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

Area of Science:

• Oncology
• Pediatric Pathology
• Gastrointestinal Neoplasms

Background:

• Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal neoplasms with uncertain pathophysiology and clinical behavior.
• Gastrointestinal tract PEComas are exceptional, especially in pediatric populations.
• PEComas exhibit dual melanocytic and smooth muscle differentiation.

Purpose of the Study:

• To present a rare case of gastric PEComa in a 7-year-old girl.
• To provide clinical and diagnostic characterization of pediatric gastric PEComa.
• To raise awareness and discuss differential diagnosis and therapeutic approaches for this rare tumor.

Main Methods:

• Case report of a pediatric patient with hematemesis and anemia.
• Diagnostic workup including imaging studies and digestive endoscopy.
• Surgical resection and pathological anatomy for definitive diagnosis.

Main Results:

• A gastric mass was identified and initially suspected as a gastrointestinal stromal tumor (GIST).
• Definitive pathological diagnosis confirmed the mass as a gastric PEComa.
• The patient presented with symptoms of hematemesis and severe anemia.

Conclusions:

• Gastric PEComa is an extremely rare diagnosis in pediatrics.
• Timely recognition, differential diagnosis, and appropriate therapeutic strategies are crucial.
• This case underscores the importance of considering rare entities in pediatric gastrointestinal pathology.