Primary Brain Tumors in Adults

  • Fernando Silva Picon
  • Adrialdo José Santos
  • Hakaru Tadokoro
  • Ramon Andrade De MelloEmail author


Primary Tumors of the Central Nervous System (PTCNS) are a very heterogeneous group of tumors, which include different types of histology. Their symptoms are variable, according with the local of invasion, compression of adjacent structures, histology and presence of increased intracranial pressure.


GBM Temozolamide Radiotherapy 


  1. 1.
    Ferlay J, Soerjomataram I, Ervik M, Dikshit R, Eser S, Mathers C, Rebelo M, Parkin DM, Forman D, Bray F (2013) GLOBOCAN 2012 v1.0, cancer incidence and mortality worldwide: IARC CancerBase No. 11 [Internet]. International Agency for Research on Cancer, Lyon. Available from:, Accessed on 22 Nov 2017Google Scholar
  2. 2.
    Bondy ML, Lustbader ED, Buffler PA, Schull WJ, Hardy RJ, Strong LC (1991) Genetic epidemiology of childhood brain tumors. Genet Epidemiol 8(4):253CrossRefGoogle Scholar
  3. 3.
    Braganza MZ, Kitahara CM, Berrington de González A, Inskip PD, Johnson KJ, Rajaraman P (2012) Ionizing radiation and the risk of brain and central nervous system tumors: a systematic review. Neuro-Oncology 14(11):1316–1324. Epub 2012 Sep 5CrossRefPubMedPubMedCentralGoogle Scholar
  4. 4.
    Myung SK, Ju W, McDonnell DD, Lee YJ, Kazinets G, Cheng CT, Moskowitz JM (2009) Mobile phone use and risk of tumors: a meta-analysis. J Clin Oncol 27(33):5565–5572. Epub 2009 Oct 13CrossRefGoogle Scholar
  5. 5.
    Ostrom QT, Gittleman H, Fulop J, Liu M, Blanda R, Kromer C, Wolinsky Y, Kruchko C, Barnholtz-Sloan JS (2015) BTRUS statistical report: primary brain and central nervous system tumors diagnosed in the United States in 2008–2012. Neuro-Oncology 17(Suppl 4):iv1–iv62. Epub 2015 Oct 27CrossRefPubMedPubMedCentralGoogle Scholar
  6. 6.
    Marosi C, Hassler M, Roessler K, Reni M, Sant M, Mazza E, Vecht C (2008) Meningioma. Crit Rev Oncol Hematol 67(2):153CrossRefGoogle Scholar
  7. 7.
    Louis DN, Perry A, Reifenberger G, von Deimling A, Figarella-Branger D, Cavenee WK, Ohgaki H, Wiestler OD, Kleihues P, Ellison DW (2016) The 2016 World Health Organization Classification of tumors of the central nervous system: a summary. Acta Neuropathol 131(6):803–820. Epub 2016 MayCrossRefGoogle Scholar
  8. 8.
    Johnson MD, Powell SZ, Boyer PJ, Weil RJ, Moots PL (2002) Dural lesions mimicking meningiomas. Hum Pathol 33(12):1211CrossRefGoogle Scholar
  9. 9.
    Nyuyki F et al (2010) Potential impact of (68)Ga-DOTATOC PET/CT on stereotactic radiotherapy planning of meningiomas. Eur J Nucl Med Mol Imaging 37(2):310–318. Epub 2009 Sep 18CrossRefPubMedGoogle Scholar
  10. 10.
    Whittle IR, Smith C, Navoo P, Collie D (2004) Meningiomas. Lancet 363(9420):1535CrossRefPubMedPubMedCentralGoogle Scholar
  11. 11.
    National Comprehensive Cancer Network. Central nervous system cancers (Version 1.2017). Accessed 25 Nov 2017
  12. 12.
