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Retinoblastom und Retinozytom (Retinom)

Retinoblastoma and retinocytoma (retinoma)

  • Schwerpunkt: Tumoren der Sinnesorgane
  • Published:
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Zusammenfassung

Retinoblastome sind außerordentlich seltene, in ca. der Hälfte der Fälle erbliche frühkindliche Augenneoplasien. Sie zeigen hochproliferative, CRX-positive („cone-rod homeobox protein“) undifferenzierte Tumorzellen (z. T. auch mit Rosettenbildung). Das Staging richtet sich nach der pTNM-Klassifikation und dem „International Retinoblastoma Staging System“ (IRSS). Die Grundlage für eine eventuelle systemische Chemotherapie und/oder Radiatio ist der Nachweis einer postlaminären Optikusinfiltration, ausgeprägten Choroideainfiltration, Sklerainvasion oder sogar extraokulärer Ausbreitung. In morphologisch schwierigen Fällen sind immunhistochemische Untersuchungen (CRX und Ki67) äußerst hilfreich. Retinozytome (Retinome) zeigen keine undifferenzierten Tumorareale, sondern ausschließlich eine Photorezeptordifferenzierung sowie eine mittels Ki67 nachgewiesene auffallend geringe Proliferation.

Abstract

Retinoblastomas are extremely rare withabout half of the cases being hereditary eye neoplasms in young children. They show highly proliferative, CRX-positive undifferentiated tumour cells (occasionally forming rosettes). Staging is performed according to the pTNM classification and the International Retinoblastoma Staging System (IRSS). The diagnostic basis for systemic administration of chemotherapy and/or radiation is postlaminar optic nerve invasion, massive choroidal tumour infiltration, scleral invasion or even extraocular invasion. In cases that are difficult to diagnose, immunohistochemical stains (CRX and Ki67) may be very helpful. Retinocytomas (retinomas) completely lack undifferentiated tumour cell areas, show an exclusive photoreceptor differentiation and an exceptionally low Ki67 index.

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Authors and Affiliations

  1. Institut für Pathologie, Universitätsklinikum Essen, Universität Duisburg-Essen, Hufelandstr. 55, 45147, Essen, Deutschland

    K. A. Metz, D. Westerwick, F. Driever & K. W. Schmid

  2. Klinik für Augenheilkunde, Universitätsklinikum Essen, Universität Duisburg-Essen, Essen, Deutschland

    C. H. D. Le Guin

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  1. K. A. Metz
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  2. D. Westerwick
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  3. F. Driever
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  5. C. H. D. Le Guin
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Correspondence to K. A. Metz.

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Interessenkonflikt

K. . Metz, D. Westerwick, F. Driever, K.W. Schmid und C.H.D. Le Guin geben an, dass kein Interessenkonflikt besteht.

Dieser Beitrag beinhaltet keine von den Autoren durchgeführten Studien an Menschen oder Tieren.

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F. Fend, Tübingen

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Metz, K.A., Westerwick, D., Driever, F. et al. Retinoblastom und Retinozytom (Retinom). Pathologe 38, 507–514 (2017). https://doi.org/10.1007/s00292-017-0384-8

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  • DOI: https://doi.org/10.1007/s00292-017-0384-8

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