Meningiomas in Genetic and Familial Syndromes: Biomolecular Correlations

Meningiomas can be sporadic, hereditary, or radiation-induced. In fact, there are many tumor predisposition syndromes that place a patient at increased risk for meningioma development. The most common genetic syndrome causing meningiomas is NF2 related Schwannomatosis. Two others schwannomatosis SMARCB1-related schwannomatosis and LZTR1-related schwannomatosis can also predispose to the development of those tumors. SMARCE1 syndrome increased risk for spinal and intracranial clear cell meningiomas in young patients. In NBCCS syndrome, meningiomas may be more frequent among patients with SUFU variant compared to PTCH1 variant. At least meningioma is one of the rare tumors that can occur in BAP1 tumor predisposition syndrome.