Primary Brain Tumors in Adults: Diagnosis and Treatment

 

Am Fam Physician. 2016 Feb 1;93(3):211-217B.

  Patient information: See related handout on primary brain tumors, written by the authors of this article.

Author disclosure: No relevant financial affiliations.

Primary intracranial tumors of the brain structures, including meninges, are rare with an overall five-year survival rate of 33.4%; they are collectively called primary brain tumors. Proven risk factors for these tumors include certain genetic syndromes and exposure to high-dose ionizing radiation. Primary brain tumors are classified by histopathologic criteria and immunohistochemical data. The most common symptoms of these tumors are headache and seizures. Diagnosis of a suspected brain tumor is dependent on appropriate brain imaging and histopathology. The imaging modality of choice is gadolinium-enhanced magnetic resonance imaging. There is no specific pathognomonic feature on imaging that differentiates between primary brain tumors and metastatic or nonneoplastic disease. In cases of suspected or pathologically proven metastatic disease, chest and abdomen computed tomography may be helpful, although determining the site of the primary tumor is often difficult, especially if there are no clinical clues from the history and physical examination. Using fluorodeoxyglucose positron emission tomography to search for a primary lesion is not recommended because of low specificity for differentiating a neoplasm from benign or inflammatory lesions. Treatment decisions are individualized by a multidisciplinary team based on tumor type and location, malignancy potential, and the patient's age and physical condition. Treatment often includes a combination of surgery, radiotherapy, and chemotherapy. After craniotomy, patients should be followed closely for complications, including deep venous thrombosis, pulmonary embolism, intracranial bleeding, wound infection, systemic infection, seizure, depression, worsening neurologic status, and adverse drug reaction. Hospice and palliative care should be offered when appropriate throughout treatment.

Primary intracranial tumors arising from the meninges, neuroepithelial tissues, pituitary and related structures, cranial nerves, germ cells, blood-forming organs, or a distant subclinical primary tumor are known collectively as primary brain tumors. These tumors in adults are rare with an estimated 23,380 new cases diagnosed in 2014, leading to 14,320 deaths; these accounted for 1.4% of all new cases of cancer and 2.4% of all cancer deaths. The incidence of a new brain tumor is 6.4 per 100,000 persons per year with an overall five-year survival rate of 33.4%. The peak prevalence is between 55 and 64 years of age, with a slightly higher incidence in men than in women. There is an approximate 0.6% lifetime risk of being diagnosed with brain or other nervous system cancer.1

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SORT: KEY RECOMMENDATIONS FOR PRACTICE

Clinical recommendationEvidence ratingReferences

Gadolinium-enhanced magnetic resonance imaging is the preferred imaging modality in the diagnosis of suspected brain tumors.

C

15, 16

Fluorodeoxyglucose positron emission tomography should not be used initially to search for a primary tumor when metastatic disease is suspected, unless suggested by history or physical examination findings.

C

19

Treatment decisions should be individualized by a multidisciplinary team (medical oncology, radiation oncology, and neurosurgery) based on tumor type, malignancy potential, tumor location, and the patient's age and physical condition.

C

22

Hospice should be offered if life expectancy is less than six months, and in patients who have poor or worsening performance status, who are not candidates for surgery or chemotherapy, who have deteriorating neurologic functions despite therapy, or who have tumor recurrence.

C

13, 46


A = consistent, good-quality patient-oriented evidence; B = inconsistent or limited-quality patient-oriented evidence; C = consensus, disease-oriented evidence, usual practice, expert opinion, or case series. For information about the SORT evidence rating system, go to http://www.aafp.org/afpsort.

SORT: KEY RECOMMENDATIONS FOR PRACTICE

Clinical recommendationEvidence ratingReferences

Gadolinium-enhanced magnetic resonance imaging is the preferred imaging modality in the diagnosis of suspected brain tumors.

C

15, 16

Fluorodeoxyglucose positron emission tomography should not be used initially to search for a primary tumor when metastatic disease is suspected, unless suggested by history or physical examination findings.

C

19

Treatment decisions should be individualized by a multidisciplinary team (medical oncology, radiation oncology, and neurosurgery) based on tumor type, malignancy potential, tumor location, and the patient's age and physical condition.

C

22

Hospice should be offered if life expectancy is less than six months, and in patients who have poor or worsening performance status, who are not candidates for surgery or chemotherapy, who have deteriorating neurologic functions despite therapy, or who have tumor recurrence.

C

13, 46


A = consistent, good-quality patient-oriented evidence; B = inconsistent or limited-quality patient-oriented evidence; C = consensus, disease-oriented evidence,

The Authors

show all author info

ALLEN PERKINS, MD, MPH, is chairman of the Department of Family Medicine at the University of South Alabama, Mobile....

GERALD LIU, MD, is a physician with Harvard Vanguard Medical Associates in Weymouth, Mass. At the time this article was written, he was an assistant professor in the Department of Family Medicine at the University of South Alabama.

