Familial multiple meningioma

Familial multiple meningioma is a rare hereditary condition characterized by the development of multiple meningiomas — benign tumors arising from the meninges, the protective membranes that surround the brain and spinal cord. Unlike sporadic meningiomas, which typically occur as single tumors, this familial form presents with two or more meningiomas occurring in multiple family members across generations, suggesting a genetic predisposition. The condition primarily affects the central nervous system, and symptoms depend on the size and location of the tumors. Common manifestations include headaches, seizures, focal neurological deficits (such as weakness, sensory changes, or visual disturbances), and increased intracranial pressure. Some patients may remain asymptomatic for years, with tumors discovered incidentally on neuroimaging. Familial multiple meningioma has been associated with mutations in the SMARCE1 gene in some families, particularly those with clear cell meningioma histology, and in the NF2 gene on chromosome 22q12, which is also implicated in neurofibromatosis type 2. However, not all families carry identifiable mutations in these genes, suggesting additional genetic loci may be involved. It is important to distinguish this condition from neurofibromatosis type 2 (NF2), which also features multiple meningiomas but additionally includes bilateral vestibular schwannomas and other tumor types. Treatment typically involves surgical resection of symptomatic or growing tumors, and radiation therapy (including stereotactic radiosurgery) may be considered for tumors that are not amenable to surgery or in cases of recurrence. Regular surveillance with MRI is recommended for affected individuals and at-risk family members to monitor for new tumor development or growth of existing lesions. Genetic counseling is advised for affected families.

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