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

Journal of Neuro-Oncology volume 76, pages 201–205 (2006)Cite this article

Summary

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.

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References

  1. Lantos PL, Bruner JM: Gliomatosis cerebri in tumours of the nervous system. In: Kleihues P, Cavence WK (eds), Lyon, IARC, 2000, pp. 92–93

  2. S Nevin (1938) ArticleTitleGliomatosis cerebri Brain 61 170–191

    Google Scholar 

  3. JM Kros P Zheng WN Dinjens JC Alers (2002) ArticleTitleGenetic aberrations in gliomatosis cerebri support monoclonal tumorigenesis J Neuropathol Exp Neurol 61 IssueID9 806–814 Occurrence Handle1:CAS:528:DC%2BD38Xnslentbc%3D Occurrence Handle12230327

    CAS  PubMed  Google Scholar 

  4. BK Hecht C Turc-Carel M Chatel et al. (1995) ArticleTitleChromosomes in gliomatosis cerebri Gene Chromosome Cancer 14 IssueID2 149–153 Occurrence Handle1:STN:280:DyaK287gvVCjug%3D%3D

    CAS  Google Scholar 

  5. E Kirches C Mawrin R Schneider-Stock G Krause C Scherlach K Dietzmann (2003) ArticleTitleMitochondrial DNA as a clonal tumor cell marker: gliomatosis cerebri J Neurooncol 61 IssueID1 1–5 Occurrence Handle10.1023/A:1021296212233 Occurrence Handle1:STN:280:DC%2BD3s%2Fns1GjsA%3D%3D Occurrence Handle12587789

    Article  CAS  PubMed  Google Scholar 

  6. M Sanson S Cartalat-Carel S Taillibert et al. (2004) ArticleTitleInitial chemotherapy in gliomatosis cerebri Neurology 63 IssueID2 270–275 Occurrence Handle1:STN:280:DC%2BD2cznsVeksA%3D%3D Occurrence Handle15277619

    CAS  PubMed  Google Scholar 

  7. J Artigas J Cervos-Navarro JR Iglesias G Ebhardt (1985) ArticleTitleGliomatosis cerebri: clinical and histological findings Clin Neuropathol 4 IssueID4 135–148 Occurrence Handle1:STN:280:DyaL28%2FivVyitw%3D%3D Occurrence Handle4053456

    CAS  PubMed  Google Scholar 

  8. MG Balko KS Blisard FJ Samaha (1992) ArticleTitleOligodendroglial gliomatosis cerebri Hum Pathol 23 IssueID6 706–707 Occurrence Handle1:STN:280:DyaK383nsFKjsQ%3D%3D Occurrence Handle1592395

    CAS  PubMed  Google Scholar 

  9. A Benjelloun J Delavelle F Lazeyras PY Dietrich (2001) ArticleTitlePossible efficacy of temozolomide in a patient with gliomatosis cerebri Neurology 57 IssueID10 1932–1933 Occurrence Handle1:STN:280:DC%2BD3MnnsleltQ%3D%3D Occurrence Handle11723300

    CAS  PubMed  Google Scholar 

  10. J Cervos-Navarro J Artigas C Aruffo J Iglesias (1987) ArticleTitleThe fine structure of gliomatosis cerebri Virchows Arch A Pathol Anat Histopathol 411 IssueID1 93–98 Occurrence Handle10.1007/BF00734520 Occurrence Handle1:STN:280:DyaL2s3gslKhtg%3D%3D Occurrence Handle3107212

    Article  CAS  PubMed  Google Scholar 

  11. JR Couch SA Weiss (1974) ArticleTitleGliomatosis cerebri. Report of four cases and review of the literature Neurology 24 IssueID6 504–511 Occurrence Handle1:STN:280:DyaL2M%2Fos1Oguw%3D%3D Occurrence Handle4499966

    CAS  PubMed  Google Scholar 

  12. SC Cozad P Townsend RA Morantz AB Jenny JJ Kepes SR Smalley (1996) ArticleTitleGliomatosis cerebri. Results with radiation therapy Cancer 7 IssueID8 1789–1793

    Google Scholar 

  13. R Carpio-O’Donovan Particledel I Korah A Salazar D Melancon (1996) ArticleTitleGliomatosis cerebri Radiology 198 IssueID3 831–835 Occurrence Handle8628879

