eMedicine Specialties > Neurology > Neuro-oncology

Brainstem Gliomas: Follow-up

Author: Joseph Landolfi, DO, Director of Neuro-oncology, New Jersey Neuroscience Institute Brain Tumor Center; Medical Director, Gamma Knife Program, JFK Medical Center; Associate Professor of Neurology, Seton Hall University School of Graduate Medical Education
Coauthor(s): Anita Venkataramana, MBBS, Clinical Instructor, Department of Neurology, Division of Neuroimmunology/HIV, Johns Hopkins University
Contributor Information and Disclosures

Updated: Jun 30, 2009

Follow-up

Further Inpatient Care

Typically, patients are monitored for worsening signs/symptoms. Admission to the hospital may be required to enable therapeutic intervention and stabilization.

Further Outpatient Care

Follow-up neuroimaging with MRI (unless contraindicated) is recommended within 72 hours after surgery and every 2-3 months to monitor response to therapy and progression of disease. This should be considered standard care for these patients.

Complications

  • Hydrocephalus, commonly obstructive
  • Growth and developmental delay (in children)
  • Cerebral herniation
  • Deep cerebral vein thrombosis
  • Paralysis/paresis
  • Cranial nerve deficits
  • Meningitis
  • Radiation necrosis
  • Hypopituitarism/hypothyroidism
  • Bone marrow suppression
  • Cognitive dysfunction

Prognosis

  • Pontine tumors are the most common variety of brainstem tumor. They also carry the worst prognosis; in children, the median survival duration is 9-12 months even with treatment. Kaplan et al reported a 37% survival rate at 1 year, 20% at 2 years, and 13% at 3 years, with a median survival of 10 months. Only 9 of 119 patients in their study were alive for more than 3 years after diagnosis.7
  • Squires et al, in a study of 12 children with midbrain tectal tumors, reported a median survival duration of more than 50 months.8
  • Favorable prognostic factors include (1) neurofibromatosis, (2) symptoms of at least 12 months' duration before diagnosis, (3) exophytic location, (4) pathology suggestive of low-grade tumor histology, (5) focal tectal and cervicomedullary tumors, and (6) calcification on CT scan.
  • Poor prognostic indicators include (1) age younger than 2 years, (2) multiple brainstem signs, (3) cranial nerve palsies, (4) diffuse intrinsic lesions of the pons, (5) short duration of signs and symptoms prior to the time of diagnosis, and (6) high-grade histology on tumor biopsy.
  • Hydrocephalus and tumor necrosis do not affect survival.
  • Race and gender do not affect survival.
  • The limited available data suggest that adults fare better than children with brainstem gliomas.
    • Grigsby et al reported a 10-year disease-free survival rate of 15.4% for adult patients with gliomas involving the midbrain, thalamus, or hypothalamus, and 29.6% for adults with pontine or medullary tumors. However, thalamic/hypothalamic neoplasms are not included historically in the classification of brainstem tumors.9
    • Landolfi et al studied 19 adults with brainstem gliomas, which included 13 diffuse intrinsic pontine, 4 cervicomedullary, and 2 tectal gliomas. They noted a trend that higher Karnofsky performance status conferred a better prognosis. Other factors did not affect survival. Median survival duration of patients in this study was 54 months, with a 5-year survival rate of 45%.10
    • Hamilton et al studied 16 adults with focal midbrain gliomas; they reported a median survival of 84 months. This indolent growth pattern is in marked contradistinction to the natural history of this disease in children. This is also the reverse of the usual behavior of hemispheric gliomas in which children typically fare better than older patients.11
    • Kesari et al reported on 101 adult patients with brainstem glioma. The overall survival for all patients at 5 and 10 years was 58% and 41%, respectively. The median survival was 85 months. They identified 4 factors that were significantly associated with survival in adults with brainstem gliomas. These factors included ethnicity, tumor location, age at diagnosis, and tumor grade.12
    • No explanation has been identified for the better outcome in adults; however, the possibility of prolonged survival with limited neurologic impairment must be recognized when counseling adults with brainstem gliomas.

Patient Education

Patients and families of patients acquire information from multiple sources, including, but not limited to, physician, patients, support groups, pharmaceutical companies, and the Internet. Physicians should be aware of this and have an open, informative relationship with their patients, empowering patients to become active members of the team with regard to the decision-making process involving their care.

