title: Pediatric/"Wildtype" gastrointestinal stromal tumors.
abstract: Pediatric/"Wildtype" gastrointestinal stromal tumor (P/WT-GIST) is a rare cancer, distinct and markedly different from the phenotype found predominantly in older patients (adult, non-wildtype GIST). Having a different molecular signature, it is not responsive to standard adjuvant therapies utilized in adult GIST, and surgery remains the only effective cure. However, even with presumed complete resections in patients with localized disease at presentation, recurrence rates are high. Furthermore, it is an indolent cancer that can persist for decades, and treatment strategies must balance the possible morbid risks of intervention with the reality of preserving quality of life in the interim. Effective adjuvant therapies remain elusive, and research is critically needed to identify both targets and drugs for treatment consideration.
authors.affiliation: Paediatric Surgery, Great Ormond Street Hospital, London, UK.
authors.forename: Dhanya
authors.initials: D
authors.lastname: Mullassery
authors.affiliation: Department of Surgery, Boston Children's Hospital, 300 Longwood Ave, Fegan 3, Boston, Massachusetts 02445. Electronic address: christopher.weldon@childrens.harvard.edu.
authors.forename: Christopher B
authors.initials: CB
authors.lastname: Weldon
mesh_terms: D000359:Aftercare; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D014408:Biomarkers, Tumor / Q000235:genetics; D017024:Chemotherapy, Adjuvant; D002648:Child; D005770:Gastrointestinal Neoplasms / Q000175:diagnosis / Q000188:drug therapy / Q000235:genetics / Q000601:surgery; D046152:Gastrointestinal Stromal Tumors / Q000175:diagnosis / Q000188:drug therapy / Q000235:genetics / Q000601:surgery; D005819:Genetic Markers; D006801:Humans; D010372:Pediatrics; D011379:Prognosis
chemical_list: D014408:Biomarkers, Tumor; D005819:Genetic Markers
keywords: Cancer; GIST; Gastrointestinal; Pediatric; Review; Teatment