J Pathol Transl Med.  2017 Jul;51(4):428-432. 10.4132/jptm.2016.09.16.

Perivascular Epithelioid Cell Tumor in the Stomach

• Shin SA
• Choi J
• Moon KC
• Kim WH

• KMID: 2392585
• DOI: http://doi.org/10.4132/jptm.2016.09.16

Abstract

Perivascular epithelioid cell tumors or PEComas can arise in any location in the body. However, a limited number of cases of gastric PEComa have been reported. We present two cases of gastric PEComas. The first case involved a 62-year-old woman who presented with a 4.2 cm gastric subepithelial mass in the prepyloric antrum, and the second case involved a 67-year-old man with a 5.0 cm mass slightly below the gastroesophageal junction. Microscopic examination revealed that both tumors were composed of perivascular epithelioid cells that were immunoreactive for melanocytic and smooth muscle markers. Prior to surgery, the clinical impression of both tumors was gastrointestinal stromal tumor (GIST), and the second case was erroneously diagnosed as GIST even after microscopic examination. Although gastric PEComa is a very rare neoplasm, it should be considered in the differential diagnosis of gastric submucosal lesions.

Keyword

Perivascular epithelioid cell neoplasms; Stomach neoplasms; HMB-45 protein, human; MART-1 antigen; Gastrointestinal stromal tumors

MeSH Terms

Aged
Diagnosis, Differential
Epithelioid Cells*
Esophagogastric Junction
Female
Gastrointestinal Stromal Tumors
Humans
MART-1 Antigen
Middle Aged
Muscle, Smooth
Perivascular Epithelioid Cell Neoplasms
Stomach Neoplasms
Stomach*
MART-1 Antigen

Figure

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  Fig. 1. Case 1. (A) Abdomen computed tomogragphy shows a 4.2-cm-sized mass (arrow) with broad base in the prepyloric antrum. (B) On gross examination, the mass shows fleshy cut surface with a well-defined border. (C) Microscopically, the tumor displays a nested pattern, and the tumor cells were epithelioid with abundant granular eosinophilic cytoplasm and round nuclei. (D) Immunohistochemistry shows positive staining for human melanoma black 45 (left), smooth muscle actin (upper right), and desmin (lower right).

• 

  Fig. 2. Case 2. (A) The tumor was a subepithelial lesion involving gastroesophageal junction. (B, C) Microscopically, the tumor was composed of perivascular epithelioid cells with heavy lymphoid cell infiltration. (D) Immunohistochemistry reveals positivity for Melan-A (left) and smooth muscle actin (right).

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