c-KIT positive malignant gastrointestinal stromal tumor in a male child: A rare case

Abstract Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the gastrointestinal tract, representing 1–3% of gastrointestinal malignancies. The majority of GISTs present at age 50–70 years. Pediatric GISTs are considered to be biologically distinct from adult GISTs. The incidence of malignant GISTs is less in children than in adults. Significant clinical and genetic differences are also noted between the two. A pediatric case typically affects females; is multifocal; is sited more commonly in the stomach and follows an indolent course. Histologically, epithelioid morphology is common and genetically most tumors lack mutations in the KIT or PDGFRA genes. We report a 12 year old male child presenting with a highly vascular stomach GIST for whom we did a pylorus-preserving wide local excision. On histopathology it was a malignant variety of GIST of epithelioid type; positive for c-KIT & CD34. The boy is now on Imatinib Mesylate chemotherapy and is asymptomatic.