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Ependymoma

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Overview


Plain-Language Overview

Ependymoma is a type of tumor that develops in the brain or spinal cord, which are parts of the central nervous system. It arises from cells called ependymal cells that line the fluid-filled spaces in the brain and spinal cord. This tumor can cause symptoms by pressing on nearby brain or spinal cord tissue, leading to problems such as headaches, balance difficulties, or weakness. The tumor may block the flow of cerebrospinal fluid, causing increased pressure inside the skull. Diagnosis often involves imaging tests like MRI to locate the tumor. Treatment usually requires surgery and sometimes radiation therapy. The impact on health depends on the tumor’s size, location, and how aggressively it grows.

Clinical Definition

Ependymoma is a glial tumor originating from ependymal cells lining the ventricular system of the brain and the central canal of the spinal cord. It is classified as a WHO grade II or III tumor depending on histologic features such as cellularity, mitotic activity, and necrosis. The tumor commonly occurs in the posterior fossa in children and the spinal cord in adults. Pathogenesis involves genetic alterations including chromosome 22q deletions and sometimes REL-A fusion genes. Clinically, it presents with symptoms of increased intracranial pressure or focal neurological deficits depending on tumor location. It is significant due to its potential for local invasion, cerebrospinal fluid dissemination, and recurrence after treatment. Histopathology shows perivascular pseudorosettes and ependymal rosettes, which are diagnostic hallmarks.

Clinical Presentation


Diagnostic Workup


Pathophysiology


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Outcome & Complications


Differential Diagnoses