Congenital gastrointestinal stromal tumor: A rare case with rarer presentation

Abstract

Gastrointestinal stromal tumors (GISTs) are the most common nonepithelial tumors of the gastrointestinal tract. Adults between fifth and seventh decades of life are most often affected. GIST is rare in pediatric age group, and GIST in neonates is so rare that not much is known about their clinical presentation and behavior. We report a case of congenital GIST in a term male neonate who presented with acute abdomen. The tumor had caused distal ileal obstruction leading to dilatation of the proximal segment associated with perforation peritonitis. The involved intestinal segment was excised, and end-to-end anastomosis was performed; however, the newborn succumbed to postsurgery. The tumor histomorphologically resembled adult GIST with spindle and epithelioid areas. Immunohistochemically, the tumor was positive for vimentin, however was negative for actin, desmin, S-100, CD34 as well as c-Kit (CD117). This is probably the first case of a congenital GIST causing intestinal obstruction associated with perforation peritonitis. The report highlights the point of distinction between adult and congenital GIST, in terms of clinical presentation, histochemical markers, prognostic factors, and behavior.