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Medulloepithelioma
Other Resources UpToDate PubMed

Medulloepithelioma

Contributors: Carla Casulo MD, Michael W. Winter MD, Richard L. Barbano MD, PhD
Other Resources UpToDate PubMed

Synopsis

A malignant and uncommon cancer of the central nervous system arising from the medullary epithelium. Usually develops in the parietal and temporal lobes, but may occur in the eye (intraocular medulloepithelioma). It typically affects children and infants. May be present at birth, but usually not diagnosed until early childhood. Characterized by headache (increased intracranial pressure), rubeosis iridis, leukocoria, focal neurologic deficit, and visual impairment. Signs and symptoms include pain, an ocular mass, glaucoma, cataracts, and seizures.

Prognosis may be good if treated before tumor spreads, but may not spare the eye.

Treatment usually involves enucleation. Other treatments may include tumor resection, radiation therapy, and chemotherapy.

Codes

ICD10CM:
C47.0 – Malignant neoplasm of peripheral nerves of head, face and neck

SNOMEDCT:
39005004 – Medulloepithelioma

Differential Diagnosis & Pitfalls

To perform a comparison, select diagnoses from the classic differential

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Best Tests

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References

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Last Updated:04/19/2016
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Medulloepithelioma
A medical illustration showing key findings of Medulloepithelioma : Headache, Visual impairment, Leukocoria, Rubeosis iridis, Focal neurologic deficit
Copyright © 2024 VisualDx®. All rights reserved.