Journal of Neuro-Oncology

, Volume 76, Issue 2, pp 201–205 | Cite as

Gliomatosis Cerebri: A Review of 296 Cases from the ANOCEF Database and the Literature

  • Sophie Taillibert
  • Catherine Chodkiewicz
  • Florence Laigle-Donadey
  • Massimo Napolitano
  • Stéphanie Cartalat-Carel
  • Marc Sanson
Clinical Study


Gliomatosis cerebri (GC) is a rare disease, defined as a diffuse neoplastic glial cell infiltration of the brain. Diagnosis and management of GC are difficult. This study analyzed 296 individual cases (90 patients followed through the ANOCEF network, and 206 cases from the literature), aged 1 month to 85 years (median 42), sex ratio = 1.31. Median survival was 14.5 months. It was higher for patients younger than 42 years (17 months vs. 13 months), with performance status ≥80 (27 months vs. 9 months), low grade gliomatosis (grade 2 = 20 months, grade 3 = 11.5 months, grade 4 = 8.5 months), oligodendroglial subtype (36 months compared to 14 months for mixed GC and 11 months for astrocytic GC). Male population was younger (median 39 years vs. 45), had a higher incidence of oligodendroglial GC (22% vs. 13%), which may explain their better prognosis (median survival 17 months vs. 11.5 months) than female population. Despite a high rate of stabilization, the impact on survival of whole brain radiotherapy, which carries the risk of severe toxicity, is still unclear. Up-front chemotherapy benefit to some patients and may be prefered to whole brain radiotherapy. However, the many bias of such retrospective heterogeneous data claim for multicentric clinical trials in this rare disease.


