PubMed1975
Since 1933, four families have been found in Marseilles with bilateral tumours of the acoustic nerve spread over three generations in three families. The fourth only showing a cutaneous neuro-fibromatosis in the second generation. The authors recall briefly the clinical characteristics and compare their cases with similar ones found in the literature. Bilateral and familial neurinomas of the VIIIth cranial verbes were found in 21 families, making a total of 111 cases, unilateral neurinoma being used as a term of comparison. Among the groups of cases published, the family observed by Gardner and Frazier is the most remarkable (43 cases over 6 generations). From the hereditary point of view, the phenomenon of antiposition is not always constant. From the evolutionary point of view, bilateral neurinomas manifest themselves earlier clinically than unilateral ones. As far as associations are concerned, cutaneous manifestations of neuro-fibromatosis are the most frequent if the neurinomas are bilateral and non familia. Among tumors of the nervous system, meningiomas and gliomas have been found, other dysembryoplastic or degenerative tumoral associations being rare. The incidence of cutaneous symptoms is very low when the neurioma is unilateral. The association of cutaneous symptoms with bilateral and familial neurinomas does not worsen the prognosis, but the presence of such cutaneous signs is significantly more serious if the neurinoma is isolated. These considerations do no more than touch the fringes of the problems raised by these sporadic or hereditary tumours of the VIII the cranial nerves, developing within the framework of phacomatosis. Great caution is necessary in assessing the correlations observed in view of the narrow range of statistics available.