Astrocytic brain tumors - Symptoms, Causes, Images, and Treatment Options

Diseases

Highlights & Basics

Key Highlights

Gliomas may be described as circumscribed or diffuse.
Circumscribed gliomas (e.g., pleomorphic xanthoastrocytoma, subependymal giant cell astrocytoma, pilocytic/pilomyxoid astrocytoma) have well-defined margins, and are generally benign. Maximal safe resection is the mainstay of treatment.
Diffuse astrocytomas (grade 2-4) represent a spectrum of the same highly infiltrative disease, with lower grades inevitably progressing to higher-grade lesions. They can be characterized on the basis of the isocitrate dehydrogenase (IDH) and histone 3 (H3) mutation status.
IDH-wildtype diffuse glioma (glioblastoma) is an aggressive primary brain tumor that presents in patients in the sixth or seventh decade of life.
IDH-mutant gliomas (including oligodendroglioma, IDH-mutant, 1p/19q codeleted, grades 2-3; and astrocytoma, IDH-mutant, grades 2-4) tend to occur in young or middle-age patients.

MRI demonstrating a right temporal glioblastoma (grade 4)

From the collection of Karine Michaud, University of California, San Francisco

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Simplified classification of diffuse gliomas based on the 2021 WHO classification of CNS tumors. *Astrocytoma, IDH-mutant, can be diagnosed as grade 2, 3, or 4 based on histopathologic grading criteria and cyclin-dependent kinase inhibitor 2A/cyclin-dependent kinase inhibitor 2B (CDKN2A/B) status. ***Oligodendroglioma, IDH-mutant with codeletion of the short arm of chromosome 1 and the long arm of chromosome 19 (1p19q), can be diagnosed as grade 2 or 3 based on histopathologic features
T2-weighted MRI without (A) and with (B) contrast demonstrating a pontine glioma
MRI: T2 and T1 post-contrast, demonstrating a tectal glioma (grade 2)
MRI demonstrating a right temporal glioblastoma (grade 4)
MRI demonstrating a right frontal grade 2 diffuse astrocytoma
MRI demonstrating a cerebellar pilocytic astrocytoma (grade 1)
Important molecular tests to establish the diagnosis and prognosis of glioblastoma

Citations

Key Articles

Louis DN, Perry A, Wesseling P, et al. The 2021 WHO classification of tumors of the central nervous system: a summary. Neuro Oncol. 2021 Aug 2;23(8):1231-51.[Abstract][Full Text]
National Comprehensive Cancer Network. NCCN clinical practice guidelines in oncology: central nervous system cancers [internet publication].[Full Text]
Weller M, van den Bent M, Preusser M, et al. EANO guidelines on the diagnosis and treatment of diffuse gliomas of adulthood. Nat Rev Clin Oncol. 2021 Mar;18(3):170-86.[Abstract][Full Text]
Mohile NA, Messersmith H, Gatson NT, et al. Therapy for diffuse astrocytic and oligodendroglial tumors in adults: ASCO-SNO guideline. J Clin Oncol. 2022 Feb 1;40(4):403-26.[Abstract][Full Text]
Liau LM. Guidelines for newly diagnosed glioblastoma. J Neurooncol. 2008 Sept;89(3).[Full Text]
Buckner JC, Shaw EG, Pugh SL, et al. Radiation plus procarbazine, CCNU, and vincristine in low-grade glioma. N Engl J Med. 2016 Apr 7;374(14):1344-55.[Abstract][Full Text]
American Association of Neurological Surgeons; Congress of Neurological Surgeons. Updated AANS/CNS guidelines for progressive glioblastoma patients. Jun 2022 [internet publication].[Full Text]

