Primary malignant gastric PEComa - Diagnostic and technical dilemmas

Area of Science:

• Oncology
• Gastroenterology
• Pathology

Background:

• Perivascular Epithelioid Cell (PEComa) tumors are rare mesenchymal neoplasms.
• Defined by the World Health Organization, they exhibit distinctive perivascular epithelioid cell differentiation.
• PEComas commonly occur in visceral and abdominopelvic locations, with a general tendency towards a benign course and female predominance.

Purpose of the Study:

• To present a rare case of malignant primary gastric PEComa.
• To highlight the diagnostic and management challenges associated with this aggressive tumor subtype.
• To underscore the potential for aggressive behavior in PEComas, contrasting with their typical presentation.

Main Methods:

• A case report of a 42-year-old male presenting with upper gastrointestinal bleeding and an epigastric mass.
• Diagnostic workup included CT scan of the abdomen, upper GI endoscopy with biopsy, and histological analysis.
• Surgical intervention involved a distal Polya gastrectomy and gastrojejunostomy.

Main Results:

• A primary malignant PEComa arising from the gastric wall was diagnosed.
• The patient presented with symptoms suggestive of advanced disease and underwent surgical resection.
• This represents the first reported case of a malignant gastric PEComa.

Conclusions:

• Malignant gastric PEComa can exhibit an aggressive clinical course, contrary to the generally benign nature of most PEComas.
• Accurate diagnosis and effective management pose significant challenges due to limited clinical data and studies.
• The case emphasizes the importance of considering rare diagnoses and the potential for aggressive behavior even in tumors typically considered benign.