Brainstem Gliomas Treatment & Management

Updated: Jun 09, 2016
  • Author: Joseph C Landolfi, DO; Chief Editor: Tarakad S Ramachandran, MBBS, MBA, MPH, FAAN, FACP, FAHA, FRCP, FRCPC, FRS, LRCP, MRCP, MRCS  more...
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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. [2] Its efficacy in adults is similarly unproven, and at present, postradiotherapy adjuvant chemotherapy cannot be recommended. The effectiveness of combined radiotherapy and chemotherapy (typically temozolomide) has not been thoroughly evaluated. 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. [7] 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. [1] 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.

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Surgical Care

Surgical resection is performed in conjunction with radiation therapy, chemotherapy, or both. Surgery is most appropriate in the following cases:

  • 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
  • Benign tumors (ie, those with slow clinical progression)

Typically, surgery is not required for treatment of diffuse intrinsic pontine gliomas or tectal gliomas.

See Brain Cancer Treatment Protocols for summarized information.

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Ancillary Procedures

Patients with hydrocephalus may require ventriculostomy or ventriculoperitoneal shunting for symptomatic relief.

Patients with difficulties in swallowing and diminished gag reflex may need feeding by gastrostomy, such as percutaneous esophagogastrostomy (PEG).

Those patients who have had multiple upper respiratory infections, pneumonia, or altered voice may need postoperative ventilatory assistance.

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Consultations

Consultation with the following may prove helpful:

  • 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
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