Referenced Articles

1. Louis DN, Perry A, Wesseling P, et al. The 2021 WHO classification of tumors of the central nervous system: a summary. Neuro Oncol. 2021 Aug 2;23(8):1231-51.[Abstract][Full Text]
2. Burkhard C, Di Patre PL, Schuler D, et al. A population-based study of the incidence and survival rates in patients with pilocytic astrocytoma. J Neurosurg. 2003 Jun;98(6):1170-4.[Abstract]
3. Ostrom QT, Price M, Neff C, et al. CBTRUS statistical report: primary brain and other central nervous system tumors diagnosed in the United States in 2015-2019. Neuro Oncol. 2022 Oct 5;24(5 suppl):v1-95.[Abstract][Full Text]
4. Thakkar JP, Dolecek TA, Horbinski C, et al. Epidemiologic and molecular prognostic review of glioblastoma. Cancer Epidemiol Biomarkers Prev. 2014 Oct;23(10):1985-96.[Abstract][Full Text]
5. Ferner RE, Huson SM, Thomas N, et al. Guidelines for the diagnosis and management of individuals with neurofibromatosis 1. J Med Genet. 2007 Feb;44(2):81-8.[Abstract][Full Text]
6. Northrup H, Aronow ME, Bebin EM, et al. Updated international tuberous sclerosis complex diagnostic criteria and surveillance and management recommendations. Pediatr Neurol. 2021 Oct;123:50-66.[Abstract][Full Text]
7. Miller JJ, Gonzalez Castro LN, McBrayer S, et al. Isocitrate dehydrogenase (IDH) mutant gliomas: a Society for Neuro-Oncology (SNO) consensus review on diagnosis, management, and future directions. Neuro Oncol. 2023 Jan 5;25(1):4-25.[Abstract][Full Text]
8. World Health Organization. Central nervous system tumours: WHO classification of tumours. 5th ed. vol 6. Lyon, France: IARD Press; 2021.
9. Paulino AC, Mai WY, Chintagumpala M, et al. Radiation-induced malignant gliomas: is there a role for reirradiation? Int J Radiat Oncol Biol Phys. 2008 Aug 1;71(5):1381-7.[Abstract]
10. Gurney JG, Kadan-Lottick N. Brain and other central nervous system tumors: rates, trends and epidemiology. Curr Opin Oncol. 2001 May;13(3):160-6.[Abstract]
11. Braganza MZ, Kitahara CM, Berrington de González A, et al. Ionizing radiation and the risk of brain and central nervous system tumors: a systematic review. Neuro Oncol. 2012 Nov;14(11):1316-24.[Abstract][Full Text]
12. Campian J, Gutmann DH. CNS tumors in neurofibromatosis. J Clin Oncol. 2017 Jul 20;35(21):2378-85.[Abstract][Full Text]
13. Roth J, Roach ES, Bartels U, et al. Subependymal giant cell astrocytoma: diagnosis, screening, and treatment - recommendations from the International Tuberous Sclerosis Complex Consensus Conference 2012. Pediatr Neurol. 2013 Dec;49(6):439-44.[Abstract][Full Text]
14. Rocca V, Blandino G, D'Antona L, et al. Li-Fraumeni syndrome: mutation of TP53 is a biomarker of hereditary predisposition to tumor: new insights and advances in the treatment. Cancers (Basel). 2022 Jul 27;14(15):3664.[Abstract][Full Text]
15. Hamilton SR, Liu B, Parsons RE, et al. The molecular basis of Turcot's syndrome. N Engl J Med. 1995 Mar 30;332(13):839-47.[Abstract][Full Text]
16. Schüz J, Pirie K, Reeves GK, et al. Cellular telephone use and the risk of brain tumors: update of the UK million women study. J Natl Cancer Inst. 2022 May 9;114(5):704-11.[Abstract][Full Text]
17. Ostrom QT, Bauchet L, Davis FG, et al. The epidemiology of glioma in adults: a "state of the science" review. Neuro Oncol. 2014 Jul;16(7):896-913.[Abstract][Full Text]
18. Turner MC. Epidemiology: allergy history, IgE, and cancer. Cancer Immunol Immunother. 2012 Sep;61(9):1493-510.[Abstract]
19. Wrensch M, Minn Y, Chew T, et al. Epidemiology of primary brain tumors: current concepts and review of the literature. Neuro Oncol. 2002 Oct;4(4):278-99.[Abstract][Full Text]
