Familial multiple meningioma

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Familial multiple meningioma

ORPHA:263662Disease

What it is

Familial multiple meningioma is a rare, benign neoplasm of the central nervous system characterized by the development of multiple or, rarely, solitary meningiomas in two or more blood relatives, without other apparent syndromic manifestations. Depending on the localization, growth rate and size of the tumors, patients can present with subtle, gradually worsening or abrupt and severe neurological compromise or can be completely asymptomatic.

Key facts

Age of onset
All ages
Inheritance
Autosomal dominant
Classified as
Disease

Genes

SMARCB1Disease-causing germline mutation(s)
SMARCE1Disease-causing germline mutation(s) (loss of function)
SUFUMajor susceptibility factor
MN1Candidate gene tested
PDGFBCandidate gene tested

ICD-10 codes

D32.9filed under a broader ICD-10 category — shared with 2 other rare diseases

ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.

Cross-references

MONDO 0016995OMIM 607174UMLS C4707361

Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.

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