    Pollock BE et al (2003) Stereotactic radiosurgery provides equivalent tumor control to Simpson Grade 1 resection for patients with small- to medium-size meningiomas. Int J Radiat Oncol Biol Phys 55(4):1000–1005CrossRefGoogle Scholar
  13. 13.
    Chamberlain MC et al (2007) Recurrent meningioma: salvage therapy with long-acting somatostatin analogue. Neurology 69(10):969–973CrossRefGoogle Scholar
  14. 14.
    Chamberlain MC, Glantz MJ (2008) Interferon-alpha for recurrent World Health Organization grade 1 intracranial meningiomas. Cancer 113(8):2146–2151. Scholar
  15. 15.
    Kumar V, Abbas AK, Aster JC (2015) Robbins and Cotran pathologic basis of disease, 9th edn. Elsevier/Saunders, PhiladelphiaGoogle Scholar
  16. 16.
    Stupp R, Brada M, van den Bent MJ, Tonn JC, Pentheroudakis JG, on behalf of the ESMO Guidelines Working Group (2014) High-grade glioma: ESMO clinical practice guidelines for diagnosis, treatment and follow-up. Ann Oncol 25(Suppl 3):iii93–iii101. Published online 29 April 2014CrossRefPubMedGoogle Scholar
  17. 17.
    Yan H et al (2009) IDH1 and IDH2 mutations in gliomas. N Engl J Med 360:765–773. Scholar
  18. 18.
    Sanson M et al (2009) Isocitrate dehydrogenase 1 codon 132 mutation is an important prognostic biomarker in gliomas. J Clin Oncol 27(25):4150–4154. Epub 2009 Jul 27CrossRefPubMedGoogle Scholar
  19. 19.
    Houillier C (2010) IDH1 or IDH2 mutations predict longer survival and response to temozolomide in low-grade gliomas. Neurology 75(17):1560–1566. Scholar
  20. 20.
    Aldape K et al (2007) Clinicopathologic aspects of 1p/19q loss and the diagnosis of oligodendroglioma. Arch Pathol Lab Med 131(2):242–251PubMedGoogle Scholar
  21. 21.
    Cairncross JG, Ueki K, Zlatescu MC, Lisle DK, Finkelstein DM, Hammond RR, Silver JS, Stark PC, Macdonald DR, Ino Y, Ramsay DA, Louis DN (1998) Specific genetic predictors of chemotherapeutic response and survival in patients with anaplastic oligodendrogliomas. J Natl Cancer Inst 90(19):1473CrossRefGoogle Scholar
  22. 22.
    Esteller M et al (2000) Inactivation of the DNA-repair gene MGMT and the clinical response of gliomas to alkylating agents. N Engl J Med 343:1350–1354. Scholar
  23. 23.
    Ebrahimi A et al (2016) Acta Neuropathol Commun 4: 60. Published online 2016 June 16. PMCID: PMC4910252
  24. 24.
    López G, Oberheim Bush NA, Berger MS, Perry A, Solomon DA (2017) Diffuse non-midline glioma with H3F3A K27M mutation: a prognostic and treatment dilemma. Acta Neuropathol Commun 5:38. Scholar
  25. 25.
    Pignatti F et al (2002) Prognostic factors for survival in adult patients with cerebral low-grade glioma. J Clin Oncol 20(8):2076–2084CrossRefGoogle Scholar
  26. 26.
    Lang FF et al (2006) Diffusely infiltrative low-grade gliomas in adults. J Clin Oncol 24(8):1236–1245. Scholar
  27. 27.
    Jakola AS, Skjulsvik AJ, Myrmel KS et al (2017) Surgical resection versus watchful waiting in low-grade gliomas. Ann Oncol 28(8):1942CrossRefPubMedPubMedCentralGoogle Scholar
  28. 28.
