Pediatric/“Wildtype” gastrointestinal stromal tumors

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• 作者：Dhanya Mullassery ; MRCS (Lon) ; PhD^a ; Christopher B. Weldon ; MD ; PhD^b ; ^{christopher.weldon@childrens.harvard.edu}
• 关键词：Gastrointestinal ; Cancer ; Pediatric ; GIST ; Review ; Teatment
• 刊名：Seminars in Pediatric Surgery
• 出版年：2016
• 出版时间：October 2016
• 年：2016
• 卷：25
• 期：5
• 页码：305-310
• 全文大小：257 K
• 卷排序：25

文摘

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.