Address correspondence to Allen Perkins, MD, MPH, University of South Alabama, 1504 Springhill Ave., Ste. 3414, Mobile, AL 36604. Reprints are not available from the authors.

Author disclosure: No relevant financial affiliations.

REFERENCES

show all references

1. Howlader N, et al. SEER cancer statistics review, 1975–2012. http://seer.cancer.gov/csr/1975_2012. Accessed November 18, 2015....

2. Farrell CJ, Plotkin SR. Genetic causes of brain tumors. Neurol Clin. 2007;25(4):925–946, viii.

3. Lu Q, Dai D, Zhao W, et al. Association between MTHFR 677C>T polymorphism and risk of gliomas. Tumour Biol. 2013;34(5):2801–2807.

4. Zhang H, Liu H, Knauss JL. Associations between three XRCC1 polymorphisms and glioma risk. Tumour Biol. 2013;34(5):3003–3013.

5. Fisher JL, Schwartzbaum JA, Wrensch M, Berger MS. Evaluation of epidemiologic evidence for primary adult brain tumor risk factors using evidence-based medicine. Prog Neurol Surg. 2006;19:54–79.

6. Ostrom QT, et al. Primary brain and central nervous system tumors diagnosed in the United States in 2007–2011. Neuro Oncol. 2014;16(suppl 4): iv1–iv63.

7. Louis DN. WHO classification of tumours of the central nervous system. 4th ed. Lyon, France: International Agency for Research on Cancer; 2007.

8. Grant R. Overview: brain tumour diagnosis and management/Royal College of Physicians guidelines. J Neurol Neurosurg Psychiatry. 2004;75(suppl 2):ii18–ii23.

9. Newton HB. Primary brain tumors: review of etiology, diagnosis and treatment. Am Fam Physician. 1994;49(4):787–797.

10. Chang SM, Parney IF, Huang W, et al. Patterns of care for adults with newly diagnosed malignant glioma. JAMA. 2005;293(5):557–564.

11. Youland RS, Schomas DA, Brown PD, et al. Changes in presentation, treatment, and outcomes of adult low-grade gliomas over the past fifty years. Neuro Oncol. 2013;15(8):1102–1110.

12. Forsyth PA, Posner JB. Headaches in patients with brain tumors: a study of 111 patients. Neurology. 1993;43(9):1678–1683.

13. Chandana SR, Movva S, Arora M, Singh T. Primary brain tumors in adults. Am Fam Physician. 2008;77(10):1423–1430.

14. Taphoorn MJ, Klein M. Cognitive deficits in adult patients with brain tumours. Lancet Neurol. 2004;3(3):159–168.

15. Bradley WG Jr, Waluch V, Yadley RA, Wycoff RR. Comparison of CT and MR in 400 patients with suspected disease of the brain and cervical spinal cord. Radiology. 1984;152(3):695–702.

16. Jacobs AH, Kracht LW, Gossmann A, et al. Imaging in neurooncology. NeuroRx. 2005;2(2):333–347.

17. Treister D, Kingston S, Hoque KE, Law M, Shiroishi MS. Multimodal magnetic resonance imaging evaluation of primary brain tumors. Semin Oncol. 2014;41(4):478–495.

18. Purdy RA, Kirby S. Headaches and brain tumors. Neurol Clin. 2004;22(1):39–53.

19. Soffietti R, Cornu P, Delattre JY, et al. EFNS Guidelines on diagnosis and treatment of brain metastases. Eur J Neurol. 2006;13(7):674–681.

20. Omuro AM, Leite CC, Mokhtari K, Delattre JY. Pitfalls in the diagnosis of brain tumours. Lancet Neurol. 2006;5(11):937–948.

21. National Cancer Institute. Adult central nervous sytem tumors treatment (PDQ). Updated August 25, 2015. http://www.cancer.gov/cancertopics/pdq/treatment/adultbrain/Patient/. Accessed November 18, 2015.

22. National Institute for Health and Care Excellence. Guidance on cancer services. Improving outcomes for people with brain and other CNS tumours. The manual. June 2006. https://www.nice.org.uk/guidance/csgbraincns/evidence/improving-outcomes-for-people-with-brain-and-other-cns-tumours-the-manual2. Accessed October 13, 2015.

23. Fadul C, Wood J, Thaler H, et al. Morbidity and mortality of craniotomy for excision of supratentorial gliomas. Neurology. 1988;38(9):1374–1379.

24. Hentschel SJ, Sawaya R. Optimizing outcomes with maximal surgical resection of malignant gliomas. Cancer Control. 2003;10(2):109–114.

25. Tsitlakidis A, Foroglou N, Venetis CA, et al. Biopsy versus resection in the management of malignant gliomas: a systematic review and meta-analysis. J Neurosurg. 2010;112(5):1020–1032.