    Aghi MK, Nahed BV, Sloan AE, Ryken TC, Kalkanis SN, Olson JJ (2015) The role of surgery in the management of patients with diffuse low grade glioma: a systematic review and evidence-based clinical practice guideline. J Neuro-Oncol 125(3):503CrossRefGoogle Scholar
  29. 29.
    van den Bent MJ, Afra D, de Witte O et al (2005) EORTC Radiotherapy and Brain Tumor Groups and the UK Medical Research Council. Long-term efficacy of early versus delayed radiotherapy for low-grade astrocytoma and oligodendroglioma in adults: the EORTC 22845 randomized trial. Lancet 366(9490):985CrossRefGoogle Scholar
  30. 30.
    Shaw E, Arusell R, Scheithauer B et al (2002) Retrospective randomized trial of low- versus high-dose radiation therapy in adults with supratentorial low-grade glioma: initial report of a North Central Cancer Treatment Group/Radiation Therapy Oncology Group/Eastern Cooperative Oncology Group study. J Clin Oncol 20(9):2267CrossRefGoogle Scholar
  31. 31.
    Karim AB, Maat B, Hatlevoll R et al (1996) A randomized trial on dose-response in radiation therapy of low-grade cerebral glioma: European Organization for Research and Treatment of Cancer (EORTC) Study 22844. Int J Radiat Oncol Biol Phys 36(3):549CrossRefGoogle Scholar
  32. 32.
    Buckner JC, Shaw EG, Pugh SL et al (2016) Radiation plus Procarbazine, CCNU, and vincristine in low-grade glioma. N Engl J Med 374(14):1344CrossRefPubMedPubMedCentralGoogle Scholar
  33. 33.
    Fisher BJ, Hu C, Macdonald DR et al (2015) Phase 2 study of temozolomide-based chemoradiation therapy for high-risk low-grade gliomas: preliminary results of Radiation Therapy Oncology Group 0424. Int J Radiat Oncol Biol Phys 91(3):497. Epub 2015 Jan 30CrossRefPubMedPubMedCentralGoogle Scholar
  34. 34.
    Laws J et al (2003) Survival following surgery and prognostic factors for recently diagnosed malignant glioma: data from the Glioma Outcomes Project. J Neurosurg 99(3):467–473CrossRefGoogle Scholar
  35. 35.
    Walker MD, Green SB, Byar DP et al (1980) Randomized comparisons of radiotherapy and nitrosoureas for the treatment of malignant glioma after surgery. N Engl J Med 303:1323–1329CrossRefGoogle Scholar
  36. 36.
    Keime-Guibert F, Chinot O, Taillandier L et al (2007) Radiotherapy for glioblastoma in the elderly. N Engl J Med 356:1527–1535CrossRefGoogle Scholar
  37. 37.
    Stupp R, Mason WP, van den Bent MJ et al (2005) Radiotherapy plus concomitant and adjuvant temozolomide for glioblastoma. N Engl J Med 352:987–996CrossRefGoogle Scholar
  38. 38.
    Stupp R, Hegi ME, Mason WP et al (2009) Effects of radiotherapy with concomitant and adjuvant temozolomide versus radiotherapy alone on survival in glioblastoma in a randomised phase III study: 5-year analysis of the EORTC-NCIC trial. Lancet Oncol 10:459–466CrossRefGoogle Scholar
  39. 39.
    van den Bent MJ, Brandes AA, Taphoorn MJ et al (2013) Adjuvant procarbazine, lomustine, and vincristine chemotherapy in newly diagnosed anaplastic oligodendroglioma: long-term follow-up of EORTC Brain Tumor Group study 26951. J Clin Oncol 31:344–350CrossRefGoogle Scholar
  40. 40.
    Cairncross G, Wang M, Shaw E et al (2013) Phase III trial of chemoradiotherapy for anaplastic oligodendroglioma: long-term results of RTOG 9402. J Clin Oncol 31:337–343CrossRefGoogle Scholar
  41. 41.
