Brain Stem Gliomas medical therapy
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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 a recurrent tumor. 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.[1][2]
The overview of the treatment regime is as follows:
1. Newly diagnosed brainstem gliomas
- A. Diffuse brainstem gliomas
- 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
- B. Focal brainstem gliomas
2. Recurrent brainstem gliomas
- A. Diffuse brainstem gliomas
- B. Focal brainstem gliomas
Newly Diagnosed Brainstem Gliomas
Diffuse brainstem gliomas
The standard treatment options for newly diagnosed diffuse brainstem gliomas include the following:
1) Radiation therapy.
- Radiation therapy is the treatment of choice for children suffering from brain stem gliomas. A radiation dose of 54 to 60 Gy is typically used for treatment. Prognosis after radiation therapy remains poor as > 90% of patients die after 18 months of initial 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, a dose of 78 Gy has been used for treatment and it has been found that increasing the dose of radiation does not improve survival in these patients.
- 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)
- Small tectal lesions and hydrocephalus without neurological deficits:
- Treat with cerebrospinal fluid diversion alone and follow-up with imaging.
- Shunting for favorable long-term outcomes
- 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
- ↑ Treatment of brainstem gliomas. National Cancer Institute. http://www.cancer.gov/types/brain/hp/child-glioma-treatment-pdq#section/_45
- ↑ Rx of Brainstem gliomas. Dr Yuranga Weerakkody and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/brainstem-glioma