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Klinik und Morphologie kindlicher Rhabdomyosarkome der Orbita

Orbital rhabdomyosarcoma. Clinicopathological study of 7 cases including electron microscopy and survey of the literature

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Summary

The clinical course of orbital rhabdomyosarcoma is described in seven male children. A survey of the literature on the subject is given. Particular attention is paid to certain diagnostic problems of this most frequent orbital malignant primary in childhood. An adequate combination therapy which results in cure in more than half of the patients should be employed provided that an exact morphological diagnosis is made; this still causes often great difficulty. Electron microscopy is of great value in distinguishing this type of tumor from other malignancies, including the so-called reticulosarcoma. In comparison with the case material of other authors, there was a relatively high rate of late deaths in our patients.

Zusammenfassung

Es wird über die Krankheitsverläufe bei 7 Knaben mit primärem Rhabdomyosarkom der Orbita berichtet und eine Übersicht über diesen häufigsten malignen Orbitatumor im Kindesalter gegeben. Auf die besondere diagnostische Problematik wird eingegangen. Eine exakte morphologische Diagnose, die gegenwärtig beim Rhabdomyosarkom vielfach noch auf erhebliche Schwierigkeiten stößt, ist Voraussetzung einer adäquaten Kombinationstherapie, die Heilungen in mehr als der Hälfte der Fälle ermöglicht. In diesem Zusammenhang wird auf den großen Wert der Elektronenmikroskopie für die Abgrenzung dieser Malignome von anderen Malignomen einschließlich des sog. Retikulosarkoms hingewiesen. Im Vergleich zu den Erhebungen anderer Autoren fällt bei den eigenen Patienten die relativ hohe Rate später Todesfälle auf.

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Gutjahr, P., Leder, LD., Müller-Hermelink, HK. et al. Klinik und Morphologie kindlicher Rhabdomyosarkome der Orbita. Albrecht von Graefes Arch. Klin. Ophthalmol. 196, 113–126 (1975). https://doi.org/10.1007/BF00414797

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