Clinical, radiological, and pathological findings in four children with gastrointestinal stromal tumors of the stomach.
The incidence of gastrointestinal stromal tumors (GISTs) in children is exceptionally low. However, during the last decade these tumors attracted increasing attention, because they were found to express the cell surface transmembrane receptor kit (CD117) that has tyrosine kinase activity. This tyrosine kinase can be semi-selectively inhibited by signal transduction inhibitors such as imatinib mesylate (Glivec), which is a competitive inhibitor of c-kit, c-abl, platelet-derived growth factor receptor-alpha (PDGFR-alpha) and PDGFR-beta, and abl-related gene (arg). The authors present the clinical, radiographic, and pathological findings of 4 children who were diagnosed with gastric GIST. One of them had an incomplete Carney triad including GIST and mediastinal paraganglioma. All 4 patients presented with anemia and anemia-related symptoms and underwent total resection of the tumor. One patient received additional chemotherapy (in the pre-imatinib era) and 2 patients received a short course of imatinib mesylate. With a follow-up of 116, 55, 23, and 10 months all patients are alive in first complete continuous remission. In children and adolescents, particularly in female patients, GISTs should be included in the differential diagnosis of anemia secondary to gastrointestinal hemorrhage. Complete surgical resection is the mainstay of treatment for this tumor, with imatinib mesylate restricted to patients with advanced or metastatic tumors. Since late recurrences (up to 30 years following initial diagnosis) are reported, a life-long follow-up is mandatory in these patients.
Sauseng
Werner
W
Division of Pediatric Hematology and Oncology/Department of Pediatrics and Adolescent Medicine, Medical University of Graz, Graz, Austria.
Benesch
Martin
M
Lackner
Herwig
H
Urban
Christian
C
Kronberger
Martina
M
Gadner
Helmut
H
Höllwarth
Michael
M
Spuller
Ekkehard
E
Aschauer
Manuela
M
Horcher
Ernst
E
D000293:Adolescent; D000970:Antineoplastic Agents / Q000627:therapeutic use; D001549:Benzamides; D002648:Child; D005260:Female; D046152:Gastrointestinal Stromal Tumors / Q000000981:diagnostic imaging* / Q000188:drug therapy / Q000473:pathology*; D006801:Humans; D000068877:Imatinib Mesylate; D008297:Male; D010879:Piperazines / Q000627:therapeutic use; D011505:Protein-Tyrosine Kinases / Q000037:antagonists & inhibitors; D019009:Proto-Oncogene Proteins c-kit / Q000737:chemistry / Q000378:metabolism; D011743:Pyrimidines / Q000627:therapeutic use; D011859:Radiography; D013274:Stomach Neoplasms / Q000000981:diagnostic imaging* / Q000188:drug therapy / Q000473:pathology*; D016896:Treatment Outcome
D000970:Antineoplastic Agents; D001549:Benzamides; D010879:Piperazines; D011743:Pyrimidines; D000068877:Imatinib Mesylate; D011505:Protein-Tyrosine Kinases; D019009:Proto-Oncogene Proteins c-kit