Gastrointestinal stromal tumors

被引:103
作者
Beham, Alexander W. [1 ]
Schaefer, Inga-Marie [2 ]
Schueler, Philipp [1 ]
Cameron, Silke [3 ]
Ghadimi, B. Michael [1 ]
机构
[1] Univ Gottingen, Dept Surg, D-37075 Gottingen, Germany
[2] Univ Gottingen, Dept Pathol, D-37075 Gottingen, Germany
[3] Univ Gottingen, Dept Med Gastroenterol & Endocrinol, D-37075 Gottingen, Germany
关键词
Gastrointestinal stromal tumor; Imatinib mesylate; Surgical resection; PHASE I-II; C-KIT; CARNEY TRIAD; GERMLINE MUTATION; PDGFRA MUTATIONS; EXTRAADRENAL PARAGANGLIOMA; LAPAROSCOPIC RESECTION; INTRAMURAL LEIOMYOMAS; INTERSTITIAL-CELLS; PROGNOSTIC-FACTORS;
D O I
10.1007/s00384-011-1353-y
中图分类号
R57 [消化系及腹部疾病];
学科分类号
摘要
The gastrointestinal stromal tumor (GIST) is the most common mesenchymal tumor of the intestinal tract, known to be refractory to conventional chemotherapy or radiation. Its pathogenesis is defined by mutations within the KIT and PDGFRA gene, which constitutively activate KIT and PDGFRA oncoproteins, and serve as crucial diagnostic and therapeutic targets. Besides surgery, therapy with imatinib mesylate, which inhibits KIT kinase activity, represents the other cornerstone for the treatment of GIST. Still, the only curative option for GIST is given after complete surgical removal even in a metastatic setting, but recurrence is common, and the risk can be defined by surgical factors like incomplete resection, intraperitoneal rupture, or bleeding and tumor associated factors like tumor size, mitotic index, or localization. Consequently, adjuvant therapy with imatinib mesylate or other tyrosine kinase inhibitors is recommended for high-risk patients after complete resection. For unresectable and advanced GIST, a partial response or stable disease can be achieved in about 80% of patients with imatinib mesylate.
引用
收藏
页码:689 / 700
页数:12
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