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Familial multiple meningioma

MedGen UID:
1644853
•Concept ID:
C4707361
•
Neoplastic Process
Synonyms: Familial multiple benign meningioma; familial multiple meningioma
SNOMED CT: Familial multiple benign meningioma (765202001)
Modes of inheritance:
Autosomal dominant inheritance
MedGen UID:
141047
•Concept ID:
C0443147
•
Intellectual Product
Source: Orphanet
A mode of inheritance that is observed for traits related to a gene encoded on one of the autosomes (i.e., the human chromosomes 1-22) in which a trait manifests in heterozygotes. In the context of medical genetics, an autosomal dominant disorder is caused when a single copy of the mutant allele is present. Males and females are affected equally, and can both transmit the disorder with a risk of 50% for each child of inheriting the mutant allele.
Autosomal dominant inheritance (Orphanet)
 
Monarch Initiative: MONDO:0016995
Orphanet: ORPHA263662

Definition

A rare benign neoplasm of the central nervous system characterised by the development of multiple or rarely solitary meningioma in two or more blood relatives without other apparent syndromic manifestations. Depending on the localisation, growth rate and size of the tumours, patients can present with subtle, gradually worsening or abrupt and severe neurological compromise or can be completely asymptomatic. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • Familial multiple meningioma

Professional guidelines

PubMed

Diagnosis and Management of Hereditary Meningioma and Vestibular Schwannoma.
Shaw A
Recent Results Cancer Res 2016;205:17-27. doi: 10.1007/978-3-319-29998-3_2. PMID: 27075346
Differential Diagnosis of Meningeal SFT-HPC and Meningioma: Which Immunohistochemical Markers Should Be Used?
Macagno N, Figarella-Branger D, Mokthari K, Metellus P, Jouvet A, Vasiljevic A, Loundou A, Bouvier C
Am J Surg Pathol 2016 Feb;40(2):270-8. doi: 10.1097/PAS.0000000000000526. PMID: 26448189
Management of endocrino-metabolic dysfunctions after allogeneic hematopoietic stem cell transplantation.
Vantyghem MC, Cornillon J, Decanter C, Defrance F, Karrouz W, Leroy C, Le Mapihan K, Couturier MA, De Berranger E, Hermet E, Maillard N, Marcais A, Francois S, Tabrizi R, Yakoub-Agha I; Société Française de Thérapie Cellulaire
Orphanet J Rare Dis 2014 Oct 29;9:162. doi: 10.1186/s13023-014-0162-0. PMID: 25496809Free PMC Article

Recent clinical studies

Etiology

Neurofibromatoses.
Jordan JT, Plotkin SR
Hematol Oncol Clin North Am 2022 Feb;36(1):253-267. Epub 2021 Oct 27 doi: 10.1016/j.hoc.2021.08.010. PMID: 34756486
Integrated Molecular-Morphologic Meningioma Classification: A Multicenter Retrospective Analysis, Retrospectively and Prospectively Validated.
Maas SLN, Stichel D, Hielscher T, Sievers P, Berghoff AS, Schrimpf D, Sill M, Euskirchen P, Blume C, Patel A, Dogan H, Reuss D, Dohmen H, Stein M, Reinhardt A, Suwala AK, Wefers AK, Baumgarten P, Ricklefs F, Rushing EJ, Bewerunge-Hudler M, Ketter R, Schittenhelm J, Jaunmuktane Z, Leu S, Greenway FEA, Bridges LR, Jones T, Grady C, Serrano J, Golfinos J, Sen C, Mawrin C, Jungk C, Hänggi D, Westphal M, Lamszus K, Etminan N, Jungwirth G, Herold-Mende C, Unterberg A, Harter PN, Wirsching HG, Neidert MC, Ratliff M, Platten M, Snuderl M, Aldape KD, Brandner S, Hench J, Frank S, Pfister SM, Jones DTW, Reifenberger G, Acker T, Wick W, Weller M, Preusser M, von Deimling A, Sahm F; German Consortium on Aggressive Meningiomas (KAM)
J Clin Oncol 2021 Dec 1;39(34):3839-3852. Epub 2021 Oct 7 doi: 10.1200/JCO.21.00784. PMID: 34618539Free PMC Article
Epidemiology of meningiomas.
Baldi I, Engelhardt J, Bonnet C, Bauchet L, Berteaud E, Grüber A, Loiseau H
Neurochirurgie 2018 Mar;64(1):5-14. Epub 2014 Sep 22 doi: 10.1016/j.neuchi.2014.05.006. PMID: 25249493
Epidemiology and etiology of meningioma.
Wiemels J, Wrensch M, Claus EB
J Neurooncol 2010 Sep;99(3):307-14. Epub 2010 Sep 7 doi: 10.1007/s11060-010-0386-3. PMID: 20821343Free PMC Article
Meningiomas and neurofibromatosis.
Goutagny S, Kalamarides M
J Neurooncol 2010 Sep;99(3):341-7. Epub 2010 Aug 17 doi: 10.1007/s11060-010-0339-x. PMID: 20714782

