KEGG   DISEASE: MeningiomaHelp
Entry
H01556                      Disease                                

Name
Meningioma
Description
Meningiomas are the second-most common central nervous system tumor in adults. These tumors arise from arachnoid cells of the meninges, the covering layer of the brain. The majority of meningiomas tend to be benign, localized, and non-invasive. However, some meningiomas tend to be more aggressive with tendencies toward invasion of the surrounding brain, high propensity for recurrence, and in rare cases extracranial metastasis. Hereditary factors and ionizing radiation play an important role in the initiation of at least some meningiomas. Inactivation of the NF2 tumor suppressor gene is likely responsible for the initiation of more than half of all meningiomas and may cause a mesenchymal-like cytomorphology. Recently, novel mutations have been discovered in non-NF2 meningiomas. The somatic mutations in components of the SHH-GLI1 and AKT1-MTOR signaling pathways indicates the potential for cross talk of these pathways in the development of meningiomas.
Category
Cancer
Brite
Human diseases [BR:br08402]
 Cancers
  Cancers of eye, brain, and central nervous system
   H01556  Meningioma
Human diseases in ICD-11 classification [BR:br08403]
 02 Neoplasms
  Neoplasms of brain or central nervous system
   2A01  Primary neoplasms of meninges
    H01556  Meningioma
BRITE hierarchy
Gene
NF2 (mutation) [HSA:4771] [KO:K16684]
SMARCB1 (mutation) [HSA:6598] [KO:K11648]
SMARCE1 (mutation) [HSA:6605] [KO:K11651]
SUFU (mutation) [HSA:51684] [KO:K06229]
PTEN (mutation) [HSA:5728] [KO:K01110]
CDKN2A (mutation) [HSA:1029] [KO:K06621]
CDKN2B (mutation) [HSA:1030] [KO:K04685]
TRAF7 (mutation) [HSA:84231] [KO:K10646]
AKT1 (mutation) [HSA:207] [KO:K04456]
KLF4 (mutation) [HSA:9314] [KO:K17846]
SMO (mutation) [HSA:6608] [KO:K06226]
Carcinogen
Ionising radiation
Comment
ICD-O-3: 9530/0 Meningioma, NOS
ICD-O-3: 9530/1 Meningiomatosis, NOS
ICD-O-3: 9230/3 Meningioma, malignant
ICD-O-3: 9531/0 Meningothelial meningioma
ICD-O-3: 9532/0 Fibrous meningioma
ICD-O-3: 9533/0 Psammomatous meningioma
ICD-O-3: 9534/0 Angiomatous meningioma
ICD-O-3: 9535/0 Hemangioblastic meningioma
ICD-O-3: 9537/0 Transitional meningioma
ICD-O-3: 9538/1 Clear cell meningioma
ICD-O-3: 9538/3 Papillary meningioma
ICD-O-3: 9539/1 Atypical meningioma
Other DBs
ICD-11: 2A01.00
ICD-10: D32.9
MeSH: D008579
Reference
  Authors
Smith MJ
  Title
Germline and somatic mutations in meningiomas.
  Journal
Cancer Genet 208:107-14 (2015)
DOI:10.1016/j.cancergen.2015.02.003
Reference
  Authors
Miller R Jr, DeCandio ML, Dixon-Mah Y, Giglio P, Vandergrift WA 3rd, Banik NL, Patel SJ, Varma AK, Das A
  Title
Molecular Targets and Treatment of Meningioma.
  Journal
J Neurol Neurosurg 1:1000101 (2014)
Reference
PMID:21529177 (gene, drug)
  Authors
Choy W, Kim W, Nagasawa D, Stramotas S, Yew A, Gopen Q, Parsa AT, Yang I
  Title
The molecular genetics and tumor pathogenesis of meningiomas and the future directions of meningioma treatments.
  Journal
Neurosurg Focus 30:E6 (2011)
DOI:10.3171/2011.2.FOCUS1116
Reference
PMID:24289126 (drug)
  Authors
Moazzam AA, Wagle N, Zada G
  Title
Recent developments in chemotherapy for meningiomas: a review.
  Journal
Neurosurg Focus 35:E18 (2013)
DOI:10.3171/2013.10.FOCUS13341
Reference
PMID:23463172 (drug, ICD-O-3)
  Authors
Fathi AR, Roelcke U
  Title
Meningioma.
  Journal
Curr Neurol Neurosci Rep 13:337 (2013)
DOI:10.1007/s11910-013-0337-4
Reference
  Authors
Shibuya M
  Title
Pathology and molecular genetics of meningioma: recent advances.
  Journal
Neurol Med Chir (Tokyo) 55:14-27 (2015)
DOI:10.2176/nmc.ra.2014-0233
Reference
PMID:19194129 (carcinogen)
  Authors
Campbell BA, Jhamb A, Maguire JA, Toyota B, Ma R
  Title
Meningiomas in 2009: controversies and future challenges.
  Journal
Am J Clin Oncol 32:73-85 (2009)
DOI:10.1097/COC.0b013e31816fc920
Reference
PMID:17110285 (ICD-O-3)
  Authors
Riemenschneider MJ, Perry A, Reifenberger G
  Title
Histological classification and molecular genetics of meningiomas.
  Journal
Lancet Neurol 5:1045-54 (2006)
DOI:10.1016/S1474-4422(06)70625-1
Reference
  Authors
Simon M, Bostrom JP, Hartmann C
  Title
Molecular genetics of meningiomas: from basic research to potential clinical applications.
  Journal
Neurosurgery 60:787-98; discussion 787-98 (2007)
DOI:10.1227/01.NEU.0000255421.78431.AE

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