Brain Stem Gliomas pathophysiology
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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
Pathophysiology
As a general rule, mesencephalic tumors tend to be of a lower grade than those in the pons and medulla.
pontine most common location classic location for the childhood 'brainstem glioma' which tends to refer to a diffuse pontine glioma focal dorsally exophytic brainstem glioma is an uncommon variant accounting for only 10% of pontine tumours, and has a much better prognosis, as it usually represents a pilocytic astrocytoma overall survival of pontine gliomas is 10% at 5 years
mesencephalic includes diffuse, focal, exophytic and tectal variants. focal brainstem gliomas are more common here than elsewhere in the brainstem tectal plate gliomas are typically indolent
medullary least common location includes focal dorsally exophytic, focal, diffuse and cervicomedullary junction variants cervicomedullary junction tumours usually represent upper cervical tumours extending superiorly most common location for NF1 associated tumours
The brain stem gliomas are classified into 4 subtypes, and each has its distinct histological features:[1]
- Diffuse brainstem gliomas
- Usually fibrillary astrocytomas
- WHO grades II-IV
- Grade does not impact on prognosis, and thus biopsy is usually not necessary
- 75% of brain stem gliomas
- Focal glioma
- Fibrillary astrocytoma (grade II): most common histology
- Pilocytic astrocytoma
- Ganglioglioma
- (Dorsally) exophytic glioma
- Low grade astrocytoma
- Ganglioglioma
- NF1-associated brainstem glioma
- Seen in up to 9% of NF1 patients
- Most frequently seen in the medulla
- Appears similar to a sporadic focal brainstem glioma but has an even better prognosis, with little if any progression
References
- ↑ Pathophysiology of Brainstem gliomas. Dr Yuranga Weerakkody and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/brainstem-glioma