Skip to main content

Advertisement

SpringerLink
Log in

Characteristics of cerebellar glioblastomas in adults

  • Clinical Study
  • Published:
Journal of Neuro-Oncology Aims and scope Submit manuscript

Abstract

Adult cerebellar glioblastomas (cGBM) are rare and their characteristics remain to be fully described. We analyzed the characteristics of 17 adult patients with cGBM and compared them to a series of 103 patients presenting a supra-tentorial glioblastoma (stGBM). The mean age at GBMc diagnosis was 53.4 years (range 28–77). A history of neurofibromatosis type I was noted in 3 patients. cGBM were hemispheric in 10 patients (58.8%), only vermian in 4 patients (23.5%), and both vermian and hemispheric in 3 patients (17.7%). A H3 K27M mutation was identified in 3/14 patients, a TERT promoter mutation in 3/14 patients and a methylated MGMT promoter in 3/14 patients. None of the patients (0/14) harbored an EGFR amplification, an IDH or a BRAF mutation. Association with neurofibromatosis type I and H3K27M mutations were mutually exclusive. Compared with stGBM, cGBM occurred in younger patients (53.4 vs. 63.2, p = 0.02), were more frequently associated with neurofibromatosis type I (18 vs. 1%, p = 0.009) and with a H3 K27M mutation (21 vs. 3%, p = 0.02). They also tended to have a more frequent multifocal presentation at diagnosis (21 vs. 4.3%, p = 0.06), more frequently resulted in leptomeningeal or intra-axial metastasis (44.5 vs. 5%, p = 0.002) and were associated with a shorter median overall survival (5.9 vs. 14.2 months, p = 0.004). The present study suggests that adult cGBM differ from their supra-tentorial counterpart and constitute a heterogeneous group of IDH wild-type gliomas with at least two subgroups, one associated with H3K27M mutations and the other with neurofibromatosis type I.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2

Similar content being viewed by others

References

  1. Chamberlain MC, Silver P, Levin VA (1990) Poorly differentiated gliomas of the cerebellum. A study of 18 patients. Cancer 65:337–340

    Article  CAS  PubMed  Google Scholar 

  2. Bi WL, Chiocca EA (2013) Adult cerebellar glioblastomas: a distinct entity or parcel of the whole? World Neurosurg 80(6):181–183

    Article  Google Scholar 

  3. Mattos JP, Marenco HA, Campos JM et al (2006) Cerebellar glioblastoma multiforme in an dult. Arq Neuropsiquiatr 64(1):132–135

    Article  PubMed  Google Scholar 

  4. Levine SA, McKeever PE, Greenberg HS (1987) Primary cerebellar glioblastoma multiforme. J Neuro-Oncology 5:231–236

    Article  CAS  Google Scholar 

  5. Adams H, Chaichana KL, Avendaño J et al (2013) Adult cerebellar glioblastoma: understanding survival and prognostic factors using a population-based database from 1973 to 2009. World Neurosurg 80(6):237–243

    Article  Google Scholar 

  6. Akimoto J, Fukami S, Tsutsumi M et al (2009) Radiopathological characteristics of cerebellar malignant glioma in adults. Brain Tumor Pathol 26:59

    Article  PubMed  Google Scholar 

  7. Babu R, Sharma R, Karikari IO et al (2013) Outcome and prognostic factors in adult cerebellar glioblastoma. J Clin Neurosci 20(8):1117–1121

    Article  CAS  PubMed  Google Scholar 

  8. Gopalakrishnan CV, Dhakoji A, Nair S et al (2012) A retrospective study of primary cerebellar glioblastoma multiforme in adults. J Clin Neurosci 19(12):1684–1688

    Article  CAS  PubMed  Google Scholar 

  9. Grahovac G, Tomac D, Lambasa S et al (2009) Cerebellar glioblastomas: pathophysiology, clinical presentation and management. Acta Neurochir 151(6):653–657

    Article  PubMed  Google Scholar 

  10. Gupta V, Goyal A, Sinha S et al (2003) Glioblastoma of the cerebellum. A report of 3 cases. J Neurosurg Sci 47(3):157–164

    CAS  PubMed  Google Scholar 

  11. Hur H, Jung S, Jung TY et al (2008) Cerebellar glioblastoma multiforme in an adult? J Korean Neurosurg 43:194–197

    Article  Google Scholar 

  12. Jing X, Shen G, Su M et al (2015) Primary glioblastoma of the cerebellar vermis: a case report. Oncol Lett 10(1):402–404

