Meningiomas in five members of a family over two generations, in one member simultaneously with acoustic neurinomas

Abstract

Despite four reports of familial occurrence of meningiomas, the inheritance of this tumor remains an open question. We describe a family in which four members in two generations had meningiomas without evidence of neurofibromatosis. Another member of the family, however, had multiple meningiomas and bilateral acoustic neurinomas, while yet another had multiple café-au-lait spots. We suspect that a highly unusual presentation of neurofibromatosis explains this otherwise very unlikely occurrence.

A G-->A transition creates a branch point sequence and activation of a cryptic exon, resulting in the hereditary disorder neurofibromatosis 2

“…The patients mentioned here are members of a family that was described by Delleman et al (18) as having the central form of Von Recklinghausen disease. Figure 2A depicts the updated pedigree.…”

Multiple Intracranial Meningiomas: Case Report and Review of the Literature

“…12 Cases of multiple familial meningiomas without evidence of neurofibromatosis occur. [29][30][31] Multiple spinal canal meningiomas are very rare; about 10 cases from the United States have been reported. 32 A case of multiple meningiomas in a child was also reported.…”

The risk of cancer in relatives of patients with brain neoplasm

“…Familial occurrence of brain tumours, in particular, has been demonstrated in numerous families l, 3,4,10,13 The occurrence of brain tumour in association with malignancies at other sites in the same patient has been frequently reported 2'7' 12,15 and there are several reports of brain tumours and extraneural malignancies among members of the same family 12. Genetic studies of brain tumour patients, however, seem to be rare and have showed contradictory results s' 6, 9…”