20. McKinnon C, Nandhabalan M, Murray SA, et al. Glioblastoma: clinical presentation, diagnosis, and management. BMJ. 2021 Jul 14;374:n1560.[Abstract]
21. National Comprehensive Cancer Network. NCCN clinical practice guidelines in oncology: central nervous system cancers [internet publication].[Full Text]
22. Weller M, van den Bent M, Preusser M, et al. EANO guidelines on the diagnosis and treatment of diffuse gliomas of adulthood. Nat Rev Clin Oncol. 2021 Mar;18(3):170-86.[Abstract][Full Text]
23. Gritsch S, Batchelor TT, Gonzalez Castro LN. Diagnostic, therapeutic, and prognostic implications of the 2021 World Health Organization classification of tumors of the central nervous system. Cancer. 2022 Jan 1;128(1):47-58.[Abstract][Full Text]
24. Rudà R, Capper D, Waldman AD, et al. EANO - EURACAN - SNO guidelines on circumscribed astrocytic gliomas, glioneuronal, and neuronal tumors. Neuro Oncol. 2022 Dec 1;24(12):2015-34.[Abstract][Full Text]
25. Gonzalez Castro LN, Milligan TA. Seizures in patients with cancer. Cancer. 2020 Apr 1;126(7):1379-89.[Abstract][Full Text]
26. Johnson DR, Glenn CA, Javan R, et al. Congress of Neurological Surgeons systematic review and evidence-based guidelines update on the role of imaging in the management of progressive glioblastoma in adults. J Neurooncol. 2022 Jun;158(2):139-65.[Abstract]
27. Wen PY, Weller M, Lee EQ, et al. Glioblastoma in adults: a Society for Neuro-Oncology (SNO) and European Society of Neuro-Oncology (EANO) consensus review on current management and future directions. Neuro Oncol. 2020 Aug 17;22(8):1073-113.[Abstract][Full Text]
28. McAleenan A, Jones HE, Kernohan A, et al. Diagnostic test accuracy and cost-effectiveness of tests for codeletion of chromosomal arms 1p and 19q in people with glioma. Cochrane Database Syst Rev. 2022 Mar 2;3(3):CD013387.[Abstract][Full Text]
29. Snyder H, Robinson K, Shah D, et al. Signs and symptoms of patients with brain tumors presenting to the emergency department. J Emerg Med. 1993 May-Jun;11(3):253-8.[Abstract]
30. Alentorn A, Hoang-Xuan K, Mikkelsen T. Presenting signs and symptoms in brain tumors. Handb Clin Neurol. 2016;134:19-26.[Abstract]
31. DeAngelis LM. Brain tumors. N Engl J Med. 2001 Jan 11;344(2):114-23.[Abstract]
32. Whittle IR, Pringle AM, Taylor R. Effects of resective surgery for left-sided intracranial tumors on language function: a prospective study. Lancet. 1998 Apr 4;351(9108):1014-8.[Abstract]
33. Wu JS, Zhou LF, Tang WJ, et al. Clinical evaluation and follow-up outcome of diffusion tensor imaging-based functional neuronavigation: a prospective, controlled study in patients with gliomas involving pyramidal tracts. Neurosurgery. 2007 Nov;61(5):935-48.[Abstract]
34. Kickingereder P, Andronesi OC. Radiomics, metabolic, and molecular MRI for brain tumors. Semin Neurol. 2018 Feb;38(1):32-40.[Abstract]
35. Grimstad IA, Hirschberg H, Rootwelt K. 99m Tc-hexamethyl propyleneamine oxime leukocyte scintigraphy and C-reactive protein levels in the differential diagnosis of brain abscess. J Neurosurg. 1992 Nov;77(5):732-6.[Abstract]
36. Berkman JM, Nakhate V, Gonzalez Castro LN. Glioblastoma in patients with multiple sclerosis. Neurohospitalist. 2022 Oct;12(4):607-16.[Abstract]
37. National Comprehensive Cancer Network. NCCN clinical practice guidelines in oncology: pediatric central nervous system cancers [internet publication].[Full Text]
38. Stupp R, Brada M, van den Bent MJ, et al. High-grade glioma: ESMO clinical practice guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2014 Sep;25(suppl 3):iii93-101.[Abstract]
