Zusammenfassung
Gastrointestinale Stromatumoren (GIST) sind die häufigsten mesenchymalen Tumoren des Gastrointestinaltraktes. Der therapeutische Goldstandard für lokalisierte GIST ist ihre vollständige chirurgische Entfernung mit einem Sicherheitsabstand von mindestens 1–2 cm. Eine intraoperative Verletzung der Tumorkapsel ist unbedingt zu vermeiden, da diese mit einem sehr hohen Risiko einer peritonealen Streuung einhergeht. Da eine Metastasierung in Lymphknoten bei GIST extrem selten vorkommt, ist eine routinemäßige Lymphknotendissektion nicht angezeigt. Kleinere GIST können nach den Regeln der Tumorchirurgie laparoskopisch entfernt werden. Abhängig von der Tumorgröße, des Mitoseindex und der Lokalisation des Primärtumors ist das Rezidivrisiko nach potenziell kurativer Resektion in vielen Fällen beträchtlich. Patienten mit intermediärem und hohem Risiko gemäß der Klassifikation von Miettinen sollten adjuvant mit dem Tyrosinkinaseinhibitor Imatinib behandelt werden. Ausgenommen sind Patienten, deren Tumor die Mutation D842V in Exon18 des PDGFRA-Gens aufweist. Nach den aktuellen ESMO-Leitlinien soll die Therapie über 3 Jahre erfolgen. Dies führt zu einer signifikanten Verbesserung des progressionsfreien Überlebens gegenüber einer Therapie über ein Jahr und auch zu einer Verbesserung des Gesamtüberlebens.
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.
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Interessenkonflikt. V. Fendrich und D.K. Bartsch geben an, dass kein Interessenkonflikt besteht. Dieser Beitrag beinhaltet keine Studien an Menschen oder Tieren.
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Fendrich, V., Bartsch, D. Gastrointestinale Stromatumoren. Chirurg 85, 545–556 (2014). https://doi.org/10.1007/s00104-013-2681-y
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DOI: https://doi.org/10.1007/s00104-013-2681-y
Schlüsselwörter
- Gastrointestinaler Strumatumor
- Operative Therapie
- Tyrosinkinaseinhibitor
- Interdisziplinäres Management
- Leitlinien