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Gliomatosis cerebri

MedGen UID:
87267
•Concept ID:
C0334576
•
Neoplastic Process
Synonym: Gliomatosis Cerebri
SNOMED CT: Gliomatosis cerebri (26138003); Gliomatosis cerebri (1156413005)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0016683
Orphanet: ORPHA251582

Definition

A rare glial tumor characterized by extensive infiltration of the brain, often extending to infratentorial structures and even the spinal cord. The tumor corresponds to WHO grade III and is composed of elongated glial cells typically resembling astrocytes. Cases in which the predominant cell type is oligodendroglial have also been described. Some tumors develop a circumscribed neoplastic mass in addition to the diffuse lesion, usually showing features of high-grade glioma. Clinical symptoms include dementia, headache, seizures, signs of increased intracranial pressure, and a variety of neurological deficits. Prognosis is generally poor. [from ORDO]

Professional guidelines

PubMed

T2/FLAIR Hyperintensity in Mesial Temporal Lobe: Challenging Differential Diagnosis.
Moon S
Curr Med Imaging 2022;18(3):285-291. doi: 10.2174/1573405617666210712130555. PMID: 34931987
Perioperative Risk Assessment of Patients with Gliomatosis Cerebri.
Shimony N, Shofty B, Ram Z, Grossman R
World Neurosurg 2017 Feb;98:334-338. Epub 2016 Nov 9 doi: 10.1016/j.wneu.2016.11.014. PMID: 27836704
Gliomatosis cerebri: treatment results with radiotherapy alone.
Elshaikh MA, Stevens GH, Peereboom DM, Cohen BH, Prayson RA, Lee SY, Barnett GH, Suh JH
Cancer 2002 Nov 1;95(9):2027-31. doi: 10.1002/cncr.10909. PMID: 12404298

Recent clinical studies

Etiology

An enduring debate on gliomatosis cerebri.
Akimoto J
Brain Tumor Pathol 2023 Apr;40(2):78-84. Epub 2023 Apr 6 doi: 10.1007/s10014-023-00454-9. PMID: 37022648
WHO 2016 Classification of gliomas.
Wesseling P, Capper D
Neuropathol Appl Neurobiol 2018 Feb;44(2):139-150. doi: 10.1111/nan.12432. PMID: 28815663
Pediatric pseudotumor cerebri.
Phillips PH
Int Ophthalmol Clin 2012 Summer;52(3):51-9, xii. doi: 10.1097/IIO.0b013e31825a12f6. PMID: 22668539
Gliomatosis cerebri.
Sharma S, Kalra V, Garg A, Suri V, Suri A
Indian J Pediatr 2009 May;76(5):553-4. Epub 2009 Apr 23 doi: 10.1007/s12098-009-0090-5. PMID: 19390795
Gliomatosis cerebri.
del Carpio-O'Donovan R, Korah I, Salazar A, Melançon D
Radiology 1996 Mar;198(3):831-5. doi: 10.1148/radiology.198.3.8628879. PMID: 8628879

Diagnosis

WHO 2016 Classification of gliomas.
Wesseling P, Capper D
Neuropathol Appl Neurobiol 2018 Feb;44(2):139-150. doi: 10.1111/nan.12432. PMID: 28815663
Pediatric pseudotumor cerebri.
Phillips PH
Int Ophthalmol Clin 2012 Summer;52(3):51-9, xii. doi: 10.1097/IIO.0b013e31825a12f6. PMID: 22668539
Gliomatosis cerebri.
Herrlinger U
Handb Clin Neurol 2012;105:507-15. doi: 10.1016/B978-0-444-53502-3.00005-7. PMID: 22230515
Gliomatosis cerebri.
Gleizniene R, Bucinskas U, Lukosevicius S, Vaitkus A, Letautiene S, Apanaviciūte D, Galvonaite M
Medicina (Kaunas) 2010;46(5):341-4. PMID: 20679750
Gliomatosis cerebri.
del Carpio-O'Donovan R, Korah I, Salazar A, Melançon D
Radiology 1996 Mar;198(3):831-5. doi: 10.1148/radiology.198.3.8628879. PMID: 8628879

Therapy

Lactoferrin- and RGD-comodified, temozolomide and vincristine-coloaded nanostructured lipid carriers for gliomatosis cerebri combination therapy.
Zhang J, Xiao X, Zhu J, Gao Z, Lai X, Zhu X, Mao G
Int J Nanomedicine 2018;13:3039-3051. Epub 2018 May 22 doi: 10.2147/IJN.S161163. PMID: 29861635Free PMC Article
Nanoscale Functional Biomaterials for Cancer Theranostics.
Liu L, Li X, Chen L, Zhang X
Curr Med Chem 2018;25(25):2987-3000. doi: 10.2174/0929867324666170406111036. PMID: 28393688
Management and Survival Patterns of Patients with Gliomatosis Cerebri: A SEER-Based Analysis.
Carroll KT, Hirshman B, Ali MA, Alattar AA, Brandel MG, Lochte B, Lanman T, Carter B, Chen CC
World Neurosurg 2017 Jul;103:186-193. Epub 2017 Mar 30 doi: 10.1016/j.wneu.2017.03.103. PMID: 28366748
Gliomatosis cerebri: clinical characteristics, management, and outcomes.
Chen S, Tanaka S, Giannini C, Morris J, Yan ES, Buckner J, Lachance DH, Parney IF
J Neurooncol 2013 Apr;112(2):267-75. Epub 2013 Jan 23 doi: 10.1007/s11060-013-1058-x. PMID: 23341100Free PMC Article
Gliomatosis cerebri.
Gleizniene R, Bucinskas U, Lukosevicius S, Vaitkus A, Letautiene S, Apanaviciūte D, Galvonaite M
Medicina (Kaunas) 2010;46(5):341-4. PMID: 20679750