    Article  PubMed  PubMed Central  Google Scholar 

  13. Lakičević G, Arnautović K, Mužević D et al (2014) Cerebellar glioblastoma multiforme presenting as hypertensive cerebellar hemorrhage: case report. J Neurol Surg Rep 75(1):e117–e121

    Article  PubMed  PubMed Central  Google Scholar 

  14. Linsenmann T, Monoranu CM, Vince GH et al (2014) Long-term tumor control of spinal dissemination of cerebellar glioblastoma multiforme by combined adjuvant bevacizumab antibody therapy: a case report. BMC Res Notes 7:496

    Article  PubMed  PubMed Central  Google Scholar 

  15. Miller EM, Mani RL, Townsend JJ (1976) Cerebellar glioblastoma multiforme in an adult. Surg Neurol 5(6):341–343

    CAS  PubMed  Google Scholar 

  16. Mishra SS, Behera SK, Dhir MK et al (2014) Cerebellar giant cell glioblastoma multiforme in an adult. J Neurosci Rural Pract 5:295–297

    Article  PubMed  PubMed Central  Google Scholar 

  17. Saito T, Hama S, Kajiwara Y et al (2006) Prognosis of cerebellar glioblastomas: correlation between prognosis and immunoreactivity for epidermal growth factor receptor compared with supratentorial glioblastomas. Anticancer Res 26:1351–1358

    CAS  PubMed  Google Scholar 

  18. Sciacero P, Girelli GF, Cante D et al (2014) Cerebellar glioblastoma multiforme in an adult woman. Tumori 100(3):74–78. https://doi.org/10.1700/1578.17236

    Google Scholar 

  19. Stark AAM, Maslehaty H, Hugo HH et al (2010) Glioblastoma of the cerebellum and brainstem. J Clin Neurosci 17:1248–1251

    Article  PubMed  Google Scholar 

  20. Tamaki T, Eguchi T, Sakamoto M et al (2004) Glioblastoma multiforme of the cerebellum in an elderly man. J Chin Med Assoc 67(6):301–304

    PubMed  Google Scholar 

  21. Tsung AJ, Prabhu SS, Lei X et al (2011) Cerebellar glioblastoma: a retrospective review of 21 patients at a single institution. J Neurooncol 105:555–562

    Article  PubMed  Google Scholar 

  22. Weber DC, Miller RC, Villà S et al (2006) Outcome and prognosis factors in cerebellar glioblastoma multiforme in adults: a retrospective study from the rare cancer network. Int J Radiat Oncol Biol Phys 1 66(1):179–186

    Article  PubMed  Google Scholar 

  23. Young I, Joung JH, Cheong KH, Bak et al (2006) Cerebellar glioblastoma presenting as a cerebellar hemorrhage in a child. J Korean Neurosurg Soc 39:374–377. https://doi.org/10.4103/0028-3886.73775

    Google Scholar 

  24. Wen PY, Macdonald DR, Reardon DA et al (2010) Updated response assessment criteria for high-grade gliomas: response assessment in neuro-oncology working group. J Clin Oncol 28:1963–1972

    Article  PubMed  Google Scholar 

  25. Meyronet D, Esteban-Mader M, Bonnet C et al (2017) Characteristics of H3 K27M-mutant gliomas in adults. Neuro-Oncology. 19:1127

    Article  PubMed  Google Scholar 

  26. Walter T, van Brakel B, Vercherat C et al (2015) O6-methylguanine-DNA methyltransferase status in neuroendocrine tumours: prognostic relevance and association with response to alkylating agents. Br J Cancer 112(3):523–531

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  27. Idbaih A, Criniere E, Marie Y et al (2008) Gene amplification is a poor prognostic factor in anaplastic oligodendrogliomas. Neuro-Oncology 10(4):540–547

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  28. Takahashi Y, Makino K, Nakamura H et al (2014) Clinical characteristics and pathogenesis of cerebellar glioblastoma. Mol Med Rep 10(5):2383–2388

    Article  CAS  PubMed  Google Scholar 

  29. Kuroiwa T, Numaguchi Y, Rothman MI et al (1995) Posterior fossa glioblastoma multiforme: MR findings. Am J Neuroradiol 16:583–589

