eMedicine Specialties > Neurology > Neuro-oncology
Brainstem Gliomas: Treatment & Medication
Updated: Jun 30, 2009
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Treatment
Medical Care
- Treatment of brainstem gliomas has been frustrating; at this point, new therapies have yielded little benefit over conventional treatment with radiotherapy alone.
- Adjuvant chemotherapy is not used in children because efficacy has not been proven. Data have suggested that preradiation chemotherapy may improve survival in pediatric diffuse intrinsic brainstem gliomas.4 Its efficacy in adults is similarly unproven, and at present, postradiotherapy adjuvant chemotherapy cannot be recommended. The effectiveness of chemotherapy at relapse is uncertain, but it may benefit some patients.
- Chemotherapy options, when considered for use in brainstem gliomas, may include conventional agents such as temozolomide and carboplatin/vincristine. Antiangiogenesis agents have been used with success in supratentorial glioblastomas. These include thalidomide and bevacizumab. Bevacizumab is a VEGF receptor inhibitor, approved as monotherapy for recurrent glioblastoma multiforme in May 2009.5 Drugs (such as erlotinib) targeted against EGFR, when present, have been modestly effective in supratentorial glioblastoma. If chemotherapy is desired adjuvantly or concurrently with radiotherapy, particularly in the pediatric population, the physician should consider entrance into a clinical trial.
- Focal radiotherapy is the cornerstone of treatment of brainstem gliomas and can improve or stabilize the patient's condition.6 The conventional dose of radiotherapy ranges from 54-60 Gy, with doses up to 72 Gy given with hyperfractionation. At present, no benefit has been demonstrated with doses greater than 72 Gy; however, this therapy has not demonstrated efficacy in children.
- Response to radiotherapy, in addition to the dose of radiation, depends on several variables such as tumor location, histologic type, and response to early treatment. Patients who underwent radiation therapy for exophytic tumors have been reported to have better survival rates than those treated for tumors without an exophytic component.
- Radiotherapy should be administered to any patient with significant and progressive neurologic symptoms. Some adult patients with a tectal or cervicomedullary lesion, or with mild symptoms of long duration, may be candidates for observation alone; radiotherapy can be reserved for patients with clear evidence of tumor progression.
Surgical Care
Surgical resection is performed in conjunction with radiation and/or chemotherapeutic agents.
- Surgery is most appropriate in tumors of the cervicomedullary junction, dorsal exophytic tumors protruding into the fourth ventricle, cystic tumors, enhancing tumors with clear margins that exert a space-occupying effect, and finally, benign tumors (ie, those with slow clinical progression).
- Typically, biopsy and/or surgery are not required for diagnosis or treatment of diffuse intrinsic pontine or tectal gliomas and cannot be recommended routinely; diagnosis can be made by MRI alone.
Consultations
- Neuro-oncologist: The neuro-oncologist should be the primary physician supervising the care of these patients. If a neuro-oncologist is not available, a medical oncologist with expertise in treating brain tumors may be consulted for guidance. Otherwise, the patient should be referred to a reputable institution that specializes in the care of patients with CNS neoplasms.
- Neurosurgeon: The treating neurosurgeon should have significant experience in resection of CNS neoplasms.
- Radiation oncologist
- Neuropathologist
- Neuroradiologist
- Neuropsychologist for pretreatment and posttreatment evaluations, when clinically indicated
- Rehabilitation medicine specialist
Medication
The goals of pharmacotherapy are to reduce morbidity and prevent complications.
Corticosteroids
These agents have anti-inflammatory properties and cause profound and varied metabolic effects. Corticosteroids modify the body's immune response to diverse stimuli.
Dexamethasone (Decadron)
Can be used to reduce tumor- and radiotherapy-associated cerebral edema.
Adult
24 mg IV bolus followed by maintenance dose of 4 mg PO tid/qid; for intracranial neoplasms, higher doses may be required in patients with severe cerebral edema or herniation syndrome secondary to tumor
Pediatric
Not established
Coadministration with warfarin or heparin increases risk of bleeding
Documented hypersensitivity; systemic fungal infections; documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Increases risks of multiple complications, including severe infections; monitor adrenal insufficiency when tapering drug; abrupt discontinuation may cause adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections are possible complications
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
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].
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].
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].
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].
Raza S, Donach M. Bevacizumab in adult malignant brainstem gliomas. J Neurooncol. Jun 9 2009;[Medline].
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].
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].
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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].
Landolfi JC, Thaler HT, DeAngelis LM. Adult brainstem gliomas. Neurology. Oct 1998;51(4):1136-9. [Medline].
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].
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].
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].
Albright AL, Guthkelch AN, Packer RJ, et al. Prognostic factors in pediatric brain-stem gliomas. J Neurosurg. Dec 1986;65(6):751-5. [Medline].
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].
Cohen ME, Duffner PK. 2nd ed. Brain Tumors in Children: Principles of Diagnosis and Treatment. New York: Raven Press; 1994.
Cohen ME, Duffner PK, Heffner RR, et al. Prognostic factors in brainstem gliomas. Neurology. May 1986;36(5):602-5. [Medline].
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].
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].
Epstein F, Wisoff J. Intra-axial tumors of the cervicomedullary junction. J Neurosurg. Oct 1987;67(4):483-7. [Medline].
Fenichel Gerald M. Clinical Pediatric Neurology: A Signs and Symptoms Approach. 3rd ed. Philadelphia: WB Saunders Company; 1997.
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].
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Further Reading
Clinical guidelines
ACR Appropriateness Criteria® ataxia.
American College of Radiology - Medical Specialty Society. 1999 (revised 2006). 10 pages. NGC:005547
Long-term follow-up guidelines for survivors of childhood, adolescent, and young adult cancers. Sections 38-91: radiation.
Children's Oncology Group - Medical Specialty Society. 2003 Sep (revised 2006 Mar). 74 pages. NGC:005599
Clinical trials
Immunotherapy for Patients With Brain Stem Glioma and Glioblastoma
DNA Analysis of Tumor Tissue Samples From Patients With Diffuse Brain Stem Glioma
A Study of Bevacizumab Therapy in Patients With Newly Diagnosed High-Grade Gliomas and Diffuse Intrinsic Pontine Gliomas
Related eMedicine topics
Glioblastoma Multiforme (Neurology)
Glioblastoma Multiforme (Radiology)
Hydrocephalus
Neurofibromatosis, Type 1
Neurofibromatosis, Type 2
Keywords
brainstem tumors, pontine lesions, tectal lesions, hydrocephalus, cervicomedullary lesions, neurofibromatosis, intrinsic pontine gliomas, tectal gliomas, cervicomedullary gliomas, intracranial tumors
Treatment & Medication: Brainstem Gliomas