Prognosis

Gliomatosis Cerebri Among Children and Adolescents: An Individual-Patient Data Meta-analysis of 182 Patients.
Georgakis MK, Tsivgoulis G, Pourtsidis A, Petridou ET
J Child Neurol 2019 Jun;34(7):394-401. Epub 2019 Mar 19 doi: 10.1177/0883073819836551. PMID: 30887873
Prognostic Factors and Survival of Gliomatosis Cerebri: A Systematic Review and Meta-Analysis.
Georgakis MK, Tsivgoulis G, Spinos D, Liaskas A, Herrlinger U, Petridou ET
World Neurosurg 2018 Dec;120:e818-e854. Epub 2018 Aug 30 doi: 10.1016/j.wneu.2018.08.173. PMID: 30172970
Incidence and survival of gliomatosis cerebri: a population-based cancer registration study.
Georgakis MK, Spinos D, Pourtsidis A, Psyrri A, Panourias IG, Sgouros S, Petridou ET
J Neurooncol 2018 Jun;138(2):341-349. Epub 2018 Feb 20 doi: 10.1007/s11060-018-2802-z. PMID: 29464663
Gliomatosis cerebri: Prognosis based on current molecular markers.
Maharaj MM, Phan K, Xu J, Fairhall J, Reddy R, Rao PJV
J Clin Neurosci 2017 Sep;43:1-5. Epub 2017 May 20 doi: 10.1016/j.jocn.2017.04.043. PMID: 28539209
Gliomatosis cerebri.
Sharma S, Kalra V, Garg A, Suri V, Suri A
Indian J Pediatr 2009 May;76(5):553-4. Epub 2009 Apr 23 doi: 10.1007/s12098-009-0090-5. PMID: 19390795

Clinical prediction guides

T2/FLAIR Hyperintensity in Mesial Temporal Lobe: Challenging Differential Diagnosis.
Moon S
Curr Med Imaging 2022;18(3):285-291. doi: 10.2174/1573405617666210712130555. PMID: 34931987
Gliomatosis Cerebri Among Children and Adolescents: An Individual-Patient Data Meta-analysis of 182 Patients.
Georgakis MK, Tsivgoulis G, Pourtsidis A, Petridou ET
J Child Neurol 2019 Jun;34(7):394-401. Epub 2019 Mar 19 doi: 10.1177/0883073819836551. PMID: 30887873
Prognostic Factors and Survival of Gliomatosis Cerebri: A Systematic Review and Meta-Analysis.
Georgakis MK, Tsivgoulis G, Spinos D, Liaskas A, Herrlinger U, Petridou ET
World Neurosurg 2018 Dec;120:e818-e854. Epub 2018 Aug 30 doi: 10.1016/j.wneu.2018.08.173. PMID: 30172970
Gliomatosis cerebri: clinical characteristics, management, and outcomes.
Chen S, Tanaka S, Giannini C, Morris J, Yan ES, Buckner J, Lachance DH, Parney IF
J Neurooncol 2013 Apr;112(2):267-75. Epub 2013 Jan 23 doi: 10.1007/s11060-013-1058-x. PMID: 23341100Free PMC Article
Gliomatosis cerebri: a review of 22 cases.
Vates GE, Chang S, Lamborn KR, Prados M, Berger MS
Neurosurgery 2003 Aug;53(2):261-71; discussion 271. doi: 10.1227/01.neu.0000073527.20655.e6. PMID: 12925240

Recent systematic reviews

Prognostic Factors and Survival of Gliomatosis Cerebri: A Systematic Review and Meta-Analysis.
Georgakis MK, Tsivgoulis G, Spinos D, Liaskas A, Herrlinger U, Petridou ET
World Neurosurg 2018 Dec;120:e818-e854. Epub 2018 Aug 30 doi: 10.1016/j.wneu.2018.08.173. PMID: 30172970
Clinical, neuroimaging and histopathological features of gliomatosis cerebri: a systematic review based on synthesis of published individual patient data.
Georgakis MK, Tsivgoulis G, Spinos D, Dimitriou NG, Kyritsis AP, Herrlinger U, Petridou ET
J Neurooncol 2018 Nov;140(2):467-475. Epub 2018 Aug 16 doi: 10.1007/s11060-018-2976-4. PMID: 30117023
Marked response of gliomatosis cerebri to temozolomide and whole brain radiotherapy.
Mattox AK, Lark AL, Adamson DC
Clin Neurol Neurosurg 2012 May;114(4):299-306. Epub 2012 Feb 17 doi: 10.1016/j.clineuro.2012.01.030. PMID: 22341931

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