Summary
An embryonal rhabdomyosarcoma of the nasopharynx of a 10 year old boy is analysed with light and electron microscopy. With regard to cell shape and cytoplasmic features the following four tumour cell types could be distinguished: 1. Undifferentiated mesenchymal cells with a big loosely packed nucleus and a small cytoplasmic rim with only few cell organelles; 2. Undifferentiated tumour cells with a broad cytoplasmic body which contains a dense network of nonspecific intermediate filaments with a diameter of about 100 Å; 3. Immature rhabdomyoblasts with randomly orientated specific myofilaments; 4. Fully differentiated rhabdomyoblasts with well developed myofibrils often showing a sarcomeric pattern. Glycogen deposits which were seen in great masses in many tumour cells were regarded to result from degenerative processes within the tumour. The cellular stages in the development of rhabdomyoblasts are basically identical to those known from the embryogenesis and regeneration of striated muscle. From these observations the two following developmental pathways are suggested: 1. Origin of the tumour from an undifferentiated mesenchymal cell; 2. Atypical regeneration of striated muscle which terminates in malignant progressive tumour growth. At present, the body of information about rhabdomyosarcomas supports the assumption of an origin from immature mesenchymal cells. Nevertheless, the second theory cannot be totally excluded.
Zusammenfassung
In der vorliegenden Untersuchung werden die cytomorphologischen Merkmale eines embryonalen Rhabdomyosarkoms des Nasenrachenraumes bei einem 10 jährigen Jungen aufgezeigt. Aufgrund der Ultrastruktur lassen sich im Tumor 4 Zelltypen darstellen: 1. Undifferenzierte mesenchymale Tumorzellen mit großem, euchromatischem Zellkern und schmalem, organellenarmem Cytoplasma; 2. undifferenzierte Tumorzellen mit einem Netzwerk von Intermediärfilamenten (Durchmesser etwa 100 Å) im breiten Cytoplasma; 3. unreife Rhabdomyoblasten mit zunehmender Entwicklung spezifische Myofilamente (Myosin: 180–210 Å, Actin: 68–80 Å Durchmesser); 4. reife Rhabdomyoblasten mit Myofibrillen in sarkomerischer Gliederung (Sarkomerlänge: 1,8 μ). Die starken Glykogenablagerungen in einigen cytoplasmareichen Tumorzellen werden als Zeichen eines Degenerationsprozesses gedeutet.
Die cellulären Entwicklungsstadien des embryonalen Rhabdomyosarkoms sind identisch mit dem Ablauf der Embryogenese sowie der Regeneration normaler quergestreifter Muskulatur. Daher bieten sich für die Entstehung des embryonalen Rhabdomyosarkoms zwei Denkmodelle an: 1. Entstehung des Tumors aus einer undifferenzierten mesenchymalen Tumorstammzelle und 2. Atypische Regeneration nach Schädigung der quergestreiften Muskulatur mit Übergang in ein autonomes Tumorwachstum. Die zweite Hypothese ist jedoch wenig wahrscheinlich, da bis heute weder morphologisch noch experimentell eine derartige Tumorentstehung nachgewiesen werden konnte und zudem ätiologisch gesicherte Faktoren zur Induktion von Rhabdomyosarkomen beim Menschen fehlen.
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Kastendieck, H., Böcker, W. & Hüsselmann, H. Zur Ultrastruktur und formalen Pathogenese des embryonalen Rhabdomyosarkoms. Z. Krebsforsch. 86, 55–68 (1976). https://doi.org/10.1007/BF00304934
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DOI: https://doi.org/10.1007/BF00304934