Brain Stem Gliomas natural history, complications and prognosis
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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
If left untreated, patients with brainstem gliomas may progress to develop increased intracranial pressure and cerebral herniation. Common complications of brainstem gliomas include loss of motor and sensory functions and loss of regulation of basic body functions like blood pressure, swallowing and respiration. Prognosis is generally good for dorsal exophytic and cervicomedullary brainstem gliomas, and diffuse subtype has the worst prognosis with treatment.
Natural history
- If left untreated, patients with brainstem gliomas may progress to develop increased intracranial pressure and cerebral herniation.
- Radiotherapy-induced neoplasms tend to be more aggressive in their natural history than their de novo counterparts.
- The duration of symptoms is usually much shorter in diffuse gliomas, in which the history is typically very short (a few days).
- FBSG usually present with greater than 3 months of symptoms, while DIPG are usually diagnosed within 3 months of symptom onset.[1]
- Diffuse gliomas are associated with multiple cranial nerve palsies.
- Clinical manifestations of brainstem glioma depend on the following:[2]
- Location of the tumor
- Size of the tumor
- Growth rate of the tumor
- Patient's age
Complications
Complications of brainstem gliomas include the following:
- Altered blood pressure
- Hypopnea
- Dysphagia
- Loss of motor and sensory functions
Sudden death can result from increased intracranial pressure and subsequent cerebral herniation. This may be a consequence either of edema induced by the tumor or of hemorrhage into the neoplasm.
Prognosis
Factors associated with worse prognosis include the following:[3]
- The type of brain stem glioma
- Where the tumor is found in the brain and if it has spread within the brain stem
- Age of the child when diagnosed
- Children younger than 3 years may have a more favorable prognosis
- Whether or not the child has a condition called neurofibromatosis type 1
- Children with NF1 and brain stem gliomas may have a better prognosis than other patients who have intrinsic lesions
- Whether the tumor has been newly diagnosed or has recurred
As a general rule, dorsal exophytic tumors and cervicomedullary tumors tend to have a good prognosis with treatment, and diffuse type has the worst prognosis with treatment.[4] The median survival for children with diffuse brainstem glioma is less than 1 year. In contrast, focal brainstem gliomas have a markedly improved prognosis, with 5-year overall survival exceeding 90%. Adults tend to have a better prognosis than childhood brainstem gliomas.
- Diffuse brainstem glioma
- Terrible prognosis
- 90-100% patients die within 2 years of diagnosis
- Focal (tectal glioma)
- Excellent long term survival with CSF shunting (essentially benign lesions)
- Focal (other)
- Good long-term prognosis with surgery
- (Dorsally) exophytic tumors
- Good long-term prognosis with surgery
References
- ↑ Green AL, Kieran MW (2015). "Pediatric brainstem gliomas: new understanding leads to potential new treatments for two very different tumors". Curr Oncol Rep. 17 (3): 436. doi:10.1007/s11912-014-0436-7. PMID 25702179.
- ↑ Symptoms of brainstem tumors. Cancer gov. http://www.cancer.gov/types/brain/patient/child-glioma-treatment-pdq#link/stoc_h2_2
- ↑ Prognosis of brainstem tumors. Cancer gov. http://www.cancer.gov/types/brain/patient/child-glioma-treatment-pdq#link/stoc_h2_2
- ↑ Prognosis of Brainstem gliomas. Dr Yuranga Weerakkody and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/brainstem-glioma