[Gastrointestinal stromal tumors: diagnostics and therapy]

Abstract

Gastrointestinal stromal tumors (GIST) are the most frequent mesenchymal tumors of the gastrointestinal tract. The standard therapy is complete surgical resection with safety margins of 1-2 cm. Intraoperative rupture of the tumor capsule must be avoided because this carries a very high risk of tumor spread. A lymph node dissection is not routinely indicated as lymph node metastases very rarely occur with GIST. Smaller GISTs can normally be removed laparoscopically according to the rules of tumor surgery. Depending on the size of the tumor, the mitosis index and the localization of the primary tumor, the risk of recurrence after potentially curative resection is considerable in many cases. Patients with intermediate and high risks according to Miettinen's classification should receive adjuvant treatment with the tyrosine kinase inhibitor imatinib. Exceptions are those patients whose tumors exhibit the mutation D842V in exon18 of the PDGFRA gene. According to current European Society for Medical Oncology (ESMO) guidelines this therapy should be continued for 3 years. This leads to a significant improvement in progression-free survival compared to a 1-year therapy, and more important to an improvement in overall survival.