Diagnosis

Disease-Based Prognostication: Neuro-Oncology.
Waite KA, Cioffi G, Malkin MG, Barnholtz-Sloan JS
Semin Neurol 2023 Oct;43(5):768-775. Epub 2023 Sep 26 doi: 10.1055/s-0043-1775751. PMID: 37751857
Clinical implications of the 2021 edition of the WHO classification of central nervous system tumours.
Horbinski C, Berger T, Packer RJ, Wen PY
Nat Rev Neurol 2022 Sep;18(9):515-529. Epub 2022 Jun 21 doi: 10.1038/s41582-022-00679-w. PMID: 35729337
Integrated Molecular-Morphologic Meningioma Classification: A Multicenter Retrospective Analysis, Retrospectively and Prospectively Validated.
Maas SLN, Stichel D, Hielscher T, Sievers P, Berghoff AS, Schrimpf D, Sill M, Euskirchen P, Blume C, Patel A, Dogan H, Reuss D, Dohmen H, Stein M, Reinhardt A, Suwala AK, Wefers AK, Baumgarten P, Ricklefs F, Rushing EJ, Bewerunge-Hudler M, Ketter R, Schittenhelm J, Jaunmuktane Z, Leu S, Greenway FEA, Bridges LR, Jones T, Grady C, Serrano J, Golfinos J, Sen C, Mawrin C, Jungk C, Hänggi D, Westphal M, Lamszus K, Etminan N, Jungwirth G, Herold-Mende C, Unterberg A, Harter PN, Wirsching HG, Neidert MC, Ratliff M, Platten M, Snuderl M, Aldape KD, Brandner S, Hench J, Frank S, Pfister SM, Jones DTW, Reifenberger G, Acker T, Wick W, Weller M, Preusser M, von Deimling A, Sahm F; German Consortium on Aggressive Meningiomas (KAM)
J Clin Oncol 2021 Dec 1;39(34):3839-3852. Epub 2021 Oct 7 doi: 10.1200/JCO.21.00784. PMID: 34618539Free PMC Article
An update on the CNS manifestations of neurofibromatosis type 2.
Coy S, Rashid R, Stemmer-Rachamimov A, Santagata S
Acta Neuropathol 2020 Apr;139(4):643-665. Epub 2019 Jun 4 doi: 10.1007/s00401-019-02029-5. PMID: 31161239Free PMC Article
Epidemiology and etiology of meningioma.
Wiemels J, Wrensch M, Claus EB
J Neurooncol 2010 Sep;99(3):307-14. Epub 2010 Sep 7 doi: 10.1007/s11060-010-0386-3. PMID: 20821343Free PMC Article

Therapy

Alliance A071401: Phase II Trial of Focal Adhesion Kinase Inhibition in Meningiomas With Somatic NF2 Mutations.
Brastianos PK, Twohy EL, Gerstner ER, Kaufmann TJ, Iafrate AJ, Lennerz J, Jeyapalan S, Piccioni DE, Monga V, Fadul CE, Schiff D, Taylor JW, Chowdhary SA, Bettegowda C, Ansstas G, De La Fuente M, Anderson MD, Shonka N, Damek D, Carrillo J, Kunschner-Ronan LJ, Chaudhary R, Jaeckle KA, Senecal FM, Kaley T, Morrison T, Thomas AA, Welch MR, Iwamoto F, Cachia D, Cohen AL, Vora S, Knopp M, Dunn IF, Kumthekar P, Sarkaria J, Geyer S, Carrero XW, Martinez-Lage M, Cahill DP, Brown PD, Giannini C, Santagata S, Barker FG 2nd, Galanis E
J Clin Oncol 2023 Jan 20;41(3):618-628. Epub 2022 Oct 26 doi: 10.1200/JCO.21.02371. PMID: 36288512Free PMC Article
Clinical implications of the 2021 edition of the WHO classification of central nervous system tumours.
Horbinski C, Berger T, Packer RJ, Wen PY
Nat Rev Neurol 2022 Sep;18(9):515-529. Epub 2022 Jun 21 doi: 10.1038/s41582-022-00679-w. PMID: 35729337
Phase 2 study of pembrolizumab in patients with recurrent and residual high-grade meningiomas.
Brastianos PK, Kim AE, Giobbie-Hurder A, Lee EQ, Wang N, Eichler AF, Chukwueke U, Forst DA, Arrillaga-Romany IC, Dietrich J, Corbin Z, Moliterno J, Baehring J, White M, Lou KW, Larson J, de Sauvage MA, Evancic K, Mora J, Nayyar N, Loeffler J, Oh K, Shih HA, Curry WT, Cahill DP, Barker FG, Gerstner ER, Santagata S
Nat Commun 2022 Mar 14;13(1):1325. doi: 10.1038/s41467-022-29052-7. PMID: 35289329Free PMC Article
Transforming Growth Factor Beta Family in the Pathogenesis of Meningiomas.
Johnson MD
World Neurosurg 2017 Aug;104:113-119. Epub 2017 Mar 23 doi: 10.1016/j.wneu.2017.03.058. PMID: 28344175
Mifepristone (RU486): a review.
Mahajan DK, London SN
Fertil Steril 1997 Dec;68(6):967-76. doi: 10.1016/s0015-0282(97)00189-1. PMID: 9418681

