Perivascular Epithelioid Cell Tumor of the Ileum Presenting as Diverticulitis

Perivascular epithelioid cell tumors (PEComas) are a group of rare mesenchymal neoplasms. Gastrointestinal PEComas are exceptionally rare, there being only a few case reports in the literature involving the colon and small intestine. Nearly all PEComas show immunoreactivity for both melanocytic (HMB...

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Main Authors: Saime Unluoglu, Umit Bayol, Nilay Korkmaz, Bekir Ozenen, Fuat Ipekci, Emel Ebru Pala
Format: Article
Language:English
Published: Wiley 2012-01-01
Series:Case Reports in Pathology
Online Access:http://dx.doi.org/10.1155/2012/476941
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author Saime Unluoglu
Umit Bayol
Nilay Korkmaz
Bekir Ozenen
Fuat Ipekci
Emel Ebru Pala
author_facet Saime Unluoglu
Umit Bayol
Nilay Korkmaz
Bekir Ozenen
Fuat Ipekci
Emel Ebru Pala
author_sort Saime Unluoglu
collection DOAJ
description Perivascular epithelioid cell tumors (PEComas) are a group of rare mesenchymal neoplasms. Gastrointestinal PEComas are exceptionally rare, there being only a few case reports in the literature involving the colon and small intestine. Nearly all PEComas show immunoreactivity for both melanocytic (HMB45 and/or Melan-A) and smooth muscle (actin and/or desmin) markers. A 36-year-old male was admitted to the hospital with acut- abdomen. At laparatomy, a nodular mass protruding from the ileum which clinically simulated a diverticulitis was noticed. Gross examination of the specimen revealed a 2×1,5×1 cm secondarily ulcerated, solid, nodular, gray white tumor mass in the ileal wall. Histologically, tumor cells were composed of nests of round-polygonal epithelioid cells with abundant clear to slightly eosinophilic granular cytoplasm and round vesicular nuclei. The nests were separated by thin fibrovascular septa. Minimal necrosis and low mitotic activity were noticed in the tumor. Immunohistochemically, tumor cells were positive for SMA, HMB45, and Melan-A and negative for CD10, RCC, CD45, CD117, CD34, EMA, and Desmin. Diagnosis was PEComa of the ileum. We report the case of ileal PEComa to remind the unusual presentation (diverticulitis) of these tumors, besides rarity and diagnostic difficulties.
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spelling doaj-art-7a58c36f47a748cf9b822c7d1e9494282025-02-03T01:21:15ZengWileyCase Reports in Pathology2090-67812090-679X2012-01-01201210.1155/2012/476941476941Perivascular Epithelioid Cell Tumor of the Ileum Presenting as DiverticulitisSaime Unluoglu0Umit Bayol1Nilay Korkmaz2Bekir Ozenen3Fuat Ipekci4Emel Ebru Pala5Department of Pathology, Tepecik Research and Training Hospital, Izmir, TurkeyDepartment of Pathology, Tepecik Research and Training Hospital, Izmir, TurkeyDepartment of Pathology, Tepecik Research and Training Hospital, Izmir, TurkeyDepartment of General Surgery, Tepecik Research and Training Hospital, Izmir, TurkeyDepartment of General Surgery, Tepecik Research and Training Hospital, Izmir, TurkeyDepartment of Pathology, Tepecik Research and Training Hospital, Izmir, TurkeyPerivascular epithelioid cell tumors (PEComas) are a group of rare mesenchymal neoplasms. Gastrointestinal PEComas are exceptionally rare, there being only a few case reports in the literature involving the colon and small intestine. Nearly all PEComas show immunoreactivity for both melanocytic (HMB45 and/or Melan-A) and smooth muscle (actin and/or desmin) markers. A 36-year-old male was admitted to the hospital with acut- abdomen. At laparatomy, a nodular mass protruding from the ileum which clinically simulated a diverticulitis was noticed. Gross examination of the specimen revealed a 2×1,5×1 cm secondarily ulcerated, solid, nodular, gray white tumor mass in the ileal wall. Histologically, tumor cells were composed of nests of round-polygonal epithelioid cells with abundant clear to slightly eosinophilic granular cytoplasm and round vesicular nuclei. The nests were separated by thin fibrovascular septa. Minimal necrosis and low mitotic activity were noticed in the tumor. Immunohistochemically, tumor cells were positive for SMA, HMB45, and Melan-A and negative for CD10, RCC, CD45, CD117, CD34, EMA, and Desmin. Diagnosis was PEComa of the ileum. We report the case of ileal PEComa to remind the unusual presentation (diverticulitis) of these tumors, besides rarity and diagnostic difficulties.http://dx.doi.org/10.1155/2012/476941
spellingShingle Saime Unluoglu
Umit Bayol
Nilay Korkmaz
Bekir Ozenen
Fuat Ipekci
Emel Ebru Pala
Perivascular Epithelioid Cell Tumor of the Ileum Presenting as Diverticulitis
Case Reports in Pathology
title Perivascular Epithelioid Cell Tumor of the Ileum Presenting as Diverticulitis
title_full Perivascular Epithelioid Cell Tumor of the Ileum Presenting as Diverticulitis
title_fullStr Perivascular Epithelioid Cell Tumor of the Ileum Presenting as Diverticulitis
title_full_unstemmed Perivascular Epithelioid Cell Tumor of the Ileum Presenting as Diverticulitis
title_short Perivascular Epithelioid Cell Tumor of the Ileum Presenting as Diverticulitis
title_sort perivascular epithelioid cell tumor of the ileum presenting as diverticulitis
url http://dx.doi.org/10.1155/2012/476941
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