Brain Stem Gliomas pathophysiology: Difference between revisions
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:*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 | :*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 | :*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 tumors''' | |||
Revision as of 13:39, 27 August 2015
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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
Brainstem gliomas are situated in the pons, medulla and midbrain. 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 tumors
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