Prognosis

Disease-Based Prognostication: Neuro-Oncology.
Waite KA, Cioffi G, Malkin MG, Barnholtz-Sloan JS
Semin Neurol 2023 Oct;43(5):768-775. Epub 2023 Sep 26 doi: 10.1055/s-0043-1775751. PMID: 37751857
Integrated Molecular-Morphologic Meningioma Classification: A Multicenter Retrospective Analysis, Retrospectively and Prospectively Validated.
Maas SLN, Stichel D, Hielscher T, Sievers P, Berghoff AS, Schrimpf D, Sill M, Euskirchen P, Blume C, Patel A, Dogan H, Reuss D, Dohmen H, Stein M, Reinhardt A, Suwala AK, Wefers AK, Baumgarten P, Ricklefs F, Rushing EJ, Bewerunge-Hudler M, Ketter R, Schittenhelm J, Jaunmuktane Z, Leu S, Greenway FEA, Bridges LR, Jones T, Grady C, Serrano J, Golfinos J, Sen C, Mawrin C, Jungk C, Hänggi D, Westphal M, Lamszus K, Etminan N, Jungwirth G, Herold-Mende C, Unterberg A, Harter PN, Wirsching HG, Neidert MC, Ratliff M, Platten M, Snuderl M, Aldape KD, Brandner S, Hench J, Frank S, Pfister SM, Jones DTW, Reifenberger G, Acker T, Wick W, Weller M, Preusser M, von Deimling A, Sahm F; German Consortium on Aggressive Meningiomas (KAM)
J Clin Oncol 2021 Dec 1;39(34):3839-3852. Epub 2021 Oct 7 doi: 10.1200/JCO.21.00784. PMID: 34618539Free PMC Article
An update on the CNS manifestations of neurofibromatosis type 2.
Coy S, Rashid R, Stemmer-Rachamimov A, Santagata S
Acta Neuropathol 2020 Apr;139(4):643-665. Epub 2019 Jun 4 doi: 10.1007/s00401-019-02029-5. PMID: 31161239Free PMC Article
Epidemiology of meningiomas.
Baldi I, Engelhardt J, Bonnet C, Bauchet L, Berteaud E, Grüber A, Loiseau H
Neurochirurgie 2018 Mar;64(1):5-14. Epub 2014 Sep 22 doi: 10.1016/j.neuchi.2014.05.006. PMID: 25249493
Meningiomas and neurofibromatosis.
Goutagny S, Kalamarides M
J Neurooncol 2010 Sep;99(3):341-7. Epub 2010 Aug 17 doi: 10.1007/s11060-010-0339-x. PMID: 20714782

