Pediatric Female with Adult Type Small Intestine Gastrointestinal Stromal Tumor [GIST]: A case report

Abstract

Background: Gastrointestinal stromal tumor [GIST] is commonest mesenchymal tumor, mainly affects adults. But, 1.5% to 2% GISTs were reported in pediatrics and adolescents. The treatment of GIST is managed as in adult because of lack of information about pediatric GIST. Aim of the work: Here we presented a case report of pediatric GIST. Current literature was reviewed and a summary was formulated to form a base for future optimal treatment of childhood GIST. Case summary: A 14 years Saudi female presented with vague abdominal pain and easy fatigability for 8 months. The pain located in lower abdomen, mild, waning, no aggravation or reliving factor and not radiating. Clinically, there was peri umbilical round, firm and not tender mass extending to right side of the abdomen till right hypochondrium, with no movement with respiratory movements. Investigations demonstrate severe microcytic hypochromic anemia. A computed tomography [CT] abdomen and pelvis showed evidence of soft tissue mass, about 13*11*12cm, of heterogeneous pattern and enhancement with area of hypodensity and cystic degeneration. There was necrosis of mesentery with some calcification seen inside the lesion suggestive of query GIST or Lymphoma. En block resection of the mass done with safety margin, biopsy from lymph node and side-to-side anastomosis done using linear stapler. The histopathological examination revealed ill and well defined fasicles of tumor cells that have spindle, oval and round shape nuclei invading mesentery and small bowel. Mitotic figurer 5-10/50 HPF, reactive lymph node. Immunohistochemistry showed diffused strong positive CD117 and CD34, negative S-100

Keywords

• pediatrics
• adolescents
• small intestine
• gastrointestinal stromal tumor
• management