Brain Stem Gliomas medical therapy

Brain Stem Gliomas Microchapters
Home
Overview
Historical Perspective
Classification
Pathophysiology
Causes
Differentiating Brain Stem Gliomas from other Diseases
Epidemiology and Demographics
Risk Factors
Screening
Natural History, Complications and Prognosis
Diagnosis
History and Symptoms
Physical Examination
Laboratory Findings
X Ray
CT
MRI
Echocardiography or Ultrasound
Other Imaging Findings
Other Diagnostic Studies
Treatment
Medical Therapy
Surgery
Cost-Effectiveness of Therapy
Future or Investigational Therapies
Case Studies
Case #1
Brain Stem Gliomas medical therapy On the Web
Most recent articles
Most cited articles
Review articles
CME Programs
Powerpoint slides
Images
American Roentgen Ray Society Images of Brain Stem Gliomas medical therapy All Images X-rays Echo & Ultrasound CT Images MRI
Ongoing Trials at Clinical Trials.gov
US National Guidelines Clearinghouse
NICE Guidance
FDA on Brain Stem Gliomas medical therapy
CDC on Brain Stem Gliomas medical therapy
Brain Stem Gliomas medical therapy in the news
Blogs on Brain Stem Gliomas medical therapy
Directions to Hospitals Treating Brain Stem Gliomas
Risk calculators and risk factors for Brain Stem Gliomas medical therapy

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]

Overview

The optimal therapy for brainstem gliomas depends on the subtype and whether it is newly diagnosed or recurrent. Patients with diffuse brainstem gliomas are treated with radiotherapy and chemotherapy, whereas patients with focal brainstem gliomas are treated with surgical resection with or without radiation therapy and chemotherapy.

Medical therapy

The treatment is significantly influenced by tumor type, morphology and location. Radiation and chemotherapy are a key part of the treatment regime. For treatment purposes, patients are grouped as having newly diagnosed or recurrent disease.

The overview of the treatment regime is as follows:

1. Newly diagnosed brainstem gliomas

A. Diffuse brainstem gliomas

• Radiation therapy
• Chemotherapy

B. Focal brainstem gliomas

• Surgical resection (with or without radiation therapy and chemotherapy)
• Observation (with or without cerebrospinal fluid diversion)
• Radiation therapy, chemotherapy, and alternative approaches for inoperable focal or low-grade tumors

2. Recurrent brainstem gliomas

A. Diffuse brainstem gliomas

• Palliative care

B. Focal brainstem gliomas

• Repeat surgical resection
• Radiation therapy
• Chemotherapy

Newly Diagnosed Brainstem Gliomas

Diffuse brainstem gliomas

The standard treatment options for newly diagnosed diffuse brainstem gliomas include the following:

1) Radiation therapy.

• Conventional treatment for children with diffuse brainstem glioma is radiation therapy. The conventional dose of radiation ranges between 54 Gy and 60 Gy given locally to the primary tumor site in single daily fractions. Such treatment will result in transient benefit for most patients, but more than 90% of patients will die within 18 months of diagnosis. Radiation-induced changes may occur a few months after the completion of radiation therapy and may mimic tumor progression. When considering the efficacy of additional treatment, care needs to be taken to separate radiation-induced change from progressive disease.
• The efficacy of hyperfractionated and hypofractionated radiation therapy and radiosensitizers have not demonstrated improved outcomes using these radiation techniques.

• Hyperfractionated (twice daily) radiation therapy techniques have been used to deliver a higher dose, and studies using doses as high as 78 Gy have been completed. Evidence demonstrates that these increased radiation therapy doses do not improve the duration or rate of survival for patients with DIPGs, whether given alone or in combination with chemotherapy.
• Hypofractionated radiation therapy results in survival rates comparable to conventional fractionated radiation therapy techniques, possibly with less treatment burden.
• Studies evaluating the efficacy of various radiosensitizers as a means for enhancing the therapeutic effect of radiation therapy have been undertaken but to date have failed to show any significant improvement in outcome.

2) Chemotherapy only (infants)

• Similar to the treatment of other brain tumors, radiation therapy is often omitted for infants with diffuse brainstem gliomas, and chemotherapy-only approaches are utilized.

Focal brainstem gliomas

The standard treatment options for newly diagnosed focal brainstem gliomas include the following:

1) Surgical resection (with or without radiation therapy and chemotherapy)

• In general, maximal surgical resection is attempted. Patients with residual tumor may be candidates for additional therapy, including 3-dimensional conformal radiation therapy approaches, with or without adjuvant chemotherapy.

2) Observation (with or without cerebrospinal fluid diversion)

• Patients with small tectal lesions and hydrocephalus but no other neurological deficits may be treated with cerebrospinal fluid diversion alone and have follow-up with sequential neuroradiographic studies unless there is evidence of progressive disease. As focal brainstem gliomas are low grade and often very slow growing, shunting is often the only required intervention for long term survival.
• In the minority of patients who progress, radiotherapy often leads to local control or even tumor regression. Surgical excision is sometimes necessary.
• Imaging predictors of patients who will need further treatment include a size greater than 2.5 cm and presence of contrast enhancement.
• A period of observation may be indicated before instituting any treatment for patients with neurofibromatosis type 1. Brain stem gliomas in these children may be indolent and may require no specific treatment for years.

3) Radiation therapy, chemotherapy, and alternative approaches for inoperable focal or low-grade tumors

• In selected circumstances, adjuvant therapy in the form of radiation therapy or chemotherapy can be considered in a child with a newly diagnosed focal or low-grade brain stem glioma. Decisions regarding the need for such therapy depend on the age of the child, the extent of resection obtainable, and associated neurologic deficits.
• Alternative approaches for the treatment of inoperable brain stem gliomas include the following:

• Stereotactic iodine I-125 brachytherapy approaches, with or without adjuvant chemotherapy
• The use of BRAF inhibitors for tumors harboring a V600E mutation

Recurrent Brainstem Gliomas

Diffuse brainstem gliomas

The standard treatment option for recurrent diffuse brainstem gliomas include the following:

1) Palliative care

• Given the dismal prognosis for patients with diffuse brainstem gliomas, progression of the pontine lesion is anticipated generally within 1 year of completing radiation therapy. In most cases, biopsy at the time of clinical or radiologic progression is not recommended. To date, no salvage regimen has been shown to extend survival. Patients should be considered for entry into trials of novel therapeutic approaches because there are no standard agents that have demonstrated a clinically significant activity.
• Palliative care is provided for these patients whether or not disease-directed therapy is administered.

Focal brainstem gliomas

The treatment considerations at the time of recurrence are dependent on prior treatment. The standard treatment options for recurrent focal brainstem gliomas include the following:

1) Repeat surgical resection

• The need for surgical intervention must be individualized on the basis of the initial tumor type, the location within the brain stem, the length of time between initial treatment, the appearance of the mass lesion, and the clinical picture.

2) Radiation therapy

• The radiation therapy include 3-dimensional conformal radiation therapy.

3) Chemotherapy

• Carboplatin and vincristine may be effective in children with recurrent low-grade exophytic gliomas.

References

Template:WikiDoc Sources