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Fibroblastisch/myofibroblastisch differenzierte Tumoren

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Pathologie

Zusammenfassung

Fibroblastisch/myofibroblastisch differenzierte Weichteiltumoren repräsentieren eine große und heterogene Gruppe mesenchymaler Tumoren, wobei zahlreiche Tumoren sowohl fibroblastisch als auch myofibroblastisch differenzierte Zellen enthalten, die möglicherweise unterschiedlichen Funktionszustände eines Zelltyps entsprechen. Myofibroblasten nehmen eine (funktionelle) Intermediärstellung zwischen kollagenproduzierenden Fibroblasten und kontraktilen glatten Muskelzellen ein und sind durch eine außerordentliche morphologische Plastizität charakterisiert. Konzeptionelle Änderungen in dieser Tumorgruppe umfassen die Einordnung des Myxofibrosarkoms (früher myxoides "MFH") in die Gruppe fibroblastär differenzierter Sarkome, die Klassifizierung der Desmoidfibromatose als intermediär, lokal aggressive Tumoren und die Erweiterung des Spektrums der solitären fibrösen Tumoren, dass die Mehrzahl der früher als "Hämangioperizytome" bezeichneten Tumoren umfaßt. Neue Tumorentitäten sind die ischämische Fasziitis, das desmoplastische Fibroblastom, das Myofibroblastom vom Mammatyp, das Gardner-Fibrom, das nuchale Fibrom, die Lipofibromatose, das zellreiche Angiofibrom, das Angiomyofibroblastom, das niedrigmaligne fibromyxoide Sarkom, das akrale myxoinflammatorische fibroblastische Sarkom, das sklerosierende epitheloide Sarkom und das niedrigmaligne myofibroblastische Sarkom.

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Mentzel, T., Schneider-Stock, R. (2009). Fibroblastisch/myofibroblastisch differenzierte Tumoren. In: Cardesa, A., Rudolph, P., Mentzel, T., Slootweg, P. (eds) Pathologie. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-540-72885-6_13

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