Von Recklinghausen's disease and melanoma of the uvea.
نویسندگان
چکیده
In I882 Professor von Recklinghausen, Director of the Institute of Pathology at our University published his original work. "Ober die multiplen Fibrome der Haut und ihre Beziehung zu den multiplen Neuromen". Since that time neurofibromatosis, which bears his name, has been the subject of many reports; in addition to the cutaneous lesions characterized by multiple neuromata and cafi au lait spots, numerous other manifestations have been described. Our understanding of these clinical features has been clarified by the work of Masson (I926), Director of the same Institute, and of Oberling (I922), his associate professor from I919 to I926. The former showed the close relationship between Schwann cells and dermal melanoblasts and the latter recognized the nature of the meningoblast as homologous to the Schwann cell. In most cases the neurofibromatosis of von Recklinghausen becomes a disease of the schwannian system, but the neoplasia of the nervous system proper still requires to be explained. Fig. i shows that, if the Schwann cells and pigment cells arise from the neural crest, this derives from the primitive neuroectoderm, which also gives rise to the other neural elements. When development extends beyond the cells of Schwann-though this is relatively rare-any differentiation of the neuroectoderm can result.
منابع مشابه
Primary tumours of the optic nerve in von Recklinghausen's disease.
THE ophthalmological symptoms of von Recklinghausen's syndrome are numerous. Multiple tumours of the superficial nerves may cause a plexiform neurofibromatosis, preferably situated in the superior eyelid and the region around the orbit. The intra-orbital and the ciliary nerves can also be affected. Defects in the bony orbital wall may cause a pulsating exophthalmos, and phakomata in the iris an...
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A 32-year-old woman who died suddenly during pregnancy as a result of an adrenal phaeochromocytoma also suffered from von Recklinghausen's disease. 21 years earlier she had an optic nerve glioma successfully removed. The association between von Reckinghausen's disease and optic nerve glioma/phaeochromocytoma is well established. So far as is known, the present case is the first ever published w...
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The case history is presented of a 61 year old man with von Recklinghausen's disease who developed a spontaneous haemothorax. In spite of being asymptomatic for five days after drainage, he died as a result of fatal sudden re-bleeding. The post mortem examination showed dissection and rupture of the left subclavian artery. Microscopically, disarrangement of smooth muscle and decrease of elastic...
متن کاملEndotracheal neurofibroma in a patient with von Recklinghausen's disease.
Benign neurogenous tumours arising in the trachea are rare. We report on a patient with von Recklinghausen's disease who presented with shortness of breath caused by endotracheal neurofibroma.
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عنوان ژورنال:
- The British journal of ophthalmology
دوره 54 10 شماره
صفحات -
تاریخ انتشار 1970