Mitteldorf CATDS, Birolini D, Camara-Lopes LHD. A perivascular epithelioid cell tumor of the stomach: An unsuspected diagnosis. World J Gastroenterol 2010; 16(4): 522-525 [PMID: 20101783 DOI: 10.3748/wjg.v16.i4.522]
Corresponding Author of This Article
Cristina Aparecida Troques da Silveira Mitteldorf, MD, PhD, Senior Pathologist, Laboratory of Surgical and Molecular Pathology, Hospital Sírio Libanês, rua Dona Adma Jafet 91, São Paulo, SP 01308-050, Brazil. crismitteldorf@ig.com.br
Article-Type of This Article
Case Report
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World J Gastroenterol. Jan 28, 2010; 16(4): 522-525 Published online Jan 28, 2010. doi: 10.3748/wjg.v16.i4.522
A perivascular epithelioid cell tumor of the stomach: An unsuspected diagnosis
Cristina Aparecida Troques da Silveira Mitteldorf, Dario Birolini, Luis Heraldo da Camara-Lopes
Cristina Aparecida Troques da Silveira Mitteldorf, Luis Heraldo da Camara-Lopes, Laboratory of Surgical and Molecular Pathology, Hospital Sírio Libanês, rua Dona Adma Jafet 91, São Paulo, SP 01308-050, Brazil
Dario Birolini, Department of Surgery of the University of São Paulo Medical School, av. Dr. Enéas de Carvalho Aguiar 255, 05403-000, São Paulo, SP, Brazil, and Hospital Sírio Libanês, rua Dona Adma Jafet 91, São Paulo, SP 01308-050, Brazil
Luis Heraldo da Camara-Lopes, Genoa Biotechnology S.A., Alameda Ministro Rocha Azevedo, 346, 1st floor, 01410-000, São Paulo, SP 01308-050, Brazil
Author contributions: Mitteldorf CATS, Camara-Lopes LH and Birolini D contributed equally to this work; Birolini D is the surgeon responsible for the patient; Mitteldorf CATS reviewed the literature and wrote the manuscript advised by Camara-Lopes LH.
Correspondence to: Cristina Aparecida Troques da Silveira Mitteldorf, MD, PhD, Senior Pathologist, Laboratory of Surgical and Molecular Pathology, Hospital Sírio Libanês, rua Dona Adma Jafet 91, São Paulo, SP 01308-050, Brazil. crismitteldorf@ig.com.br
Telephone: +55-11-31550556 Fax: +55-11-32312249
Received: July 29, 2008 Revised: December 14, 2009 Accepted: December 21, 2009 Published online: January 28, 2010
Abstract
Perivascular epithelioid cell tumor (PEComa) is a rare mesenchymal neoplasia and currently well recognized as a distinct entity with characteristic morphological, immunohistochemical and molecular findings. We report a case of PEComa arising in the antrum of a 71-year-old female with melena. The tumor, located predominantly in the submucosa as a well delimited nodule, measured 3.0 cm in diameter and was completely resected, with no evidence of the disease elsewhere. Histologically, it was composed predominantly of eosinophilic epithelioid cells arranged in small nests commonly related to variably sized vessels, with abundant extracellular material, moderate nuclear variation and discrete mitotic activity. No necrosis, angiolymphatic invasion or perineural infiltration was seen. Tumor cells were uniformly positive for vimentin, smooth muscle actin, desmin and melan A. Although unusual, PEComa should be considered in the differential diagnosis of gastric neoplasia with characteristic epithelioid and oncocytic features and prominent vasculature.