    Taal W, Brandsma D, de Bruin HG (2008) Incidence of early pseudo-progression in a cohort of malignant glioma patients treated with chemoirradiation with temozolomide. Cancer 113(2):405CrossRefGoogle Scholar
  42. 42.
    Parvez K, Parvez A, Zadeh G (2014) The diagnosis and treatment of pseudoprogression, radiation necrosis and brain tumor recurrence. Int J Mol Sci 15(7):11832–11846CrossRefPubMedPubMedCentralGoogle Scholar
  43. 43.
    Friedman HS, Prados MD, Wen PY et al (2009) Bevacizumab alone and in combination with irinotecan in recurrent glioblastoma. J Clin Oncol 27:4733–4740CrossRefGoogle Scholar
  44. 44.
    Kreisl TN, Kim L, Moore K et al (2009) Phase II trial of single-agent bevacizumab followed by bevacizumab plus irinotecan at tumor progression in recurrent glioblastoma. J Clin Oncol 27:740–745CrossRefGoogle Scholar
  45. 45.
    Metellus P et al (2007) Multicentric French study on adult intracranial ependymomas: prognostic factors analysis and therapeutic considerations from a cohort of 152 patients. Brain 130(Pt 5):1338–1349. Epub 2007 Apr 19CrossRefGoogle Scholar
  46. 46.
    DeVita, Hellman, and Rosenberg’s Cancer: principles & practice of oncology, 10th edn. Wolters Kluwer Health/Lippincott Williams & WilkinsGoogle Scholar
  47. 47.
    Metellus P et al (2008) Supratentorial ependymomas: prognostic factors and outcome analysis in a retrospective series of 46 adult patients. Cancer 113(1):175–185. Scholar
  48. 48.
    Mansur DB et al (2005) Postoperative radiation therapy for grade II and III intracranial ependymoma. Int J Radiat Oncol Biol Phys 61(2):387–391CrossRefGoogle Scholar
  49. 49.
    Vanuytsel L, Brada M (1991) The role of prophylactic spinal irradiation in localized intracranial ependymoma. Int J Radiat Oncol Biol Phys 21(3):825–830CrossRefGoogle Scholar
  50. 50.
    Smoll NR, Drummond KJ (2012) The incidence of medulloblastomas and primitive neurectodermal tumours in adults and children. J Clin Neurosci 19(11):1541–1544. Epub 2012 Sep 13CrossRefGoogle Scholar
  51. 51.
    Eberhart CG, Kepner JL, Goldthwaite PT et al (2002) Histopathologic grading of medulloblastomas: a Pediatric Oncology Group study. Cancer 94(2):552CrossRefGoogle Scholar
  52. 52.
    Poretti A, Meoded A, Huisman TA (2012) Neuroimaging of pediatric posterior fossa tumors including review of the literature. J Magn Reson Imaging 35(1):32–47. Epub 2011 Oct 11CrossRefGoogle Scholar
  53. 53.
    Padovani L, Sunyach MP, Perol D et al (2007) Common strategy for adult and pediatric medulloblastoma: a multicenter series of 253 adults. Int J Radiat Oncol Biol Phys 68(2):433–440CrossRefGoogle Scholar
  54. 54.
    Chan AW, Tarbell NJ, Black PM et al (2000) Adult medulloblastoma: prognostic factors and patterns of relapse. Neurosurgery 47(3):623–631PubMedGoogle Scholar
  55. 55.
    Merchant ET, Larry EK, Matthew JK et al (2008) Multi-institution prospective trial of reduced-dose craniospinal irradiation (23.4 Gy) followed by conformal posterior fossa (36 Gy) and primary site irradiation (55.8 Gy) and dose-intensive chemotherapy for average-risk medulloblastoma. Int J Radiat Oncol Biol Phys 70(3):782–787. Epub 2007 Sep 24CrossRefGoogle Scholar
  56. 56.
