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Les neurofibromes de la maladie de Recklinghausen

Neurofibromas in von Recklinghausen's Disease. Ultrastructural study and nosologic relation to neurinomas

Etude ultrastructurale et place nosologique par rapport aux neurinomes

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Summary

Six neurofibromas from patients with von Recklinghausen's disease have been examined by light and electron microscopy: 2 types of cells have been found (Schwann cells and fibroblasts) scattered in extracellular spaces rich in collagen fibers. Myelinated and nonmyelinated axons were observed.

These data have been compared with other ones collected from 3 acoustic neurinomas. These latter contained only one cellular type (very likely to be Schwann cell), much less collagen and no axon at all. A pathogenic and nosologic discussion is given.

Résumé

L'examen en microscopie optique et électronique de 6 neurofibromes prélevés chirurgicalement chez des patients atteints de maladie de Recklinghausen autorise un certain nombre de conclusions:

l'ultrastructure des neurofibromes est faite de 2 types de cellules (cellules de Schwann et fibroblastes) dispersées au sein d'un espace extra-cellulaire contenant de nombreuses fibres de collagène. Des axones myélinisés et amyéliniques sont présents;

l'aspect ultrastructural de 3 neurinomes de l'acoustique a été comparé à celui des neurofibromes et apparaît comme différent. Les neurinomes sont faits d'un seul type cellulaire (très probablement cellule de Schwann), le collagène y est beaucoup moins abondant, les axones absents. Quelques déductions pathogéniques et nosologiques sont proposées.

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Authors and Affiliations

  1. Laboratoire de Neuropathologie «Charles Foix». Clinique des Maladies du Système Nerveux, Hôpital de la Salpêtrière, Paris 13e

    J. Poirier, R. Escourolle & P. Castaigne

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  1. J. Poirier
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  2. R. Escourolle
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  3. P. Castaigne
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Poirier, J., Escourolle, R. & Castaigne, P. Les neurofibromes de la maladie de Recklinghausen. Acta Neuropathol 10, 279–294 (1968). https://doi.org/10.1007/BF00690704

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