39. Mohile NA, Messersmith H, Gatson NT, et al. Therapy for diffuse astrocytic and oligodendroglial tumors in adults: ASCO-SNO guideline. J Clin Oncol. 2022 Feb 1;40(4):403-26.[Abstract][Full Text]
40. Walbert T, Harrison RA, Schiff D, et al. SNO and EANO practice guideline update: anticonvulsant prophylaxis in patients with newly diagnosed brain tumors. Neuro Oncol. 2021 Nov 2;23(11):1835-44.[Abstract][Full Text]
41. Roth P, Pace A, Le Rhun E, et al. Neurological and vascular complications of primary and secondary brain tumours: EANO-ESMO clinical practice guidelines for prophylaxis, diagnosis, treatment and follow-up. Ann Oncol. 2021 Feb;32(2):171-82.[Abstract][Full Text]
42. Youssef G, Wen PY. Medical and neurological management of brain tumor complications. Curr Neurol Neurosci Rep. 2021 Sep 20;21(10):53.[Abstract]
43. Liau LM. Guidelines for newly diagnosed glioblastoma. J Neurooncol. 2008 Sept;89(3).[Full Text]
44. Zhang L, Li D, Xiao D, et al. Improving brain health by identifying structure-function relations in patients with neurosurgical disorders. BMJ. 2020 Oct 9;371:m3690.[Abstract][Full Text]
45. Fountain DM, Bryant A, Barone DG, et al. Intraoperative imaging technology to maximise extent of resection for glioma: a network meta-analysis. Cochrane Database Syst Rev. 2021 Jan 4;1(1):CD013630.[Abstract][Full Text]
46. Dhawan S, Patil CG, Chen C, et al. Early versus delayed postoperative radiotherapy for treatment of low-grade gliomas. Cochrane Database Syst Rev. 2020 Jan 20;1(1):CD009229.[Abstract][Full Text]
47. Halasz LM, Attia A, Bradfield L, et al. Radiation therapy for IDH-mutant grade 2 and grade 3 diffuse glioma: an ASTRO clinical practice guideline. Pract Radiat Oncol. 2022 Sep-Oct;12(5):370-86.[Abstract][Full Text]
48. Bell EH, Zhang P, Shaw EG, et al. Comprehensive genomic analysis in NRG oncology/RTOG 9802: a phase III trial of radiation versus radiation plus procarbazine, lomustine (CCNU), and vincristine in high-risk low-grade glioma. J Clin Oncol. 2020 Oct 10;38(29):3407-17.[Abstract][Full Text]
49. Lawrie TA, Gillespie D, Dowswell T, et al. Long-term neurocognitive and other side effects of radiotherapy, with or without chemotherapy, for glioma. Cochrane Database Syst Rev. 2019 Aug 5;8(8):CD013047.[Abstract][Full Text]
50. Yu Y, Villanueva-Meyer J, Grimmer MR, et al. Temozolomide-induced hypermutation is associated with distant recurrence and reduced survival after high-grade transformation of low-grade IDH-mutant gliomas. Neuro Oncol. 2021 Nov 2;23(11):1872-84.[Abstract][Full Text]
51. Buckner JC, Shaw EG, Pugh SL, et al. Radiation plus procarbazine, CCNU, and vincristine in low-grade glioma. N Engl J Med. 2016 Apr 7;374(14):1344-55.[Abstract][Full Text]
52. van den Bent MJ, Tesileanu CMS, Wick W, et al. Adjuvant and concurrent temozolomide for 1p/19q non-co-deleted anaplastic glioma (CATNON; EORTC study 26053-22054): second interim analysis of a randomised, open-label, phase 3 study. Lancet Oncol. 2021 Jun;22(6):813-23.[Abstract][Full Text]
53. Khan L, Soliman H, Sahgal A, et al. External beam radiation dose escalation for high grade glioma. Cochrane Database Syst Rev. 2020 May 21;5(5):CD011475.[Abstract][Full Text]
54. Stupp R, Taillibert S, Kanner A, et al. Effect of tumor-treating fields plus maintenance temozolomide vs maintenance temozolomide alone on survival in patients with glioblastoma: a randomized clinical trial. JAMA. 2017 Dec 19;318(23):2306-16.[Abstract][Full Text]
55. Hart MG, Grant R, Garside R, et al. Chemotherapy wafers for high grade glioma. Cochrane Database Syst Rev. 2011 Mar 16;(3):CD007294.[Abstract][Full Text]
