The question of familial meningiomas and schwannomas:

Meningiomas and schwannomas are common CNS neoplasms, but patients harboring multiple meningiomas or schwannomas are rare. The finding of family members also with multiple meningiomas or schwannomas suggests an inherited cancer predisposition syndrome, such as neurofibromatosis 2 (NF2). In the patient without a family history or insufficient criteria for NF2, the diagnosis of schwannomatosis or meningiomatosis may be entertained. Because it is unclear whether these conditions are distinct clinical entities or represent variants of NF2 (i.e., “NF2A” or “NF2B”),1 Antinheimo et al. undertook a careful analysis of the frequency of meningiomatosis and schwannomatosis in a well-defined population.2 Their results provide important information regarding the evaluation of patients presenting with these common CNS tumors.

In their study, the incidence and familial association of meningiomatosis and schwannomatosis were estimated and the relationship to NF2 was determined.2 Patients derived from a large catchment area of 1.7 million people listed in two reliable population and cancer registries in Finland. The authors determined that isolated schwannomatosis (2% of cases) or meningiomatosis (4% of cases) was rare in patients without clinical or radiographic evidence of NF2. Because it was not clear whether …