gliomatosis cerebri chemotherapy review 


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. 1.
    Lantos PL, Bruner JM: Gliomatosis cerebri in tumours of the nervous system. In: Kleihues P, Cavence WK (eds), Lyon, IARC, 2000, pp. 92–93Google Scholar
  2. 2.
    Nevin, S 1938Gliomatosis cerebriBrain61170191Google Scholar
  3. 3.
    Kros, JM, Zheng, P, Dinjens, WN, Alers, JC 2002Genetic aberrations in gliomatosis cerebri support monoclonal tumorigenesisJ Neuropathol Exp Neurol61806814PubMedGoogle Scholar
  4. 4.
    Hecht, BK, Turc-Carel, C, Chatel, M,  et al. 1995Chromosomes in gliomatosis cerebriGene Chromosome Cancer14149153Google Scholar
  5. 5.
    Kirches, E, Mawrin, C, Schneider-Stock, R, Krause, G, Scherlach, C, Dietzmann, K 2003Mitochondrial DNA as a clonal tumor cell marker: gliomatosis cerebriJ Neurooncol6115CrossRefPubMedGoogle Scholar
  6. 6.
    Sanson, M, Cartalat-Carel, S, Taillibert, S,  et al. 2004Initial chemotherapy in gliomatosis cerebriNeurology63270275PubMedGoogle Scholar
  7. 7.
    Artigas, J, Cervos-Navarro, J, Iglesias, JR, Ebhardt, G 1985Gliomatosis cerebri: clinical and histological findingsClin Neuropathol4135148PubMedGoogle Scholar
  8. 8.
    Balko, MG, Blisard, KS, Samaha, FJ 1992Oligodendroglial gliomatosis cerebriHum Pathol23706707PubMedGoogle Scholar
  9. 9.
    Benjelloun, A, Delavelle, J, Lazeyras, F, Dietrich, PY 2001Possible efficacy of temozolomide in a patient with gliomatosis cerebriNeurology5719321933PubMedGoogle Scholar
  10. 10.
    Cervos-Navarro, J, Artigas, J, Aruffo, C, Iglesias, J 1987The fine structure of gliomatosis cerebriVirchows Arch A Pathol Anat Histopathol4119398CrossRefPubMedGoogle Scholar
  11. 11.
    Couch, JR, Weiss, SA 1974Gliomatosis cerebri. Report of four cases and review of the literatureNeurology24504511PubMedGoogle Scholar
  12. 12.
    Cozad, SC, Townsend, P, Morantz, RA, Jenny, AB, Kepes, JJ, Smalley, SR 1996Gliomatosis cerebri. Results with radiation therapyCancer717891793Google Scholar
  13. 13.
    Carpio-O’Donovan, R, Korah, I, Salazar, A, Melancon, D 1996Gliomatosis cerebriRadiology198831835PubMedGoogle Scholar
  14. 14.
    Elshaikh, MA, Stevens, GH, Peereboom, DM,  et al. 2002Gliomatosis cerebri: treatment results with radiotherapy aloneCancer9520272031CrossRefPubMedGoogle Scholar
  15. 15.
    Fallentin, E, Skriver, E, Herning, M, Broholm, H 1997Gliomatosis cerebri – An appropriate diagnosis? Case reportsActa Radiol38381390PubMedCrossRefGoogle Scholar
  16. 16.
    Felsberg, GJ, Glass, JP, Tien, RD, McLendon, R 1996Gliomatosis cerebri presenting with optic nerve involvement: MRINeuroradiology38774777CrossRefPubMedGoogle Scholar
  17. 17.
    Giovagnoli, AR, Strada, L, Pollo, B, Boiardi, A 1992Gliomatosis cerebri. Report of a case with isolated amnesic disordersItal J Neurol Sci13503506CrossRefPubMedGoogle Scholar
  18. 18.
    Gottesman, M, Laufer, H, Patel, M. 1991Gliomatosis cerebri: a case reportClin Neuropathol10303305PubMedGoogle Scholar
  19. 19.
    Hara, A, Sakai, N, Yamada, H, Tanaka, T, Mori, H. 1991Assessment of proliferative potential in gliomatosis cerebriJ Neurol2388082CrossRefPubMedGoogle Scholar
  20. 20.
    Herrlinger, U, Felsberg, J, Kuker, W,  et al. 2002Gliomatosis cerebri: molecular pathology and clinical courseAnn Neurol52390399CrossRefPubMedGoogle Scholar
  21. 21.
    Jennings, MT, Frenchman, M, Shehab, T,  et al. 1995Gliomatosis cerebri presenting as intractable epilepsy during early childhoodJ Child Neurol103745PubMedGoogle Scholar
  22. 22.
    Kawano, N, Miyasaka, Y, Yada, K, Atari, H, Sasaki, K 1978Diffuse cerebrospinal gliomatosis. Case reportJ Neurosurg49303307PubMedGoogle Scholar
  23. 23.
    Kandler, RH, Smith, CM, Broome, JC, Davies-Jones, GA 1991Gliomatosis cerebri: a clinical, radiological and pathological report of four casesBr J Neurosurg5187193PubMedGoogle Scholar
  24. 24.
    Kim, DG, Yang, HJ, Park, IA,  et al. 1998Gliomatosis cerebri: clinical features, treatment, and prognosisActa Neurochir (Wien)140755762CrossRefGoogle Scholar
  25. 25.
    Koslow, SA, Claassen, D, Hirsch, WL, Jungreis, CA. 1992Gliomatosis cerebri: a case report with autopsy correlationNeuroradiology34331333CrossRefPubMedGoogle Scholar
  26. 26.
    Lodi, R, Setola, E, Tonon, C,  et al. 2003Gliomatosis cerebri: clinical, neurochemical and neuroradiological response to temozolomide administrationMagn Reson Imaging2110031007PubMedGoogle Scholar
  27. 27.
    Malamud, N, Wise, BL, Jones, OW,Jr. 1952Gliomatosis cerebriJ Neurosurg9409417PubMedGoogle Scholar
  28. 28.
    Meligonis, G, Sur, M, Ouma, J,  et al. 2002Gliomatosis of the brain and spinal cord masquerading as infective lesionsSurg Neurol57399404discussion 404PubMedGoogle Scholar
  29. 29.
    Miller, RR, Lin, F, Mallonee, MM 1981Cytologic diagnosis of gliomatosis cerebriActa Cytol253739PubMedGoogle Scholar
  30. 30.
    Mineura, K, Sasajima, T, Kowada, M,  et al. 1991Innovative approach in the diagnosis of gliomatosis cerebri using carbon-11-L-methionine positron emission tomographyJ Nucl Med32726728PubMedGoogle Scholar
  31. 31.
    Nahser, HC, Gerhard, L, Reinhardt, V,  et al. 1981Diffuse and multicentric brain tumors – Correlation of histological, clinical and CT appearanceActa Neuropathol Suppl (Berl)7101104Google Scholar