Miscellaneous

Medicolegal Pitfalls

  • Failure to identify presenting signs and symptoms, thus causing a delay in, or missing, the diagnosis
  • Not recognizing the limitation of ones' knowledge and failing to obtain MRI imaging studies or to call upon appropriate consultations, especially in neuroradiology and neurosurgery, and occasionally in psychiatry or psychology, for patients whose symptoms are misinterpreted as being "functional"
 


More on Brainstem Gliomas

Overview: Brainstem Gliomas
Differential Diagnoses & Workup: Brainstem Gliomas
Treatment & Medication: Brainstem Gliomas
Follow-up: Brainstem Gliomas
Multimedia: Brainstem Gliomas
References
Further Reading
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References

  1. Frazier JL, Lee J, Thomale UW, Noggle JC, Cohen KJ, Jallo GI. Treatment of diffuse intrinsic brainstem gliomas: failed approaches and future strategies. J Neurosurg Pediatr. Apr 2009;3(4):259-69. [Medline].

  2. Ueoka DI, Nogueira J, Campos JC, Maranhão Filho P, Ferman S, Lima MA. Brainstem gliomas--retrospective analysis of 86 patients. J Neurol Sci. Jun 15 2009;281(1-2):20-3. [Medline].

  3. Grau SJ, Rachinger W, Holtmannspoetter M, Herms J, Tonn JC, Kreth FW. Serial Stereotactic Biopsy of Brainstem Lesions in Adults Improves Diagnostic Accuracy Compared to MRI Only. J Neurol Neurosurg Psychiatry. Jun 10 2009;[Medline].

  4. Frappaz D, Schell M, Thiesse P et al. Preradiation chemotherapy may improve survival in pediatric diffuse intrinsic pontine gliomas: Final results of BSG 98 prospective trial. Neuro Oncol. Aug/2008;10(4):599-607. [Medline].

  5. Raza S, Donach M. Bevacizumab in adult malignant brainstem gliomas. J Neurooncol. Jun 9 2009;[Medline].

  6. Vesper J, Bölke B, Wille C, Gerber PA, Matuschek C, Peiper M, et al. Current concepts in stereotactic radiosurgery - a neurosurgical and radiooncological point of view. Eur J Med Res. Mar 17 2009;14(3):93-101. [Medline].

  7. Kaplan AM, Albright AL, Zimmerman RA, Rorke LB, Li H, Boyett JM, et al. Brainstem gliomas in children. A Children's Cancer Group review of 119 cases. Pediatr Neurosurg. 1996;24(4):185-92. [Medline].

  8. Squires LA, Allen JC, Abbott R, Epstein FJ. Focal tectal tumors: management and prognosis. Neurology. May 1994;44(5):953-6. [Medline].

  9. Grigsby PW, Garcia DM, Simpson JR, et al. Prognostic factors and results of therapy for adult thalamic and brainstem tumors. Cancer. Jun 1 1989;63(11):2124-9. [Medline].

  10. Landolfi JC, Thaler HT, DeAngelis LM. Adult brainstem gliomas. Neurology. Oct 1998;51(4):1136-9. [Medline].

  11. Hamilton MG, Lauryssen C, Hagen N. Focal midbrain glioma: long term survival in a cohort of 16 patients and the implications for management. Can J Neurol Sci. Aug 1996;23(3):204-7. [Medline].

  12. Kesari S, Kim RS, Markos V, Drappatz J, Wen PY, Pruitt AA. Prognostic factors in adult brainstem gliomas: a multicenter, retrospective analysis of 101 cases. J Neurooncol. Jun 2008;88(2):175-83. [Medline].

  13. Abbott R, Shiminski-Maher T, Epstein FJ. Intrinsic tumors of the medulla: predicting outcome after surgery. Pediatr Neurosurg. Jul 1996;25(1):41-4. [Medline].

  14. Albright AL, Guthkelch AN, Packer RJ, et al. Prognostic factors in pediatric brain-stem gliomas. J Neurosurg. Dec 1986;65(6):751-5. [Medline].

  15. Barkovich AJ, Krischer J, Kun LE, et al. Brain stem gliomas: a classification system based on magnetic resonance imaging. Pediatr Neurosurg. 1990-91;16(2):73-83. [Medline].

  16. Cohen ME, Duffner PK. 2nd ed. Brain Tumors in Children: Principles of Diagnosis and Treatment. New York: Raven Press; 1994.

  17. Cohen ME, Duffner PK, Heffner RR, et al. Prognostic factors in brainstem gliomas. Neurology. May 1986;36(5):602-5. [Medline].

  18. Dunkel IJ, O'Malley B, Finlay JL. Is there a role for high-dose chemotherapy with stem cell rescue for brain stem tumors of childhood?. Pediatr Neurosurg. 1996;24(5):263-6. [Medline].