    PubMed  Google Scholar 

  14. MA Elshaikh GH Stevens DM Peereboom et al. (2002) ArticleTitleGliomatosis cerebri: treatment results with radiotherapy alone Cancer 95 IssueID9 2027–2031 Occurrence Handle10.1002/cncr.10909 Occurrence Handle12404298

    Article  PubMed  Google Scholar 

  15. E Fallentin E Skriver M Herning H Broholm (1997) ArticleTitleGliomatosis cerebri – An appropriate diagnosis? Case reports Acta Radiol 38 IssueID3 381–390 Occurrence Handle1:STN:280:DyaK2szjsVOlsA%3D%3D Occurrence Handle9191428 Occurrence Handle10.3109/02841859709172088

    CAS  PubMed  Article  Google Scholar 

  16. GJ Felsberg JP Glass RD Tien R McLendon (1996) ArticleTitleGliomatosis cerebri presenting with optic nerve involvement: MRI Neuroradiology 38 IssueID8 774–777 Occurrence Handle10.1007/s002340050345 Occurrence Handle1:STN:280:DyaK2s7hs1Oiug%3D%3D Occurrence Handle8957803

    Article  CAS  PubMed  Google Scholar 

  17. AR Giovagnoli L Strada B Pollo A Boiardi (1992) ArticleTitleGliomatosis cerebri. Report of a case with isolated amnesic disorders Ital J Neurol Sci 13 IssueID6 503–506 Occurrence Handle10.1007/BF02230871 Occurrence Handle1:STN:280:DyaK3s%2FltlGitg%3D%3D Occurrence Handle1428787

    Article  CAS  PubMed  Google Scholar 

  18. M Gottesman H Laufer M. Patel (1991) ArticleTitleGliomatosis cerebri: a case report Clin Neuropathol 10 IssueID6 303–305 Occurrence Handle1:STN:280:DyaK387htFSgtg%3D%3D Occurrence Handle1764854

    CAS  PubMed  Google Scholar 

  19. A Hara N Sakai H Yamada T Tanaka H. Mori (1991) ArticleTitleAssessment of proliferative potential in gliomatosis cerebri J Neurol 238 IssueID2 80–82 Occurrence Handle10.1007/BF00315685 Occurrence Handle1:STN:280:DyaK3MzhtFCqtg%3D%3D Occurrence Handle1856741

    Article  CAS  PubMed  Google Scholar 

  20. U Herrlinger J Felsberg W Kuker et al. (2002) ArticleTitleGliomatosis cerebri: molecular pathology and clinical course Ann Neurol 52 IssueID4 390–399 Occurrence Handle10.1002/ana.10297 Occurrence Handle1:CAS:528:DC%2BD38XotlOjsbo%3D Occurrence Handle12325066

    Article  CAS  PubMed  Google Scholar 

  21. MT Jennings M Frenchman T Shehab et al. (1995) ArticleTitleGliomatosis cerebri presenting as intractable epilepsy during early childhood J Child Neurol 10 IssueID1 37–45 Occurrence Handle1:STN:280:DyaK2M3os12itQ%3D%3D Occurrence Handle7539465

    CAS  PubMed  Google Scholar 

  22. N Kawano Y Miyasaka K Yada H Atari K Sasaki (1978) ArticleTitleDiffuse cerebrospinal gliomatosis. Case report J Neurosurg 49 IssueID2 303–307 Occurrence Handle1:STN:280:DyaE1c3hvF2nsA%3D%3D Occurrence Handle671086

    CAS  PubMed  Google Scholar 

  23. RH Kandler CM Smith JC Broome GA Davies-Jones (1991) ArticleTitleGliomatosis cerebri: a clinical, radiological and pathological report of four cases Br J Neurosurg 5 IssueID2 187–193 Occurrence Handle1:STN:280:DyaK3MzitFOksg%3D%3D Occurrence Handle1650563

    CAS  PubMed  Google Scholar 

  24. DG Kim HJ Yang IA Park et al. (1998) ArticleTitleGliomatosis cerebri: clinical features, treatment, and prognosis Acta Neurochir (Wien) 140 IssueID8 755–762 Occurrence Handle10.1007/s007010050176 Occurrence Handle1:STN:280:DyaK1M%2FivVWgtA%3D%3D