Clinical prediction guides

Clinical implementation of integrated molecular-morphologic risk prediction for meningioma.
Hielscher T, Sill M, Sievers P, Stichel D, Brandner S, Jones DTW, von Deimling A, Sahm F, Maas SLN
Brain Pathol 2023 May;33(3):e13132. Epub 2022 Nov 14 doi: 10.1111/bpa.13132. PMID: 36377252Free PMC Article
Integrated Molecular-Morphologic Meningioma Classification: A Multicenter Retrospective Analysis, Retrospectively and Prospectively Validated.
Maas SLN, Stichel D, Hielscher T, Sievers P, Berghoff AS, Schrimpf D, Sill M, Euskirchen P, Blume C, Patel A, Dogan H, Reuss D, Dohmen H, Stein M, Reinhardt A, Suwala AK, Wefers AK, Baumgarten P, Ricklefs F, Rushing EJ, Bewerunge-Hudler M, Ketter R, Schittenhelm J, Jaunmuktane Z, Leu S, Greenway FEA, Bridges LR, Jones T, Grady C, Serrano J, Golfinos J, Sen C, Mawrin C, Jungk C, Hänggi D, Westphal M, Lamszus K, Etminan N, Jungwirth G, Herold-Mende C, Unterberg A, Harter PN, Wirsching HG, Neidert MC, Ratliff M, Platten M, Snuderl M, Aldape KD, Brandner S, Hench J, Frank S, Pfister SM, Jones DTW, Reifenberger G, Acker T, Wick W, Weller M, Preusser M, von Deimling A, Sahm F; German Consortium on Aggressive Meningiomas (KAM)
J Clin Oncol 2021 Dec 1;39(34):3839-3852. Epub 2021 Oct 7 doi: 10.1200/JCO.21.00784. PMID: 34618539Free PMC Article
Visual loss and recovery in chiasmal compression.
Danesh-Meyer HV, Yoon JJ, Lawlor M, Savino PJ
Prog Retin Eye Res 2019 Nov;73:100765. Epub 2019 Jun 14 doi: 10.1016/j.preteyeres.2019.06.001. PMID: 31202890
Management of multiple meningiomas.
Tsermoulas G, Turel MK, Wilcox JT, Shultz D, Farb R, Zadeh G, Bernstein M
J Neurosurg 2018 May;128(5):1403-1409. Epub 2017 Jul 21 doi: 10.3171/2017.2.JNS162608. PMID: 28731398
Epidemiology of meningiomas.
Baldi I, Engelhardt J, Bonnet C, Bauchet L, Berteaud E, Grüber A, Loiseau H
Neurochirurgie 2018 Mar;64(1):5-14. Epub 2014 Sep 22 doi: 10.1016/j.neuchi.2014.05.006. PMID: 25249493

Recent systematic reviews

Frailty and postoperative outcomes in brain tumor patients: a systematic review subdivided by tumor etiology.
Qureshi HM, Tabor JK, Pickens K, Lei H, Vasandani S, Jalal MI, Vetsa S, Elsamadicy A, Marianayagam N, Theriault BC, Fulbright RK, Qin R, Yan J, Jin L, O'Brien J, Morales-Valero SF, Moliterno J
J Neurooncol 2023 Sep;164(2):299-308. Epub 2023 Aug 25 doi: 10.1007/s11060-023-04416-1. PMID: 37624530Free PMC Article
Associations of race and socioeconomic status with outcomes after intracranial meningioma resection: a systematic review and meta-analysis.
Lei H, Tabor JK, O'Brien J, Qin R, Pappajohn AF, Chavez MAM, Morales-Valero SF, Moliterno J
J Neurooncol 2023 Jul;163(3):529-539. Epub 2023 Jul 13 doi: 10.1007/s11060-023-04393-5. PMID: 37440095
Management experience of pediatric meningioma in a Mexican reference center.
Santana-González A, Pérez-Ramírez M, Ramírez-Reyes G, Sánchez-Rodríguez G, Ferman-Cano F, López-Aguilar E, Siordia-Reyes AG
Ann Diagn Pathol 2021 Dec;55:151811. Epub 2021 Sep 1 doi: 10.1016/j.anndiagpath.2021.151811. PMID: 34500134
Surveillance for subsequent neoplasms of the CNS for childhood, adolescent, and young adult cancer survivors: a systematic review and recommendations from the International Late Effects of Childhood Cancer Guideline Harmonization Group.
Bowers DC, Verbruggen LC, Kremer LCM, Hudson MM, Skinner R, Constine LS, Sabin ND, Bhangoo R, Haupt R, Hawkins MM, Jenkinson H, Khan RB, Klimo P Jr, Pretorius P, Ng A, Reulen RC, Ronckers CM, Sadighi Z, Scheinemann K, Schouten-van Meeteren N, Sugden E, Teepen JC, Ullrich NJ, Walter A, Wallace WH, Oeffinger KC, Armstrong GT, van der Pal HJH, Mulder RL
Lancet Oncol 2021 May;22(5):e196-e206. Epub 2021 Apr 9 doi: 10.1016/S1470-2045(20)30688-4. PMID: 33845037

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