Clinical and molecular characteristics of pediatric gastrointestinal stromal tumors (GISTs).

摘要: Background To describe the clinical characteristics, molecular features, treatment, and outcome of six pediatric patients with gastrointestinal stromal tumors (GISTs). Patients Methods Retrospective review GISTs, seen at The Hospital for Sick Children (HSC) Toronto, over an 11-year period. All specimens were stained CD 117 34 antigens. Three sequenced mutations in exons 9, 11, 13 c-kit gene. Results Five evaluated treated HSC one was referred histopathological consultation only. median patient age diagnosis 13.6 years, (6.9–14.8 years); four female. presented anemia secondary to (GI) bleeding. disease localized five two had other malignancies consistent diagnoses Carney's triad. Immunohistochemical staining showed heavy cytoplasmic localization all tumor cells. A novel point mutation KIT codon 456 exon 9 found case. Complete surgical resection achieved managed our center none received adjuvant therapies. Disease recurred locally patient. Four are alive is lost follow-up. Conclusions In children adolescents, GISTs should be considered differential GI hemorrhage. absence 11 identification a suggest that may respond differently currently available targeted therapies therefore studied within context collaborative group trials. Pediatr Blood Cancer 2005; 45:20–24. © 2005 Wiley-Liss, Inc.