    Article  CAS  Google Scholar 

  25. SA Koslow D Claassen WL Hirsch CA. Jungreis (1992) ArticleTitleGliomatosis cerebri: a case report with autopsy correlation Neuroradiology 34 IssueID4 331–333 Occurrence Handle10.1007/BF00588195 Occurrence Handle1:STN:280:DyaK38vgvFShtA%3D%3D Occurrence Handle1528447

    Article  CAS  PubMed  Google Scholar 

  26. R Lodi E Setola C Tonon et al. (2003) ArticleTitleGliomatosis cerebri: clinical, neurochemical and neuroradiological response to temozolomide administration Magn Reson Imaging 21 IssueID9 1003–1007 Occurrence Handle1:CAS:528:DC%2BD3sXpvVaitrk%3D Occurrence Handle14684203

    CAS  PubMed  Google Scholar 

  27. N Malamud BL Wise OW Jones SuffixJr. (1952) ArticleTitleGliomatosis cerebri J Neurosurg 9 IssueID4 409–417 Occurrence Handle1:STN:280:DyaG38%2Fns1KqsQ%3D%3D Occurrence Handle14946583

    CAS  PubMed  Google Scholar 

  28. G Meligonis M Sur J Ouma et al. (2002) ArticleTitleGliomatosis of the brain and spinal cord masquerading as infective lesions Surg Neurol 57 IssueID6 399–404 Occurrence Handle12176200

    PubMed  Google Scholar 

  29. RR Miller F Lin MM Mallonee (1981) ArticleTitleCytologic diagnosis of gliomatosis cerebri Acta Cytol 25 IssueID1 37–39 Occurrence Handle1:STN:280:DyaL3M7ivFKitA%3D%3D Occurrence Handle6937066

    CAS  PubMed  Google Scholar 

  30. K Mineura T Sasajima M Kowada et al. (1991) ArticleTitleInnovative approach in the diagnosis of gliomatosis cerebri using carbon-11-L-methionine positron emission tomography J Nucl Med 32 IssueID4 726–728 Occurrence Handle1:STN:280:DyaK3M7ptlyitg%3D%3D Occurrence Handle2013813

    CAS  PubMed  Google Scholar 

  31. HC Nahser L Gerhard V Reinhardt et al. (1981) ArticleTitleDiffuse and multicentric brain tumors – Correlation of histological, clinical and CT appearance Acta Neuropathol Suppl (Berl) 7 101–104 Occurrence Handle1:STN:280:DyaL3M7otValsg%3D%3D

    CAS  Google Scholar 

  32. H Nishioka H Ito T Miki (1996) ArticleTitleDifficulties in the antemortem diagnosis of gliomatosis cerebri: report of a case with diffuse increase of gemistocyte-like cells, mimicking reactive gliosis Br J Neurosurg 10 IssueID1 103–107 Occurrence Handle1:STN:280:DyaK283lvFOrsA%3D%3D Occurrence Handle8672252 Occurrence Handle10.1080/bjn.10.1.103

    CAS  PubMed  Article  Google Scholar 

  33. P Peretti-Viton H Brunel O Chinot et al. (2002) ArticleTitleHistological and MR correlations in Gliomatosis cerebri J Neurooncol 59 IssueID3 249–259 Occurrence Handle10.1023/A:1019934901750 Occurrence Handle12241123

    Article  PubMed  Google Scholar 

  34. GH Perkins DF Schomer GN Fuller et al. (2003) ArticleTitleGliomatosis cerebri: improved outcome with radiotherapy Int J Radiat Oncol Biol Phys 56 IssueID4 1137–1146 Occurrence Handle10.1016/S0360-3016(03)00293-1 Occurrence Handle12829152

    Article  PubMed  Google Scholar 

  35. JR Priest J Watterson JH Sung C Cox (1993) ArticleTitleGliomatosis cerebri following radiation and chemotherapy J Neurosurg 78 IssueID1 158–159 Occurrence Handle1:STN:280:DyaK3s%2FpsVyrtw%3D%3D Occurrence Handle8416236

    CAS  PubMed  Google Scholar 

  36. J Pyhtinen E Paakko (1996) ArticleTitleA difficult diagnosis of gliomatosis cerebri Neuroradiology 38 IssueID5 444–448 Occurrence Handle1:STN:280:DyaK28vjsVWjuw%3D%3D Occurrence Handle8837088

