Zusammenfassung
In erster Linie muss ein gastrointestinaler Stromatumor (GIST) in Erwägung gezogen werden, wenn ein mesenchymaler Tumor im Gastrointestinaltrakt gefunden wird, wobei diese Tumoren am häufigsten im Magen vorkommen. Die Diagnose beruht in der täglichen Praxis auf histologischen (Zellbild und Gewebsarchitektur) sowie immunhistochemischen Merkmalen (zelluläre CD117- und oft CD34-Positivität). Die Expression von CD117 ist Ausdruck einer Aktivierung der Typ-III-Rezeptor-Tyrosinkinase KIT, welcher eine aktivierende Mutation des KIT-Gens zugrunde liegt. Bei etwa 5% der Fälle ist alternativ eine Mutation des PDGF-Rezeptor-α-Gens mit Expression des entsprechenden Wachstumsfaktorrezeptors nachzuweisen. Die Einschätzung der Dignität bei einem GIST ist schwierig und im Einzelfall mit Unsicherheiten behaftet. Eine Malignitätsgraduierung entsprechend den Kriterien bei Weichgewebstumoren ist nicht möglich. Es besteht heute aber allgemeiner Konsensus darüber, dass die wichtigsten Einzelparameter für die prognostische Bewertung der Tumoren ihre Größe und die Zahl der Mitosen sind, neuerdings wird überdies die Tumorlokalisation berücksichtigt. Es hat sich in den letzten Jahren gezeigt, dass auch die Mutationsanalyse Beiträge zur Bestimmung der Tumoraggressivität bzw. Metastasenwahrscheinlichkeit und Therapieansprechbarkeit leisten kann.
Abstract
A diagnosis of gastrointestinal stromal tumor must be considered if a mesenchymal tumor is localized in the gastrointestinal tract, especially in the stomach. In daily practice diagnosis is based on the histology (cellular features and histologic architecture) and immunohistochemistry (cellular positivity with antibodies to CD117 and often to CD34). Expression of CD117 indicates the autoactivation of a type-III-receptor tyrosine kinase mediated by mutation of the KIT gene. This is lacking in the roughly 5% of cases which instead show a mutation of the PDGF receptor alpha gene. The estimation of dignity is difficult and can be uncertain in some cases. A malignancy grading according to the procedure in soft tissue tumors is not possible. Nowadays, however, the general consensus is that size of the tumor and number of mitoses are the most important criteria for appraising tumor aggressiveness and risk of metastasis. The tumor localization was later added to these criteria. Recent years have shown that mutation analysis can also provide information for judging tumor agressiveness and predicting possible metastasis and response to therapy.
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Katenkamp, D., Katenkamp, K. Gastrointestinale Stromatumoren. Chirurg 79, 625–629 (2008). https://doi.org/10.1007/s00104-008-1525-7
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DOI: https://doi.org/10.1007/s00104-008-1525-7