Pediatric Gastrointestinal Stromal Tumors

Department of Pediatrics, Texas Children's Cancer Center, 6621 Fannin Street, CC1510.00, Houston, TX 77030, USA.
Hematology/oncology clinics of North America (Impact Factor: 2.3). 03/2009; 23(1):15-34, vii. DOI: 10.1016/j.hoc.2008.11.005
Source: PubMed


Gastrointestinal stromal tumors (GISTs) rarely occur in pediatric patients, but increased recognition of adult GIST has led to better awareness of the existence of this entity in the pediatric population. GIST occurring in pediatric patients has a unique biology and clinical behavior and warrants discussion as an independent entity. The generally accepted definition of pediatric GIST is a tumor that is diagnosed at the age of 18 years or younger. This review highlights the clinical features, molecular biology, and clinical management of this rare pediatric entity.

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    • "Gastrointestinal stromal tumors (GISTs) represent the most common mesenchymal tumor of the gastrointestinal tract in the adult population, with a reported incidence rate of 6.8 cases per million [1]. These tumors are commonly diagnosed in the stomach or small intestine, and the median age at diagnosis is 60 years [2]. GISTs are far less common in children , and pediatric GISTs appear to be different in relation to the disease observed in adults. "
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    ABSTRACT: Gastrointestinal stromal tumors (GISTs) in adolescence are far less common than adult GISTs and have varied GIST genotypes that present diagnostic and therapeutic challenges. Here, we discuss a 21-year-old male with diagnosis of unresectable, imatinib-resistant GIST. At initial evaluation, a neoadjuvant treatment approach was recommended. As such, the patient received imatinib over the course of one year. Unfortunately, the GIST increased in size, and a subsequent attempt at surgical resection was aborted fearing infiltration of major vascular structures. The patient was then referred to our institution, at which time imatinib therapy was discontinued. Surgical intervention was again considered and the patient underwent successful resection of massive intra-abdominal GIST with total gastrectomy and Roux-en-Y esophagojejunostomy. Since pediatric GISTs are typically resistant to imatinib, we performed genotype analysis of the operative specimen that revealed KIT mutations associated with imatinib sensitivity and resistance. Given the sequencing data and operative findings, the patient was started postoperatively on sunitinib. This case illustrates the importance of understanding both adult and pediatric GISTs when implementing appropriate treatment regimens. Since the genotype of GISTs dictates phenotypic behavior, mutational analysis is an important component of care especially for adolescents whose disease may mirror the pediatric or adult population.
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    ABSTRACT: Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the gastrointestinal tract. The vast majority of GISTs harbor a KIT or PDGFRA mutation and express KIT by immunohistochemistry. However, KIT-negative tumors and tumors showing unusual morphologic features can cause major diagnostic problems. The ability to inhibit the active KIT or PDGFRA kinase with tyrosine kinase inhibitors and alternative drugs demands more than ever accurate tumor classification and risk assessment. This article focuses on the pathology of GIST, including unusual variants and morphologic changes resulting from treatment. Parameters for risk assessment, potentially helpful new immunohistochemical markers, differential diagnosis, and the application of molecular classification schemes are discussed.
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