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Nierenzellkarzinome bei jungen Patienten

Pediatric renal cell carcinoma

  • Schwerpunkt: Kinderpathologie
  • Published:
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Zusammenfassung

Bei Kindern und Jugendlichen kommen Nierenzellkarzinome sehr selten vor und machen nur etwa 1% der Nierentumoren in dieser Altersgruppe aus. Seit der letzten WHO-Klassifikation der Nierenzellkarzinome 1997 wurden neue Entitäten bei jungen Patienten beschrieben, die nun in die neue WHO-Klassifikation 2004 aufgenommen wurden.

Das Neuroblastom-assoziierte Nierenzellkarzinom macht 2,5% der Nierenzellkarzinome bei jungen Patienten aus. Es tritt mehrere Jahre nach einem Neuroblastom auf. Bei einem hohem Prozentsatz dieser Karzinome kann eine allelische Instabilität für die Chromosomen 20q13, 2p31–32.2, 13q22 und 14q31 nachgewiesen werden.

TFE3-Translokationskarzinome stellen etwa 20% der Nierenzellkarzinome im Kindes- und Adoleszentenalter. Die Translokationen t(X;17)(p11.2;q25) und t(X;1)(p11.2;q21.2) führen beide zur Überexpression des TFE3-Proteins. TFE3-Translokationskarzinome zeichnen sich durch reichliches „voluminöses“ klares Zytoplasma und eine teilweise papilläre Architektur aus.

Auch nach der aktualisierten WHO-Klassifikation sind im Vergleich zum Erwachsenenalter wesentlich mehr Nierenzellkarzinome im jungen Alter noch nicht klassifizierbar, sodass eine große heterogene unklassifizierte Tumorgruppe mit morphologisch distinkten Tumoren die Quelle zukünftiger Subtypen bildet.

Abstract

Renal cell carcinoma in children and adolescents is rare and comprises only about 1% of renal tumors in this age group. Since the last WHO classification in 1997, new entities of renal tumors in young patients have been described and have been included into the new 2004 WHO renal cell carcinoma classification.

Renal cell carcinoma associated with neuroblastoma comprises 2.5% of renal cell carcinoma in young patients. It occurs several years after neuroblastoma. A large proportion of these tumors show allelic imbalance of chromosomes 20q13, 2p31–32.2, 13q22 and 14q31.

TFE3-translocation carcinomas correspond to approximately 20% of renal cell carcinomas in the pediatric and adolescent age group. Both translocations t(X;17)(p11.2;q25) and t(X;1)(p11.2;q21.2) result in immunohistochemically detectable TFE3 protein overexpression. By conventional morphology, TFE3-translocation carcinomas typically show prominent “voluminous” clear cytoplasm and partially papillary architecture.

Even according to the revised 2004 WHO classification, in children and adolescents, far more renal cell carcinomas than in the adult age group are currently not classifiable but constitute a phenotypically heterogeneous group with ample potential for future renal cell carcinoma subtypes.

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  1. Institut für Pathologie der Universität Basel

    E. Bruder

  2. Institut für Klinische Pathologie, Department Pathologie, USZ, Universität Zürich

    H. Moch

  3. Institut für Pathologie der Universität Basel, Schönbeinstraße 40, 4031, Basel, Schweiz

    E. Bruder

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  1. E. Bruder
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Correspondence to E. Bruder.

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Bruder, E., Moch, H. Nierenzellkarzinome bei jungen Patienten. Pathologe 25, 324–327 (2004). https://doi.org/10.1007/s00292-004-0699-0

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  • DOI: https://doi.org/10.1007/s00292-004-0699-0

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