    Douglas JC, Barker JL, Ellenbogen RG et al (2004) Concurrent chemotherapy and reduced-dose cranial spinal irradiation followed by conformal posterior fossa tumor bed boost for average-risk medulloblastoma: efficacy and patterns of failure. ASTRO Annual Meeting, New Orleans, LA, October 6–10, 2004Google Scholar
  57. 57.
    Packer RJ, Gajjar A, Vezina G et al (2006) Phase III study of craniospinal radiation therapy followed by adjuvant chemotherapy for newly diagnosed average-risk medulloblastoma. J Clin Oncol 24(25):4202–4208CrossRefGoogle Scholar
  58. 58.
    Gill P, Litzow M, Buckner J (2008) High-dose chemotherapy with autologous stem cell transplantation in adults with recurrent embryonal tumors of the central nervous system. Cancer 112(8):1805–1811. Scholar
  59. 59.
    Dunkel IJ, Gardner SL, Garvin JH Jr et al (2010) High-dose carboplatin, thiotepa, and etoposide with autologous stem cell rescue for patients with previously irradiated recurrent medulloblastoma. Neuro-Oncology 12(3):297–303. Epub 2010 Jan 11CrossRefPubMedPubMedCentralGoogle Scholar
  60. 60.
    Nicholson HS, Kretschmar CS, Krailo M (2007) 58 Phase 2 study of temozolomide in children and adolescents with recurrent central nervous system tumors: a report from the Children’s Oncology Group. Cancer 110(7):1542–1550CrossRefGoogle Scholar
  61. 61.
    Villano JL, Koshy M, Shaikh H, Dolecek TA, McCarthy BJ (2011) Age, gender, and racial differences in incidence and survival in primary CNS lymphoma. Br J Cancer 105(9):1414. Epub 2011 Sep 13CrossRefPubMedPubMedCentralGoogle Scholar
  62. 62.
    Bataille B, Delwail V, Menet E, Vandermarcq P et al (2000) Primary intracerebral malignant lymphoma: report of 248 cases. J Neurosurg 92(2):261–266CrossRefGoogle Scholar
  63. 63.
    Batchelor T, Carson K, O’Neill A (2003) Treatment of primary CNS lymphoma with methotrexate and deferred radiotherapy: a report of NABTT 96-07. J Clin Oncol 21(6):1044–1049CrossRefGoogle Scholar
  64. 64.
    Ferreri AJ, Reni M, Foppoli M et al (2009) High-dose cytarabine plus high-dose methotrexate versus high-dose methotrexate alone in patients with primary CNS lymphoma: a randomised phase 2 trial. Lancet 374(9700):1512–1520CrossRefGoogle Scholar
  65. 65.
    Gregory G, Arumugaswamy A, Leung T (2013) Rituximab is associated with improved survival for aggressive B cell CNS lymphoma. Neuro-Oncology 15(8):1068–1073CrossRefPubMedPubMedCentralGoogle Scholar
  66. 66.
    Skiest DJ, Crosby C (2003) Survival is prolonged by highly active antiretroviral therapy in AIDS patients with primary central nervous system lymphoma. AIDS 17:1787CrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • Fernando Silva Picon
    • 1
  • Adrialdo José Santos
    • 2
  • Hakaru Tadokoro
    • 3
  • Ramon Andrade De Mello
    • 4
    • 5
    Email author
  1. 1.Department of Medical Oncology, Escola Paulista de MedicinaUniversidade Federal de São PauloSão PauloBrazil
  2. 2.Neuro-Oncology, Hospital São PauloUniversidade Federal de São PauloSão PauloBrazil
  3. 3.Division of Medical Oncology, Escola Paulista de MedicinaFederal University of São PauloSão PauloBrazil
  4. 4.Division of Medical OncologyFederal University of São Paulo (UNIFESP) & Hospital Israelita Albert EinsteinSão PauloBrazil
  5. 5.Department of Biomedical Sciences and MedicineUniversity of AlgarveFaroPortugal

Personalised recommendations