    CAS  PubMed  Google Scholar 

  30. Utsuki S, Oka H, Miyajima Y et al (2012) Adult cerebellar glioblastoma cases have different characteristics from supratentorial glioblastoma. Brain Tumor Pathol 29(2):87–95

    Article  PubMed  Google Scholar 

  31. Kulkarni AV, Becker LE, Jay V et al (1999) Primary cerebellar glioblastomas multiforme in children. Report of four cases. J Neurosurg 90(3):546–550

    Article  CAS  PubMed  Google Scholar 

  32. Zito JL, Siva A, Smith TW et al (1983) Glioblastoma of the cerebellum. Computed tomographic and pathologic considerations. Surg Neurol 19(4):373–378

    Article  CAS  PubMed  Google Scholar 

  33. Pato-Pato A, Cimas-Hernando I, Lorenzo-González JR et al (2004) Cerebellar glioblastoma multiforme: a case report. Rev Neurol 38(9):839–842

    CAS  PubMed  Google Scholar 

  34. Lobão CAF, Barbosa AS, Nogueira J et al (2008) Cerebellar glioblastoma mutiforme in an adult. Arq Neuropsiquiatr 66(4):879–880

    Article  PubMed  Google Scholar 

  35. Singla N, Kapoor A, Savardekar A et al (2016) Malignant cerebellar peduncle lesions - rapid progression and poor outcome. Surg Neurol Int 7:S170–S173

    Article  PubMed  PubMed Central  Google Scholar 

  36. Sturm D, Witt H, Hovestadt V et al (2012) Hotspot mutations in H3F3A and IDH1 define distinct epigenetic and biological subgroups of glioblastoma. Cancer Cell 22(4):425–437

    Article  CAS  PubMed  Google Scholar 

  37. Kawarabuki K, Ohta T, Hashimoto N et al (2005) Cerebellar glioblastoma genetically defined as a secondary one. Clin Neuropathol 24(2):64–68

    CAS  PubMed  Google Scholar 

  38. Yang SY, Wang KC, Cho BK et al (2005) Radiation-induced cerebellar glioblastoma at the site of a treated medulloblastoma: case report. J Neurosurg 102(4 Suppl):417–422

    CAS  PubMed  Google Scholar 

  39. Ceccarelli M, Barthel FP, Malta TM et al (2016) Molecular profiling reveals biologically discrete subsets and pathways of progression in diffuse glioma. Cell 164(3):550–563

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  40. Nakata S, Nobusawa S, Yamazaki T et al (2017) Histone H3 K27M mutations in adult cerebellar high-grade gliomas. Brain Tumor Pathol. https://doi.org/10.1007/s10014-017-0288-6

    Google Scholar 

  41. Gerrit H, Gielen M, Gessi J, Hammes et al (2013) H3F3A K27M mutation in pediatric CNS tumors: a marker for diffuse high-grade astrocytoma. Am J Clin Pathol 139(3):345–349

    Article  Google Scholar 

  42. Aboian MS, Solomon DA, Felton E et al (2017) Imaging characteristics of pediatric diffuse midline gliomas with histone H3 K27M mutation. Am J Neuroradiol 38(4):795–800

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  43. Venneti S, Santi M, Felicella MM et al (2014) A sensitive and specific histopathologic prognostic marker for H3F3A K27M mutant pediatric glioblastomas. Acta Neuropathol 128(5):743–753

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  44. Reyes-Botero G, Giry M, Mokhtari K et al (2014) Molecular analysis of diffuse intrinsic brainstem gliomas in adults. J Neurooncol 116(2):405–411

    Article  CAS  PubMed  Google Scholar 

  45. Nomura M, Mukasa A, Nagae G et al (2017) Distinct molecular profile of diffuse cerebellar gliomas. Acta Neuropathol 134:941–956

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  46. Broekman ML, Risselada R, Engelen-Lee J et al (2009) Glioblastoma multiforme in the posterior cranial fossa in a patient with neurofibromatosis type I. Case Rep Med. https://doi.org/10.1155/2009/757898

    PubMed  PubMed Central  Google Scholar 

  47. Guillamo JS, Créange A, Kalifa C et al (2003) Prognostic factors of CNS tumours in neurofibromatosis 1 (NF1): a retrospective study of 104 patients. Brain 126(Pt 1):152–160

    Article  PubMed  Google Scholar 

  48. Vizcaíno MA, Shah S, Eberhart CG et al (2015) Clinicopathologic implications of NF1 gene alterations in diffuse gliomas. Hum Pathol 46(9):1323–1330

