RECKLINGHAUSEN'S DISEASE: A MULTIFACETTED SYNDROME

1970; Wiley; Volume: 78A; Issue: 4 Linguagem: Inglês

10.1111/j.1699-0463.1970.tb02523.x

ISSN

0365-4184

Autores

J Schönebeck, Otto Ljungberg,

Tópico(s)

Adrenal and Paraganglionic Tumors

Resumo

A case of multiple neurofibromatosis associated with café‐au‐lait coloured skin spots, a metastasizing para‐aortic phaeochromocytoma and a parathyroid adenoma is reported. Investigation of the patient's family has revealed seven further cases of multiple neurofibromatosis. In four of these café‐au‐lait coloured skin spots and in one bilateral opticus glioma have also been found. The relationship between von Recklinghausen's disease and allied conditions such as the “neuro‐cutaneous”—, “medullary thyroid carcinoma‐phaeochromocytoma”—and “pluriglandular” syndrome is discussed.

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