Janeway Katherine A, Weldon Christopher B
Department of Pediatric Oncology, Dana Farber Cancer Institute-Children's Hospital Boston, Boston, Massachusetts, USA.
Semin Pediatr Surg. 2012 Feb;21(1):31-43. doi: 10.1053/j.sempedsurg.2011.10.003.
Pediatric gastrointestinal stromal tumor (GIST) is a rare entity that can be quite different from its adult counterpart. This report provides a comprehensive review on the diagnosis and management of this tumor in children and adolescents, including its oncogenesis and associated syndromes. Surgery remains a mainstay of treatment, but there are no standard guidelines available at this time regarding the best practice for multimodality therapy as our understanding of the biology of GIST is still in evolution. Therefore, pediatric patients with GIST should be ideally treated in the context of clinical trials at specialized, multidisciplinary centers throughout the course of their disease, especially because these patients may live for years after diagnosis.
小儿胃肠道间质瘤（GIST）是一种罕见的疾病，与成人的同类疾病有很大不同。本报告全面综述了儿童和青少年该肿瘤的诊断与管理，包括其肿瘤发生机制及相关综合征。手术仍是主要治疗手段，但由于我们对GIST生物学的理解仍在不断发展，目前尚无关于多模式治疗最佳实践的标准指南。因此，小儿GIST患者在疾病全过程中，理想情况下应在专门的多学科中心参与临床试验进行治疗，特别是因为这些患者在确诊后可能存活数年。