    CAS  PubMed  Google Scholar 

  37. IB Ross Y Robitaille JG Villemure D Tampieri (1991) ArticleTitleDiagnosis and management of gliomatosis cerebri: recent trends Surg Neurol 36 IssueID6 431–440 Occurrence Handle1:STN:280:DyaK387gtFWgsQ%3D%3D Occurrence Handle1759182

    CAS  PubMed  Google Scholar 

  38. P Rust K Ashkan C Ball et al. (2001) ArticleTitleGliomatosis cerebri: pitfalls in diagnosis J Clin Neurosci 8 IssueID4 361–363 Occurrence Handle10.1054/jocn.2000.0864 Occurrence Handle1:STN:280:DC%2BD3MzosVOjuw%3D%3D Occurrence Handle11437581

    Article  CAS  PubMed  Google Scholar 

  39. M Schmidbauer C Muller I Podreka et al. (1989) ArticleTitleDiffuse cerebrospinal gliomatosis presenting as motor neuron disease for two years J Neurol Neurosurg Psychiat 52 IssueID2 275–278 Occurrence Handle1:STN:280:DyaL1M7pvFSitA%3D%3D Occurrence Handle2703845 Occurrence Handle10.1136/jnnp.52.2.275

    CAS  PubMed  Article  Google Scholar 

  40. R Schober JK Mai B Volk W Wechsler (1991) ArticleTitleGliomatosis cerebri: bioptical approach and neuropathological verification Acta Neurochir (Wien) 113 IssueID3–4 131–137 Occurrence Handle1:STN:280:DyaK387ovVOqsw%3D%3D

    CAS  Google Scholar 

  41. J Schoenen L Leval ParticleDe M Reznik (1996) ArticleTitleGliomatosis cerebri: clinical, radiological and pathological report of a case with a stroke-like onset Acta Neurol Belg 96 IssueID4 294–300 Occurrence Handle1:STN:280:DyaK2s7mtFGnsQ%3D%3D Occurrence Handle9008778

    CAS  PubMed  Google Scholar 

  42. MV Spagnoli RI Grossman RJ Packer et al. (1987) ArticleTitleMagnetic resonance imaging determination of gliomatosis cerebri Neuroradiology 29 IssueID1 15–18 Occurrence Handle10.1007/BF00341031 Occurrence Handle1:STN:280:DyaL2s7ksV2rsA%3D%3D Occurrence Handle3822096

    Article  CAS  PubMed  Google Scholar 

  43. K Yanaka T Kamezaki E Kobayashi K Matsueda Y Yoshii T Nose (1992) ArticleTitleMR imaging of diffuse glioma Am J Neuroradiol 13 IssueID1 349–351 Occurrence Handle1:STN:280:DyaK383otVGgsg%3D%3D Occurrence Handle1595474

    CAS  PubMed  Google Scholar 

  44. M Yaguchi A Nakasone M Sohmiya et al. (2003) ArticleTitleGliomatosis cerebri involving the lumbosacral spinal cord Internal Med 42 IssueID7 615–618 Occurrence Handle10.2169/internalmedicine.42.615

    Article  Google Scholar 

  45. E Bertrand T Kryst-Widzgowska M Baranska (1994) ArticleTitleGliomatosis cerebri at the 17-year-old girl correlations of histological, CT and MRI appearance Polia Neuropathol 32 IssueID4 245–249 Occurrence Handle1:STN:280:DyaK2M7ps1Sisg%3D%3D

    CAS  Google Scholar 

  46. A Iglesias M Garcia J San Millan C Villanueva G Fraile M Serrano (1997) ArticleTitleGliomatosis cerebri mimicking a metastatic breast cancer: fatal outcome J Neuro-Oncol 32 175–178 Occurrence Handle10.1023/A:1005779919226 Occurrence Handle1:STN:280:DyaK2s3gt1emsw%3D%3D

    Article  CAS  Google Scholar 

  47. G Trapani Particledi M Sabatelli A Carnevale C Colosimo T Mignogna P Tonali (1995) ArticleTitleGliomatosis cerebri: report of an atypical case Clin Neuropathol 14 IssueID1 13–18 Occurrence Handle7729074

