Gastric PEComa as a rare cause of upper gastrointestinal bleeding in pediatrics: Case report.

Perivascular epithelioid cell tumors, known as PEComas, are rare mesenchymal neoplasms with dual melanocytic and smooth muscle differentiation, whose pathophysiology and clinical behavior are still being studied. Their occurrence in the gastrointestinal tract is exceptional, particularly in children. We present the case of a 7-year-old girl who consulted for hematemesis and severe anemia. Imaging studies and digestive endoscopy revealed a gastric mass initially suspected to be a gastrointestinal stromal tumor (GIST). Surgical resection was performed, and the definitive diagnosis by pathological anatomy was gastric PEComa. This paper aims to provide a clinical and diagnostic characterization of this extremely rare entity in pediatrics, raise awareness of its timely recognition, reflect on its differential diagnosis, and discuss its therapeutic approach.