Subependymoma (WHO Grade 1)

Last edit by Alaric Steinmetz on

Synonyms: Subependymoma

ICD-11: XH8FZ9

Subependymomas are slow-growing tumors and are classified as WHO grade 1 tumors.

Epidemiology

Subependymomas account for 0.51 % of all tumors in the central nervous system and approximately 8 % of all ependymal tumors[^1] [^2].

Etiology

Subependymomas arise from the Ependymal cells in the Ventricles, protrude into the ventricular system, and do not grow invasively.

Diagnosis

Subependymomas are usually an incidental finding on imaging or become symptomatic due to Hydrocephalus. Subependymomas can occur in the Brain as well as the Spinal cord.

Imaging

The gold standard for imaging subependymomas is MRI imaging. On MRI, subependymomas appear as hypo- to hyperintense lesions on T1- and T2-weighted sequences and usually show no or only minimal contrast enhancement[^1] [^2].

Intramedullary subependymoma on MRI
Visualization of a histopathologically verified intramedullary subependymoma in the cervical spine on various MRI sequences.
Subependymoma MRI
Three different MRI sequences (T2, T1, and T1 with contrast medium from left to right) in a patient with a subependymoma in the left lateral ventricle.
Contrast enhancement

Subependymomas typically appear as non-enhancing intraventricular lesions[^1].

Treatment

Subependymomas are slow-growing tumors and can be monitored if they are asymptomatic. If surgical removal is indicated, gross total resection (GTR) is usually curative, and subtotal resection is also generally sufficient due to the slow tumor growth[^1].

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