aged; D016609:Neoplasms, Second Primary / Q000000981:diagnostic imaging / Q000473:pathology*; D011859:Radiography