  19. Edwards MS, Wara WM, Urtasun RC, et al. Hyperfractionated radiation therapy for brain-stem glioma: a phase I-II trial. J Neurosurg. May 1989;70(5):691-700. [Medline].

  20. Epstein F, Wisoff J. Intra-axial tumors of the cervicomedullary junction. J Neurosurg. Oct 1987;67(4):483-7. [Medline].

  21. Fenichel Gerald M. Clinical Pediatric Neurology: A Signs and Symptoms Approach. 3rd ed. Philadelphia: WB Saunders Company; 1997.

  22. Guiney MJ, Smith JG, Hughes P, et al. Contemporary management of adult and pediatric brain stem gliomas. Int J Radiat Oncol Biol Phys. Jan 15 1993;25(2):235-41. [Medline].

  23. Jallo GI, Biser-Rohrbaugh A, Freed D. Brainstem gliomas. Childs Nerv Syst. Mar 2004;20(3):143-53. [Medline].

  24. Kaye AH, Laws ER. Brain Tumors. New York: Churchill Livingstone; 1995.

  25. Milstein JM, Geyer JR, Berger MS, Bleyer WA. Favorable prognosis for brainstem gliomas in neurofibromatosis. J Neurooncol. Nov 1989;7(4):367-71. [Medline].

  26. Rosenblum RK. Brain stem glioma: two case studies. J Pediatr Oncol Nurs. Mar-Apr 2005;22(2):114-8. [Medline].

  27. Tokuriki Y, Handa H, Yamashita J, et al. Brainstem glioma: an analysis of 85 cases. Acta Neurochir (Wien). 1986;79(2-4):67-73. [Medline].

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Keywords

brainstem tumors, pontine lesions, tectal lesions, hydrocephalus, cervicomedullary lesions, neurofibromatosis, intrinsic pontine gliomas, tectal gliomas, cervicomedullary gliomas, intracranial tumors

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Contributor Information and Disclosures

Author

Joseph Landolfi, DO, Director of Neuro-oncology, New Jersey Neuroscience Institute Brain Tumor Center; Medical Director, Gamma Knife Program, JFK Medical Center; Associate Professor of Neurology, Seton Hall University School of Graduate Medical Education
Joseph Landolfi, DO is a member of the following medical societies: Alpha Omega Alpha
Disclosure: Schering-Plough Honoraria Speaking and teaching; Genetech Honoraria Speaking and teaching

Coauthor(s)

Anita Venkataramana, MBBS, Clinical Instructor, Department of Neurology, Division of Neuroimmunology/HIV, Johns Hopkins University
Disclosure: Nothing to disclose.

Medical Editor

Edward L Hogan, MD, Professor, Department of Neurology, Medical College of Georgia; Emeritus Professor and Chair, Department of Neurology, Medical University of South Carolina
Edward L Hogan, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Neurology, American Neurological Association, American Society for Biochemistry and Molecular Biology, Phi Beta Kappa, Sigma Xi, Society for Neuroscience, and Southern Clinical Neurological Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Jorge Kattah, MD, Head, Program Director, Professor, Department of Neurology, University of Illinois College of Medicine at Peoria
Jorge Kattah, MD is a member of the following medical societies: American Academy of Neurology, American Neurological Association, and New York Academy of Sciences
Disclosure: Biogen Honoraria Consulting; Bayer Corporation Honoraria Consulting

CME Editor

Matthew J Baker, MD, Consulting Staff, Collier Neurologic Specialists, Naples Community Hospital
Matthew J Baker, MD is a member of the following medical societies: American Academy of Neurology
Disclosure: Nothing to disclose.

Chief Editor

Tarakad S Ramachandran, MBBS, FRCP(C), FACP, Professor of Neurology, Clinical Professor of Medicine, Clinical Professor of Family Medicine, Clinical Professor of Neurosurgery, State University of New York Upstate Medical University; Chair, Department of Neurology, Crouse Irving Memorial Hospital
Tarakad S Ramachandran, MBBS, FRCP(C), FACP is a member of the following medical societies: American Academy of Neurology, American Academy of Pain Medicine, American College of Forensic Examiners, American College of International Physicians, American College of Managed Care Medicine, American College of Physicians, American Heart Association, American Stroke Association, Royal College of Physicians, Royal College of Physicians and Surgeons of Canada, Royal College of Surgeons of England, and Royal Society of Medicine
Disclosure: Abbott Labs  Honoraria Consulting; Teva Marion Honoraria Consulting; Boeringer-Ingelheim Honoraria Speaking and teaching

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