56. Patrick HH, Sherman JH, Elder JB, et al. Congress of neurological surgeons systematic review and evidence-based guidelines update on the role of cytoreductive surgery in the management of progressive glioblastoma in adults. J Neurooncol. 2022 Jun;158(2):167-77.[Abstract]
57. American Association of Neurological Surgeons; Congress of Neurological Surgeons. Updated AANS/CNS guidelines for progressive glioblastoma patients. Jun 2022 [internet publication].[Full Text]
58. Goodman AL, Velázquez Vega JE, Glenn C, et al. Congress of neurological surgeons systematic review and evidence-based guidelines update on the role of neuropathology in the management of progressive glioblastoma in adults. J Neurooncol. 2022 Jun;158(2):179-224.[Abstract]
59. McBain C, Lawrie TA, Rogozińska E, et al. Treatment options for progression or recurrence of glioblastoma: a network meta-analysis. Cochrane Database Syst Rev. 2021 May 4;5(1):CD013579.[Abstract][Full Text]
60. Ziu M, Goyal S, Olson JJ. Congress of Neurological Surgeons systematic review and evidence-based guidelines update on the role of radiation therapy in the management of progressive and recurrent glioblastoma in adults. J Neurooncol. 2022 Jun;158(2):255-64.[Abstract]
61. Germano IM, Ziu M, Wen P, et al. Congress of Neurological Surgeons systematic review and evidence-based guidelines update on the role of cytotoxic chemotherapy and other cytotoxic therapies in the management of progressive glioblastoma in adults. J Neurooncol. 2022 Jun;158(2):225-53.[Abstract]
62. Wang H, Guo J, Wang T, et al. Efficacy and safety of bevacizumab in the treatment of adult gliomas: a systematic review and meta-analysis. BMJ Open. 2021 Dec 2;11(12):e048975.[Abstract][Full Text]
63. Winograd E, Germano I, Wen P, et al. Congress of Neurological Surgeons systematic review and evidence-based guidelines update on the role of targeted therapies and immunotherapies in the management of progressive glioblastoma. J Neurooncol. 2022 Jun;158(2):265-321.[Abstract][Full Text]
64. Taylor LP, Besbris JM, Graf WD, et al. Clinical guidance in neuropalliative care: an AAN position statement. Neurology. 2022 Mar 8;98(10):409-16.[Abstract][Full Text]
65. Mellinghoff IK, van den Bent MJ, Blumenthal DT, et al. Vorasidenib in IDH1- or IDH2-mutant low-grade glioma. N Engl J Med. 2023 Aug 17;389(7):589-601.[Abstract]
66. Lombardi G, De Salvo GL, Brandes AA, et al. Regorafenib compared with lomustine in patients with relapsed glioblastoma (REGOMA): a multicentre, open-label, randomised, controlled, phase 2 trial. Lancet Oncol. 2019 Jan;20(1):110-9.[Abstract]
67. Juric V, Murphy B. Cyclin-dependent kinase inhibitors in brain cancer: current state and future directions. Cancer Drug Resist. 2020;3(1):48-62.[Abstract][Full Text]
68. Bota DA, Mason W, Kesari S, et al. Marizomib alone or in combination with bevacizumab in patients with recurrent glioblastoma: phase I/II clinical trial data. Neurooncol Adv. 2021 Jan-Dec;3(1):vdab142.[Abstract][Full Text]
69. ClinicalTrials.gov. Stage 1: marizomib + bevacizumab in WHO Gr IV GBM; Stage 2: marizomib alone; Stage 3: combination of marizomib and bevacizumab. Jun 2022 [internet publication].[Full Text]
70. Sim HW, Galanis E, Khasraw M. PARP inhibitors in glioma: a review of therapeutic opportunities. Cancers (Basel). 2022 Feb 16;14(4):1003.[Abstract][Full Text]
71. Reardon DA, Brandes AA, Omuro A, et al. Effect of nivolumab vs bevacizumab in patients with recurrent glioblastoma: the CheckMate 143 phase 3 randomized clinical trial. JAMA Oncol. 2020 Jul 1;6(7):1003-10.[Abstract][Full Text]
