Gastrointestinal stromal tumor in a child and review of the literature

Abstract

Gastrointestinal stromal tumors (GIST) are c-KIT positive neoplasm that occur predominantly in adults. A 13-year-old boy who presented with massive gastrointestinal hemorrhage was subsequently diagnosed with GIST of the stomach. The tumor originated from the lesser curvature with a narrow base and no infiltration into surrounding organs. Two metastatic lesions in the liver were also found. Total excision of the gastric lesion was performed followed by adjuvant imatinib mesylate chemotherapy. He has had no signs of recurrence during 8 months of follow-up. GIST in children are rare and their behavior is notoriously difficult to predict. We report our experience and review the literature.

Gastrointestinal stromal tumor of the lesser omentum in a young adult patient with a history of hepatoblastoma: Report of a case

“…Miettinen et al reported that only 3% of GISTs are diagnosed before the age of 21 years and GISTs arise only rarely in children. 8 In a 11 and found that GISTs are more predominant in young females than in young males and show less metastasis in pediatric cases than in adult cases. Juvenile GISTs usually originate in the stomach, often in multiples, less active, and usually negative for a kit mutation.…”

Gastrointestinal stromal tumor of the stomach in a child with a 3-year follow-up period—Case report

“…3 Although the response to conventional chemotherapy and radiotherapy is poor, the oral protein kinase inhibitor, imatinib mesylate, has shown promising antiproliferative and apoptotic effects and may be beneficial for patients with GISTs, particularly those with recurrent metastatic disease. 1,4 The indication for adjuvant imatinib mesylate therapy has not been established in pediatric GIST cases thus far. 4 Because of the infrequency of GIST in pediatric patients, there is currently no standardized treatment protocol.…”

“…However in children metastases are seen at the time of diagnosis in more than 50% of pediatric patients. 4 Liver involvement or multifocal omental implants has been described as the most common location of metastatic spread. GISTs can occur anywhere along the gastroin- testinal tract, including stomach (70%), small intestine (20-30%), anorectum (7%), colon, and esophagus.…”

“…The likelihood of metastatic disease correlates with tumor size and degree of mitotic activity: one mitosis or fewer per 10 (higher power fields (HPF) is considered to be low grade, whereas 2 or more mitoses per 10 HPF is thought to have a greater malignant potential. [3][4][5][6] Surgical resection is considered the current standard of care for GIST, and lymphadenectomy has not been shown to improve survival. 3 Although the response to conventional chemotherapy and radiotherapy is poor, the oral protein kinase inhibitor, imatinib mesylate, has shown promising antiproliferative and apoptotic effects and may be beneficial for patients with GISTs, particularly those with recurrent metastatic disease.…”

“…2 Presenting signs and symptoms are variable depending on the size and anatomic location of the tumor and can include abdominal pain, hematemesis, melena, and anemia from occult gastrointestinal bleeding. 4 The use of CT or abdominal ultrasound has been the most frequently used imaging tools to establish the diagnosis of GIST. Typically, the lesion is a lobulated, irregular, intraluminal mass, which contains ulcerations and which may be quite large.…”