    PubMed  Google Scholar 

  48. E Horst O Micke ML Romppainen et al. (2000) ArticleTitleRadiation therapy approach in gliomatosis cerebri Acta Oncol 39 IssueID6 747–751 Occurrence Handle1:STN:280:DC%2BD3M%2FnvVGmsw%3D%3D Occurrence Handle11130015 Occurrence Handle10.1080/028418600750063839

    CAS  PubMed  Article  Google Scholar 

  49. P Weston J Lear (1995) ArticleTitleGliomatosis cerebri or benign intracranial hypertension? Postgrad Med J 71 IssueID836 380–381 Occurrence Handle1:STN:280:DyaK2Mznt1aksw%3D%3D Occurrence Handle7644408

    CAS  PubMed  Google Scholar 

  50. M Vassallo S Allen (1995) ArticleTitleAn unusual cause of dementia Postgrad Med J 71 IssueID838 483–484 Occurrence Handle1:STN:280:DyaK28%2FhsVylug%3D%3D Occurrence Handle7567756 Occurrence Handle10.1136/pgmj.71.838.483

    CAS  PubMed  Article  Google Scholar 

  51. S Kannoki H Hondo K Li T Hirose K Matsumoto (1997) ArticleTitleGliomatosis cerebri with good prognosis Brain Tumor Pathol 14 53–57

    Google Scholar 

  52. CL Penteado Lancellotti MF Carriel Amary AM Barbastefano CP. Tilbery (1997) ArticleTitleGliomatosis cerebri simulating an acute diffuse encephalomyelitis Arq Neuropsiquiatr 55 488–495

    Google Scholar 

  53. ES. Mohlo (2004) ArticleTitleGliomatosis cerebri may present as an atypical parkinsonian syndrom Movement Disord 19 IssueID3 341–344

    Google Scholar 

  54. CM Filley BK Kleinschmidt-DeMasters KO Lillehei DM Damek JG Harris (2003) ArticleTitleGliomatosis Cerebri: neurobehavioral and neuropathological observations Cogn Behav Neurol 16 IssueID3 149–159 Occurrence Handle14501536

    PubMed  Google Scholar 

  55. N Levin JM Gomori T Siegal (2004) ArticleTitleChemotherapy as initial treatment in gliomatosis cerebri: results with temozolomide Neurology 63 IssueID2 354–356 Occurrence Handle15277636

    PubMed  Google Scholar 

  56. JG Cairncross K Ueki MC Zlatescu et al. (1998) ArticleTitleSpecific genetic predictors of chemotherapeutic response and survival in patients with anaplastic oligodendrogliomas J Natl Cancer Inst 90 IssueID19 1473–1479 Occurrence Handle10.1093/jnci/90.19.1473 Occurrence Handle1:STN:280:DyaK1cvkslejug%3D%3D Occurrence Handle9776413

    Article  CAS  PubMed  Google Scholar 

  57. K Hoang-Xuan J He S Huguet K Mokhtari et al. (2001) ArticleTitleMolecular heterogeneity of oligodendrogliomas suggests alternative pathways in tumor progression Neurology 57 IssueID7 1278–1281 Occurrence Handle1:STN:280:DC%2BD3Mrlt12isQ%3D%3D Occurrence Handle11591848

    CAS  PubMed  Google Scholar 

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  1. Fédération de Neurologie, Groupe hospitalier Pitié-Salpétrière, 47-83 bd de l’hôpital, 75651, Paris, cedex 13, France

    Sophie Taillibert, Florence Laigle-Donadey, Massimo Napolitano & Marc Sanson

  2. Department of Interdisciplinary Oncology, H. Lee Moffitt Cancer Center & Research Institute, Tampa, Florida, USA

    Catherine Chodkiewicz

  3. Neurologie B, Hôpital Neurologique, Lyon, France

    Stéphanie Cartalat-Carel

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  1. Sophie Taillibert
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  2. Catherine Chodkiewicz
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  3. Florence Laigle-Donadey
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  4. Massimo Napolitano
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  5. Stéphanie Cartalat-Carel
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Correspondence to Marc Sanson.

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Taillibert, S., Chodkiewicz, C., Laigle-Donadey, F. et al. Gliomatosis Cerebri: A Review of 296 Cases from the ANOCEF Database and the Literature. J Neurooncol 76, 201–205 (2006). https://doi.org/10.1007/s11060-005-5263-0

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Keywords

  • gliomatosis cerebri
  • chemotherapy
  • review