Brain Stem Gliomas overview

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Brain Stem Gliomas Microchapters

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Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Brain Stem Gliomas from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

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Case #1

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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.

References

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