  32. 32.
    Nishioka, H, Ito, H, Miki, T 1996Difficulties in the antemortem diagnosis of gliomatosis cerebri: report of a case with diffuse increase of gemistocyte-like cells, mimicking reactive gliosisBr J Neurosurg10103107PubMedCrossRefGoogle Scholar
  33. 33.
    Peretti-Viton, P, Brunel, H, Chinot, O,  et al. 2002Histological and MR correlations in Gliomatosis cerebriJ Neurooncol59249259CrossRefPubMedGoogle Scholar
  34. 34.
    Perkins, GH, Schomer, DF, Fuller, GN,  et al. 2003Gliomatosis cerebri: improved outcome with radiotherapyInt J Radiat Oncol Biol Phys5611371146CrossRefPubMedGoogle Scholar
  35. 35.
    Priest, JR, Watterson, J, Sung, JH, Cox, C 1993Gliomatosis cerebri following radiation and chemotherapyJ Neurosurg78158159PubMedGoogle Scholar
  36. 36.
    Pyhtinen, J, Paakko, E 1996A difficult diagnosis of gliomatosis cerebriNeuroradiology38444448PubMedGoogle Scholar
  37. 37.
    Ross, IB, Robitaille, Y, Villemure, JG, Tampieri, D 1991Diagnosis and management of gliomatosis cerebri: recent trendsSurg Neurol36431440PubMedGoogle Scholar
  38. 38.
    Rust, P, Ashkan, K, Ball, C,  et al. 2001Gliomatosis cerebri: pitfalls in diagnosisJ Clin Neurosci8361363CrossRefPubMedGoogle Scholar
  39. 39.
    Schmidbauer, M, Muller, C, Podreka, I,  et al. 1989Diffuse cerebrospinal gliomatosis presenting as motor neuron disease for two yearsJ Neurol Neurosurg Psychiat52275278PubMedCrossRefGoogle Scholar
  40. 40.
    Schober, R, Mai, JK, Volk, B, Wechsler, W 1991Gliomatosis cerebri: bioptical approach and neuropathological verificationActa Neurochir (Wien)113131137Google Scholar
  41. 41.
    Schoenen, J, Leval, L, Reznik, M 1996Gliomatosis cerebri: clinical, radiological and pathological report of a case with a stroke-like onsetActa Neurol Belg96294300PubMedGoogle Scholar
  42. 42.
    Spagnoli, MV, Grossman, RI, Packer, RJ,  et al. 1987Magnetic resonance imaging determination of gliomatosis cerebriNeuroradiology291518CrossRefPubMedGoogle Scholar
  43. 43.
    Yanaka, K, Kamezaki, T, Kobayashi, E, Matsueda, K, Yoshii, Y, Nose, T 1992MR imaging of diffuse gliomaAm J Neuroradiol13349351PubMedGoogle Scholar
  44. 44.
    Yaguchi, M, Nakasone, A, Sohmiya, M,  et al. 2003Gliomatosis cerebri involving the lumbosacral spinal cordInternal Med42615618CrossRefGoogle Scholar
  45. 45.
    Bertrand, E, Kryst-Widzgowska, T, Baranska, M 1994Gliomatosis cerebri at the 17-year-old girl correlations of histological, CT and MRI appearancePolia Neuropathol32245249Google Scholar
  46. 46.
    Iglesias, A, Garcia, M, San Millan, J, Villanueva, C, Fraile, G, Serrano, M 1997Gliomatosis cerebri mimicking a metastatic breast cancer: fatal outcomeJ Neuro-Oncol32175178CrossRefGoogle Scholar
  47. 47.
    Trapani, G, Sabatelli, M, Carnevale, A, Colosimo, C, Mignogna, T, Tonali, P 1995Gliomatosis cerebri: report of an atypical caseClin Neuropathol141318PubMedGoogle Scholar
  48. 48.
    Horst, E, Micke, O, Romppainen, ML,  et al. 2000Radiation therapy approach in gliomatosis cerebriActa Oncol39747751PubMedCrossRefGoogle Scholar
  49. 49.
    Weston, P, Lear, J 1995Gliomatosis cerebri or benign intracranial hypertension?Postgrad Med J71380381PubMedGoogle Scholar
  50. 50.
    Vassallo, M, Allen, S 1995An unusual cause of dementiaPostgrad Med J71483484PubMedCrossRefGoogle Scholar
  51. 51.
    Kannoki, S, Hondo, H, Li, K, Hirose, T, Matsumoto, K 1997Gliomatosis cerebri with good prognosisBrain Tumor Pathol145357Google Scholar
  52. 52.
    Penteado Lancellotti, CL, Carriel Amary, MF, Barbastefano, AM, Tilbery, CP. 1997Gliomatosis cerebri simulating an acute diffuse encephalomyelitisArq Neuropsiquiatr55488495Google Scholar
  53. 53.
    Mohlo, ES. 2004Gliomatosis cerebri may present as an atypical parkinsonian syndromMovement Disord19341344Google Scholar
  54. 54.
    Filley, CM, Kleinschmidt-DeMasters, BK, Lillehei, KO, Damek, DM, Harris, JG 2003Gliomatosis Cerebri: neurobehavioral and neuropathological observationsCogn Behav Neurol16149159PubMedGoogle Scholar
  55. 55.
    Levin, N, Gomori, JM, Siegal, T 2004Chemotherapy as initial treatment in gliomatosis cerebri: results with temozolomideNeurology63354356PubMedGoogle Scholar
  56. 56.
    Cairncross, JG, Ueki, K, Zlatescu, MC,  et al. 1998Specific genetic predictors of chemotherapeutic response and survival in patients with anaplastic oligodendrogliomasJ Natl Cancer Inst9014731479CrossRefPubMedGoogle Scholar
  57. 57.
    Hoang-Xuan, K, He, J, Huguet, S, Mokhtari, K,  et al. 2001Molecular heterogeneity of oligodendrogliomas suggests alternative pathways in tumor progressionNeurology5712781281PubMedGoogle Scholar

Copyright information

© Springer 2005

Authors and Affiliations

  • Sophie Taillibert
    • 1
  • Catherine Chodkiewicz
    • 2
  • Florence Laigle-Donadey
    • 1
  • Massimo Napolitano
    • 1
  • Stéphanie Cartalat-Carel
    • 3
  • Marc Sanson
    • 1
  1. 1.Fédération de NeurologieGroupe hospitalier Pitié-SalpétrièreParisFrance
  2. 2.Department of Interdisciplinary OncologyH. Lee Moffitt Cancer Center & Research Institute TampaUSA
  3. 3.Neurologie BHôpital NeurologiqueLyonFrance

Personalised recommendations