Undifferenziertes Karzinom des distalen Gallengangs vom Spindel- und Riesenzelltyp: Ein Fallbericht

Zusammenfassung

Undifferenzierte Karzinome der Gallenwege sind eine Rarität und manifestieren sich häufiger in der Gallenblase als in den extrahepatischen Gallenwegen. Wir berichten hier von einem äußerst seltenen undifferenzierten Karzinom des distalen Gallengangs vom Spindel- und Riesenzelltyp. Der Tumor ging in Form atypischer Drüsen und Einzelzellkomplexe aus dem biliären Epithel hervor und graduell in eine vorwiegend spindelzellige Architektur über, die mehr als 98 % des Tumorvolumens ausmachte. Fokal gelangten osteoklastäre, multinukleäre Riesenzellen zur Darstellung. Die immunhistochemischen Untersuchungen zeigten in den besser differenzierten, drüsigen Tumoranteilen eine kräftige Expression epithelialer Marker, die in den spindelzelligen Arealen bei Hinzutreten einer Positivität für Vimentin nur noch einzelzellig erhalten blieb. Der Tumor wies eine hohe Ki-67-Proliferationsaktivität auf und zeigte Mutationen in den Genen Cyclin D3 (CCND3), Fibroblast Growth Factor Receptor 4 (FGFR4), Neurofibromin 1 (NF1) und NOTCH3. Der hier vorgestellte Fall unterstreicht die Relevanz dieser Tumorentität jenseits von Pankreas und Gallenblase und verdeutlicht die zur Diagnosefindung unverzichtbare Kombination aus Morphologie und Immunhistochemie.

Abstract

Undifferentiated carcinoma of the biliary tract is rare and more frequently occurs in the gall bladder than in the extrahepatic bile ducts. We report on an extremely rare case of a spindle and giant cell type undifferentiated carcinoma of the distal bile duct. In the presented tumor, atypical glands and single-cell clusters arising from the bile duct epithelium gradually transitioned into a predominantly sarcomatoid architecture, which constituted more than 98 % of the whole tumor volume. Focally, osteoclast-like giant cells were intermixed with the spindle cells. The tumor showed a high proliferation activity (Ki-67) and was demonstrated to harbor mutations in the genes for cyclin D3 (CCND3), fibroblast growth factor receptor 4 (FGFR4), neurofibromin 1 (NF1) and NOTCH3, as assessed by next-generation sequencing analysis. The presented case underscores the relevance of this tumor entity beyond the pancreas and gall bladder and emphasizes the indispensable combination of morphology and immunohistochemistry regarding the diagnostic process.

Key words

Gastro-entero-pankreatische Tumoren - Leber - Virushepatitis

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