Zusammenfassung
Gastrointestinale Stromatumoren (GIST) sind die häufigsten mesenchymalen Tumoren des Gastrointestinaltraktes. Wir beschreiben erstmalig einen Patienten mit einem riesigen GIST der Bauchwand, der keinerlei strukturelle Verbindung zum Gastrointestinaltrakt, Omentum oder Mesenterium zeigte. Unter Berücksichtigung der Größe von 24 cm bei niedrigem Mitoseindex ist dieser GIST bezüglich seiner Metastasierungspotenz als "intermediate risk" einzuschätzen. Die operative Entfernung des Tumors wurde entsprechend der allgemein anerkannten Radikalitätsprinzipien durchgeführt. Der klassische, immunhistochemische Phänotyp des hier vorliegenden Tumors ermöglicht eine differenzialdiagnostische Abgrenzung zum abdominellen Desmoidtumor sowie zum "solitary fibrous tumor". Im Falle des Auftretens von Metastasen im weiteren Verlauf erscheint der therapeutische Nihilismus seit Einführung von Imatinib, einem Tyrosinkinaseinhibitor, der in der Behandlung des metastasierten GIST viel versprechende Ergebnisse zeigt, nicht mehr gerechtfertigt.
Abstract
Gastrointestinal stromal tumors (GIST) are the most common mesenchymal tumors of the gastrointestinal tract. We describe here for the first time a patient with a huge GIST of the abdominal wall without any relation to the gastrointestinal tract, omentum, or mesentery. With regard to the size of 24 cm and a low mitotic index, this GIST is considered an intermediate risk for metastasis. Radical surgical resection was performed with negative pathologic resection margins. The classic immunohistochemical phenotype of the tumor described facilitates the differential diagnosis to exclude abdominal desmoid tumor and solitary fibrous tumor (SFT). In the case of metastasis, therapeutic nihilism no longer seems justified with the availability of imatinib, a tyrosine kinase inhibitor, which shows encouraging results in the therapy of metastatic GIST.
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Thalheimer, A., Meyer, D., Gattenlöhner, S. et al. Gastrointestinaler Stromatumor der Bauchwand. Chirurg 75, 708–712 (2004). https://doi.org/10.1007/s00104-003-0696-5
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DOI: https://doi.org/10.1007/s00104-003-0696-5