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Maligne odontogene Tumoren

Malignant odontogenic tumors

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Zusammenfassung

Maligne odontogene Tumoren sind extrem selten. Analog zu den benignen Varianten wird deshalb zwischen malignen epithelialen odontogenen Tumoren, den odontogenen Karzinomen und den noch selteneren malignen mesenchymalen Tumoren, den odontogenen Sarkomen unterschieden. Odontogene Karzinosarkome werden bisher von der WHO nicht als eigenständige Entität anerkannt. Zu den odontogenen Karzinomen gehört das ameloblastische Karzinom, das primäre intraossäre Karzinom, das hellzellige odontogene Karzinom, das geisterzellhaltige odontogene Karzinom und als Sonderfall das metastasierende Ameloblastom. Die odontogenen Sarkome umfassen das ameloblastische Fibrosarkom und das ameloblastische Fibrodentino- und Fibroodontosarkom. Das metastasierende Ameloblastom kann nur an seinen Metastasen diagnostiziert werden. Alle anderen malignen Tumoren weisen eindeutige zelluläre Atypien, ein invasives Wachstum und vermehrt Mitosen auf. Während die odontogenen Sarkome ein niedriges Metastasierungspotenzial zeigen, besitzen odontogene Karzinome, besonders das ameloblastische Karzinom (AmCa) und das odontogene Schattenzellkarzinom (OGCC) sowie das primäre intraossäre Karzinom (PIOC), eine mäßige bis schlechte Prognose mit Überlebensraten nach 5 (AmCa; OGCC) bzw. 3 Jahren (PIOC) von etwa 70% bzw. 37%. Die Behandlung der Karzinome muss deshalb radikal und gegebenenfalls multimodal erfolgen.

Abstract

Malignant odontogenic tumors are extremely rare. As with benign odontogenic tumors, malignant epithelial odontogenic tumors or odontogenic carcinomas are distinguished from the even rarer mesenchymal ones, the odontogenic sarcomas. The existence of odontogenic carcinosarcomas is not yet acknowledged by the World Health Organization. Odontogenic carcinomas comprise ameloblastic carcinoma (AmCa), primary intraosseous carcinoma (PIOC), clear cell odontogenic carcinoma, odontogenic ghost cell carcinoma (OGCC), and the special case of metastasizing ameloblastoma. Odontogenic sarcomas consist of ameloblastic fibrosarcoma and ameloblastic fibrodentinosarcoma and fibroodontosarcoma. Whereas metastasizing ameloblastoma can be diagnosed only after having metastasized, all other malignant odontogenic tumors present with atypia, increased cellularity and mitoses, and invasion. Odontogenic sarcomas are regarded as low-grade tumors that rarely metastasize. Odontogenic carcinomas, however, especially AmCa, OGCC, and PIOC, are more aggressive, with a 5-year survival rate of about 70% for AmCa and OGCC and a 3-year survival rate of about 37% for PIOC. Radical surgery, eventually in combination with radiotherapy, is the treatment of choice.

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Authors and Affiliations

  1. Institut für Pathologie, DÖSAK-Zentralregister am Knochentumor-Referenzzentrum, Universitätsspital Basel, Schoenbeinstraße 40, 4031, Basel, Schweiz

    G. Jundt

  2. Abteilung Oralchirurgie und Zahnärztliche Röntgenologie, Charité - Universitätsmedizin Berlin, Berlin, Deutschland

    P.A. Reichart

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  1. G. Jundt
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Correspondence to G. Jundt.

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Widmung

Herrn Professor Dr. med. Wolfgang Remagen zu seinem 80. Geburtstag gewidmet.

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Jundt, G., Reichart, P. Maligne odontogene Tumoren. Pathologe 29, 205–213 (2008). https://doi.org/10.1007/s00292-008-0998-y

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