Gastrointestinale Stromatumoren (GIST): variable klinische Manifestationen vom Zufallsbefund bis zur akuten gastrointestinalen Blutung

Zusammenfassung

Drei Fallbeispiele verdeutlichen das breite klinische Spektrum gastrointestinaler Stromatumoren (GIST), der häufigsten Untergruppe gastrointestinaler mesenchymaler Tumoren (GIMT), mit daraus resultierenden Folgen für Therapie und Prognose. Die erste Kasuistik beschreibt einen 82-jährigen Patienten mit einer hämodynamisch wirksamen oberen gastrointestinalen Blutung, deren Ursache ein GIST des Magens war. Gastrointestinale Blutungen sind neben abdominellen Schmerzen und tastbaren oder sonographisch nachweisbaren abdominellen Raumforderungen die typischen klinisch apparenten Manifestationen dieser Tumoren. In der zweiten Kasuistik wird ein GIST bei einer 44-jährigen Patientin als Zufallsbefund erhoben, beispielhaft für den andererseits oft asymptomatischen Verlauf. Übersichtsartig werden die mittlerweile durch immunhistochemische Methoden gut zu differenzierenden Tumorgruppen vorgestellt, die man heute unter dem Begriff der gastrointestinalen mesenchymalen Tumoren (GIMT) subsumiert. Ausgehend vom dritten Fallbeispiel eines 40-jährigen GIST-Patienten mit postoperativem Rezidiv und sekundärer Resistenz nach einjähriger überaus erfolgreicher Therapie mit Imatinib (Gleevec®), einem Antagonisten der pathogenetisch relevanten Rezeptortyrosinkinase c-KIT, werden prognostisch wichtige Faktoren gastrointestinaler Stromatumoren (GIST) diskutiert und Möglichkeiten und Grenzen dieser ersten effektiven, seit wenigen Jahren verfügbaren medikamentösen Therapie fortgeschrittener GIST dargestellt.

Abstract

Three cases of gastrointestinal stromal tumors (GIST) are reported as typical examples of the broad clinical spectrum in which these rare tumors can be detected. The first case describes an 82-year-old patient with a hemorrhagic shock due to upper gastrointestinal bleeding from a GIST of the stomach. GIST most frequently present with either gastrointestinal bleeding, abdominal pain or a detectable mass on physical examination or by ultrasound imaging. Clinically asymptomatic tumor growth also occurs as demonstrated by the second case of a 44-year-old ­woman with an incidental finding of GIST during surgery of the esophagus. The cases are used to discuss the consequences for therapy and prognosis resulting from the heterogeneity of this tumor entity; the relevant immunohistochemical markers used to distinguish between various tumor subtypes of gastrointestinal mesenchymal tumors (GIMT) are listed. Since gastrointestinal stromal tumors (GIST) represent the most common subgroup of GIMT, we focus on the clinicopathological prognostic factors of GIST. The third case of a 40-year-old patient with a malignant GIST recurrence after surgery and exhibiting secondary resistance after one year of successful therapy with the receptor tyrosine kinase inhibitor imatinib (Gleevec®), antagonizing pathogenetically relevant constitutive c-KIT activation, illustrates the potential and limitations of the only effective drug treatment for advanced GIST.

Gastrointestinaler mesenchymaler Tumor (GIMT) - gastrointestinaler Stromatumor (GIST) - Schwannom - Leiomyom - gastrointestinale Blutung - STI-571/Imatinib

Gastrointestinal mesenchymal tumor (GIMT) - gastrointestinal stromal tumor (GIST) - schwannoma - leiomyoma - gastrointestinal bleeding - STI-571/imatinib

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