26. Sanai N, Berger MS. Glioma extent of resection and its impact on patient outcome. Neurosurgery. 2008;62(4):753–764.

27. Sanai N, Berger MS. Operative techniques for gliomas and the value of extent of resection. Neurotherapeutics. 2009;6(3):478–486.

28. Keime-Guibert F, Chinot O, Taillandier L, et al.; Association of French-Speaking Neuro-Oncologists. Radiotherapy for glioblastoma in the elderly. N Engl J Med. 2007;356(15):1527–1535.

29. Walker MD, et al. Evaluation of BCNU and/or radiotherapy in the treatment of anaplastic gliomas. J Neurosurg. 1978;49(3):333–343.

30. Mehta MP, Won M, Shaw EG, et al. Mature survival data from RTOG 9802: A phase III study of radiation therapy (RT) with or without procarbazine, CCNU, and vincristine (PCV) for adult patients with high-risk low-grade glioma (LGG). Int J Radiat Oncol. 2014;90(1 suppl):S37–S38.

31. Rock K, McArdle O, Forde P, et al. A clinical review of treatment outcomes in glioblastoma multiforme. Br J Radiol. 2012;85(1017):e729–e733.

32. Hart MG, Grant R, Garside R, et al. Chemotherapy wafers for high grade glioma. Cochrane Database Syst Rev. 2011;(3):CD007294.

33. Hart MG, Garside R, Rogers G, Stein K, Grant R. Temozolomide for high grade glioma. Cochrane Database Syst Rev. 2013;(4):CD007415.

34. Hegi ME, Diserens AC, Gorlia T, et al. MGMT gene silencing and benefit from temozolomide in glioblastoma. N Engl J Med. 2005;352(10):997–1003.

35. Quon H, Abdulkarim B. Adjuvant treatment of anaplastic oligodendrogliomas and oligoastrocytomas. Cochrane Database Syst Rev. 2008;(2):CD007104.

36. Chang SM, et al. Perioperative complications and neurological outcomes of first and second craniotomies among patients enrolled in the Glioma Outcome Project. J Neurosurg. 2003;98(6):1175–1181.

37. McGirt MJ, Chaichana KL, Attenello FJ, et al. Extent of surgical resection is independently associated with survival in patients with hemispheric infiltrating low-grade gliomas. Neurosurgery. 2008;63(4):700–707.

38. Deutsch MB, Panageas KS, Lassman AB, Deangelis LM. Steroid management in newly diagnosed glioblastoma. J Neurooncol. 2013;113(1):111–116.

39. Marras LC, Geerts WH, Perry JR. The risk of venous thromboembolism is increased throughout the course of malignant glioma: an evidence-based review. Cancer. 2000;89(3):640–646.

40. Lyman GH, et al. Venous thromboembolism prophylaxis and treatment in patients with cancer: American Society of Clinical Oncology clinical practice guideline update 2014. J Clin Oncol. 2015;33(6):654–656.

41. van Breemen MS, Wilms EB, Vecht CJ. Epilepsy in patients with brain tumours. Lancet Neurol. 2007;6(5):421–430.

42. Glantz MJ, Cole BF, Forsyth PA, et al. Practice parameter: anticonvulsant prophylaxis in patients with newly diagnosed brain tumors. Neurology. 2000;54(10):1886–1893.

43. Sirven JI, Wingerchuk DM, Drazkowski JF, et al. Seizure prophylaxis in patients with brain tumors. Mayo Clin Proc. 2004;79(12):1489–1494.

44. Catt S, Chalmers A, Fallowfield L. Psychosocial and supportive-care needs in high-grade glioma. Lancet Oncol. 2008;9(9):884–891.

45. Gehring K, Sitskoorn MM, Gundy CM, et al. Cognitive rehabilitation in patients with gliomas: a randomized, controlled trial. J Clin Oncol. 2009; 27(22):3712–3722.

46. Krex D, Klink B, Hartmann C, et al. Long-term survival with glioblastoma multiforme. Brain. 2007;130(pt 10):2596–2606.

47. Ohgaki H, Kleihues P. Population-based studies on incidence, survival rates, and genetic alterations in astrocytic and oligodendroglial gliomas. J Neuropathol Exp Neurol. 2005;64(6):479–489.

48. Pignatti F, van den Bent M, Curran D, et al.; European Organization for Research and Treatment of Cancer Brain Tumor Cooperative Group; European Organization for Research and Treatment of Cancer Radiotherapy Cooperative Group. Prognostic factors for survival in adult patients with cerebral low-grade glioma. J Clin Oncol. 2002;20(8):2076–2084.

49. Jeremic B, Milicic B, Grujicic D, Dagovic A, Aleksandrovic J, Nikolic N. Clinical prognostic factors in patients with malignant glioma treated with combined modality approach. Am J Clin Oncol. 2004;27(2):195–204.

50. Laws ER, Parney IF, Huang W, et al.; Glioma Outcomes Investigators. Survival following surgery and prognostic factors for recently diagnosed malignant glioma. J Neurosurg. 2003;99(3):467–473.


 

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