Brain Stem Gliomas overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Brainstem gliomas are tumors that occur in the region of the brain referred to as the brain stem, which is the area between the aqueduct of Sylvius and the fourth ventricle.
Historical prospective
In the era preceding modern imaging, all brainstem gliomas were regarded as a solitary pathological entity with poor prognosis. In the late 1960s, Matson suggested that all brainstem tumors were malignant and were deemed inoperable regardless of their histopathological characteristics or location.This assertion was questioned shortly thereafter by Pool, who was one of the first to report tumor resection in the brainstem, which in the case described was inside the aqueduct. In 1980, Hoffman et. described the dorsally exophytic group of brainstem gliomas as a distinct subgroup, and reported that these lesions were surgically curable with aggressive resection. Over the past 3 decades, the treatment of brainstem gliomas has notably progressed as a result of the gradual advancements in microsurgical techniques, sophisticated imaging technology and, most importantly, the availability of MRI. These modalities have revealed that brainstem gliomas are a heterogeneous group of tumors.
Classification
Brainstem gliomas may be classified into four subtypes: diffuse, focal, dorsal exophytic, and cervicomedullary.
Pathophysiology
On gross pathology, brainstem gliomas can be classified into four subtypes: diffuse, focal, dorsal exophytic and cervicomedullary. Each of the four subtypes has its distinct microscopic pathology. Genes involved in the pathogenesis of diffuse intrinsic pontine gliomas include Histone H3, ACVR1, PDGFRA, and TP53.
Epidemiology and Demographics
The incidence of brainstem gliomas is estimated to be 0.05 - 0.1 cases per 100,000 individuals in the USA. A bimodal distribution by age is noted with peak incidences rates in children and older adults. The prevalence and incidence of brainstem gliomas do not vary by either race or gender.
Risk Factors
Neurofibromatosis type 1 is a common risk factor in the development of brainstem glioma.
Natural history, complications and prognosis
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.