    Article  PubMed  PubMed Central  Google Scholar 

  49. Aihara K, Mukasa A, Gotoh K et al (2014) H3F3A K27M mutations in thalamic gliomas from young adult patients. Neuro-Oncology 16(1):140–146

    Article  CAS  PubMed  Google Scholar 

  50. Ameratunga M, McArthur G, Gan H et al (2016) Prolonged disease control with MEK inhibitor in neurofibromatosis type I-associated glioblastoma. J Clin Pharm Ther 41(3):357–359

    Article  CAS  PubMed  Google Scholar 

  51. Vanan MI, Underhill DA, Eisenstat DD (2017) Targeting epigenetic pathways in the treatment of pediatric Diffuse (high grade) gliomas. Neurotherapeutics 14(2):274–283

    Article  CAS  PubMed  Google Scholar 

Download references

Acknowledgements

Dr ROBINSON Philip.

Author information

Authors and Affiliations

  1. Service de Neurochirurgie D, Groupe Hospitalier Est, Hospices Civils de Lyon, 59 Bvd Pinel, 69677, Bron Cedex, France

    Thiébaud Picart & Jacques Guyotat

  2. Service de Biologie moléculaire, Groupe Hospitalier Est, Hospices Civils de Lyon, 59 Bvd Pinel, 69394, Lyon Cedex, France

    Marc Barritault & Delphine Poncet

  3. Department of Cancer Cell Plasticity, Cancer Research Centre of Lyon, INSERM U1052, CNRS UMR5286, Lyon, France

    Marc Barritault, David Meyronet & François Ducray

  4. Université Claude Bernard Lyon 1, Lyon, France

    Marc Barritault, David Meyronet, Alexandre Vasiljevic, Jérôme Honnorat, Emmanuel Jouanneau, Delphine Poncet & François Ducray

  5. Délégation à la Recherche Clinique et à l’Innovation, Cellule Innovation, Hôpital Neurologique Pierre Wertheimer, Hospices Civils de Lyon, 69677, Bron Cedex, France

    Julien Berthillier

  6. Service de Neuropathologie, Groupe Hospitalier Est, Hospices Civils de Lyon, 59 Bvd Pinel, 69394, Lyon Cedex, France

    David Meyronet & Alexandre Vasiljevic

  7. Centre de Lutte Contre le Cancer Léon Bérard, 28 rue Laennec, 69008, Lyon, France

    Didier Frappaz

  8. Service de Neuro-Oncologie, Groupe Hospitalier Est, Hospices Civils de Lyon, 59 Bvd Pinel, 69394, Lyon Cedex, France

    Jérôme Honnorat & François Ducray

  9. French Refererence Center on Paraneoplastic Neurological Diseases and Autoimmune Encephalitis, INSERM U1217/UMR CNRS, 5310, Lyon, France

    Jérôme Honnorat

  10. Service de Neurochirurgie B, Groupe Hospitalier Est, Hospices Civils de Lyon, 59 Bvd Pinel, 69677, Bron Cedex, France

    Emmanuel Jouanneau

  11. Signaling, Metabolism and Tumor Progression, Cancer Research Centre of Lyon, INSERM U1052, CNRS UMR5286, Lyon, France

    Emmanuel Jouanneau

Authors
  1. Thiébaud Picart
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Marc Barritault
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Julien Berthillier
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. David Meyronet
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Alexandre Vasiljevic
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Didier Frappaz
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Jérôme Honnorat
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Emmanuel Jouanneau
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Delphine Poncet
    View author publications

    You can also search for this author in PubMed Google Scholar

  10. François Ducray
    View author publications

    You can also search for this author in PubMed Google Scholar

  11. Jacques Guyotat
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Jacques Guyotat.

Ethics declarations

Conflict of interest

Thiébaud Picart, David Meyronet, Julien Berthillier, Marc Barritault, Alexandre Vasiljevic, Didier Frappaz, Jérôme Honnorat, Emmanuel Jouanneau, Delphine Poncet, François Ducray and Jacques Guyotat declare that they have no conflict of interest.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Informed consent

Informed consent was obtained from all individual participants included in the study.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Picart, T., Barritault, M., Berthillier, J. et al. Characteristics of cerebellar glioblastomas in adults. J Neurooncol 136, 555–563 (2018). https://doi.org/10.1007/s11060-017-2682-7

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s11060-017-2682-7

Keywords

Search

Navigation