72. Lee A, Arasaratnam M, Chan DLH, et al. Anti-epidermal growth factor receptor therapy for glioblastoma in adults. Cochrane Database Syst Rev. 2020 May 12;5(5):CD013238.[Abstract][Full Text]
73. Youssef G, Dietrich J. Ipilimumab: an investigational immunotherapy for glioblastoma. Expert Opin Investig Drugs. 2020 Nov;29(11):1187-93.[Abstract]
74. Cloughesy TF, Petrecca K, Walbert T, et al. Effect of vocimagene amiretrorepvec in combination with flucytosine vs standard of care on survival following tumor resection in patients with recurrent high-grade glioma: a randomized clinical trial. JAMA Oncol. 2020 Dec 1;6(12):1939-46.[Abstract][Full Text]
75. Liau LM, Ashkan K, Brem S, et al. Association of autologous tumor lysate-loaded dendritic cell vaccination with extension of survival among patients with newly diagnosed and recurrent glioblastoma: a phase 3 prospective externally controlled cohort trial. JAMA Oncol. 2023 Jan 1;9(1):112-21.[Abstract][Full Text]
76. Platten M, Bunse L, Wick A, et al. A vaccine targeting mutant IDH1 in newly diagnosed glioma. Nature. 2021 Apr;592(7854):463-8.[Abstract][Full Text]
77. Maggs L, Cattaneo G, Dal AE, et al. CAR T cell-based immunotherapy for the treatment of glioblastoma. Front Neurosci. 2021 May 25;15:662064.[Abstract][Full Text]
78. Majzner RG, Ramakrishna S, Yeom KW, et al. GD2-CAR T cell therapy for H3K27M-mutated diffuse midline gliomas. Nature. 2022 Mar;603(7903):934-41.[Abstract][Full Text]
79. Kristeleit R, Evans J, Molife LR, et al. Phase 1/2a trial of intravenous BAL101553, a novel controller of the spindle assembly checkpoint, in advanced solid tumours. Br J Cancer. 2020 Oct;123(9):1360-9.[Abstract][Full Text]
80. Ha W, Sevim-Nalkiran H, Zaman AM, et al. Ibudilast sensitizes glioblastoma to temozolomide by targeting macrophage migration Inhibitory Factor (MIF). Sci Rep. 2019 Feb 27;9(1):2905.[Abstract][Full Text]
81. Dhillon S. Decitabine/cedazuridine: first approval. Drugs. 2020 Sep;80(13):1373-8.[Abstract][Full Text]
82. Dhillon S. Correction to: Decitabine/cedazuridine: first approval. Drugs. 2021 Jan;81(1):179.[Abstract][Full Text]
83. Pace A, Dirven L, Koekkoek JAF, et al. European Association for Neuro-Oncology (EANO) guidelines for palliative care in adults with glioma. Lancet Oncol. 2017 Jun;18(6):e330-40.[Abstract]
84. Johnson DR, Brown PD, Galanis E, et al. Pilocytic astrocytoma survival in adults: analysis of the surveillance, epidemiology, and end results program of the National Cancer Institute. J Neurooncol. 2012 May;108(1):187-93.[Abstract]
85. Tabash MA. Characteristics, survival and incidence rates and trends of pilocytic astrocytoma in children in the United States; SEER-based analysis. J Neurol Sci. 2019 May 15;400:148-52.[Abstract]
86. von Deimling A, Ono T, Shirahata M, et al. Grading of diffuse astrocytic gliomas: a review of studies before and after the advent of IDH testing. Semin Neurol. 2018 Feb;38(1):19-23.[Abstract]
87. Hamilton MG, Hull RD, Pineo GH. Venous thromboembolism in neurosurgery and neurology patients: a review. Neurosurgery. 1994 Feb;34(2):280-96.[Abstract]
88. Beevers Z, Hussain S, Boele FW, et al. Pharmacological treatment of depression in people with a primary brain tumour. Cochrane Database Syst Rev. 2020 Jul 17;7(7):CD006932.[Abstract][Full Text]
89. Kirkman MA, Day J, Gehring K, et al. Interventions for preventing and ameliorating cognitive deficits in adults treated with cranial irradiation. Cochrane Database Syst Rev. 2022 Nov 25;11(11):CD011335.[Abstract][Full Text]