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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.
 
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

Moon S
Curr Med Imaging 2022;18(3):285-291. doi: 10.2174/1573405617666210712130555. PMID: 34931987
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
Chamberlain MC
Neurology 2004 Jul 27;63(2):204-5. doi: 10.1212/01.wnl.0000133905.38657.d6. PMID: 15277608

Recent clinical studies

Etiology

Muench A, Teichmann D, Spille D, Kuzman P, Perez E, May SA, Mueller WC, Kombos T, Nazari-Dehkordi S, Onken J, Vajkoczy P, Ntoulias G, Bettencourt C, von Deimling A, Paulus W, Heppner FL, Koch A, Capper D, Kaul D, Thomas C, Schweizer L
Am J Surg Pathol 2023 Dec 1;47(12):1364-1375. Epub 2023 Sep 22 doi: 10.1097/PAS.0000000000002118. PMID: 37737691
Wesseling P, Capper D
Neuropathol Appl Neurobiol 2018 Feb;44(2):139-150. doi: 10.1111/nan.12432. PMID: 28815663
Phillips PH
Int Ophthalmol Clin 2012 Summer;52(3):51-9, xii. doi: 10.1097/IIO.0b013e31825a12f6. PMID: 22668539
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
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

Wesseling P, Capper D
Neuropathol Appl Neurobiol 2018 Feb;44(2):139-150. doi: 10.1111/nan.12432. PMID: 28815663
Phillips PH
Int Ophthalmol Clin 2012 Summer;52(3):51-9, xii. doi: 10.1097/IIO.0b013e31825a12f6. PMID: 22668539
Herrlinger U
Handb Clin Neurol 2012;105:507-15. doi: 10.1016/B978-0-444-53502-3.00005-7. PMID: 22230515
Nandhagopal R, Al-Asmi A, Arunodaya GR, Jacob PC, Al-Azri F, Burney IA
QJM 2011 Jul;104(7):619-20. Epub 2010 Jun 8 doi: 10.1093/qjmed/hcq089. PMID: 20530537
Kaplan SS, Park TS, McKinstry RC, Fuller C, Ojemann JG
Pediatr Neurosurg 2002 Feb;36(2):111-2. doi: 10.1159/000048363. PMID: 11893895

Therapy

Liu L, Li X, Chen L, Zhang X
Curr Med Chem 2018;25(25):2987-3000. doi: 10.2174/0929867324666170406111036. PMID: 28393688
Montes A, Fernández A, Camacho V, de Quintana C, Gallego O, Craven-Bartle J, López D, Molet J, Gómez-Ansón B, Carrió I
Rev Esp Med Nucl Imagen Mol 2017 Jul-Aug;36(4):227-232. Epub 2017 Feb 17 doi: 10.1016/j.remn.2016.11.005. PMID: 28219644
Zhao J, Bao X, Fu N, Ye J, Li T, Yuan Y, Zhang C, Zhang Y, Zhang Y, Qin J, Wu X
J Child Neurol 2014 Aug;29(8):NP28-34. Epub 2013 Jul 19 doi: 10.1177/0883073813495444. PMID: 23872916
Gleizniene R, Bucinskas U, Lukosevicius S, Vaitkus A, Letautiene S, Apanaviciūte D, Galvonaite M
Medicina (Kaunas) 2010;46(5):341-4. PMID: 20679750
Romeike B, Mawrin C
Expert Rev Neurother 2008 Apr;8(4):587-97. doi: 10.1586/14737175.8.4.587. PMID: 18416661

Prognosis

Trkova K, Sumerauer D, Bubenikova A, Krskova L, Vicha A, Koblizek M, Zamecnik J, Jurasek B, Kyncl M, Malinova B, Ondrova B, Jones DTW, Sill M, Strnadova M, Stolova L, Misove A, Benes V 3rd, Zapotocky M
Sci Rep 2024 Feb 7;14(1):3118. doi: 10.1038/s41598-024-53434-0. PMID: 38326438Free PMC Article
Muench A, Teichmann D, Spille D, Kuzman P, Perez E, May SA, Mueller WC, Kombos T, Nazari-Dehkordi S, Onken J, Vajkoczy P, Ntoulias G, Bettencourt C, von Deimling A, Paulus W, Heppner FL, Koch A, Capper D, Kaul D, Thomas C, Schweizer L
Am J Surg Pathol 2023 Dec 1;47(12):1364-1375. Epub 2023 Sep 22 doi: 10.1097/PAS.0000000000002118. PMID: 37737691
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
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
Romeike B, Mawrin C
Expert Rev Neurother 2008 Apr;8(4):587-97. doi: 10.1586/14737175.8.4.587. PMID: 18416661

Clinical prediction guides

Nussbaumer G, Benesch M, Grabovska Y, Mackay A, Castel D, Grill J, Alonso MM, Antonelli M, Bailey S, Baugh JN, Biassoni V, Blattner-Johnson M, Broniscer A, Carai A, Colafati GS, Colditz N, Corbacioglu S, Crampsie S, Entz-Werle N, Eyrich M, Friker LL, Frühwald MC, Garrè ML, Gerber NU, Giangaspero F, Gil-da-Costa MJ, Graf N, Hargrave D, Hauser P, Herrlinger U, Hoffmann M, Hulleman E, Izquierdo E, Jacobs S, Karremann M, Kattamis A, Kebudi R, Kortmann RD, Kwiecien R, Massimino M, Mastronuzzi A, Miele E, Morana G, Noack CM, Pentikainen V, Perwein T, Pfister SM, Pietsch T, Roka K, Rossi S, Rutkowski S, Schiavello E, Seidel C, Štěrba J, Sturm D, Sumerauer D, Tacke A, Temelso S, Valentini C, van Vuurden D, Varlet P, Veldhuijzen van Zanten SEM, Vinci M, von Bueren AO, Warmuth-Metz M, Wesseling P, Wiese M, Wolff JEA, Zamecnik J, Morales La Madrid A, Bison B, Gielen GH, Jones DTW, Jones C, Kramm CM
Neuro Oncol 2024 Sep 5;26(9):1723-1737. doi: 10.1093/neuonc/noae080. PMID: 38717379Free PMC Article
Moon S
Curr Med Imaging 2022;18(3):285-291. doi: 10.2174/1573405617666210712130555. PMID: 34931987
Traynis I, Singer S, Winterkorn J, Rosenblum M, Dinkin M
J Neuroophthalmol 2014 Mar;34(1):44-6. doi: 10.1097/WNO.0b013e31827912d7. PMID: 23287703
Romeike B, Mawrin C
Expert Rev Neurother 2008 Apr;8(4):587-97. doi: 10.1586/14737175.8.4.587. PMID: 18416661
Mawrin C, Kirches E, Schneider-Stock R, Scherlach C, Vorwerk C, Von Deimling A, Van Landeghem F, Meyermann R, Bornemann A, Müller A, Romeike B, Stoltenburg-Didinger G, Wickboldt J, Pilz P, Dietzmann K
Acta Neuropathol 2003 Jun;105(6):529-36. Epub 2003 Feb 26 doi: 10.1007/s00401-003-0674-5. PMID: 12734658

Recent systematic reviews

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
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
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
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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