International Journal of Head and Neck Surgery

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VOLUME 1 , ISSUE 1 ( January-April, 2010 ) > List of Articles

CASE REPORT

Colossal Malignant Nonteratoid Medulloepithelioma of the Eye with Unusual Metastases: The Natural History

Qureshi Sajid

Citation Information : Sajid Q. Colossal Malignant Nonteratoid Medulloepithelioma of the Eye with Unusual Metastases: The Natural History. Int J Head Neck Surg 2010; 1 (1):39-42.

DOI: 10.5005/jp-journals-10001-1008

Published Online: 00-04-2010

Copyright Statement:  Copyright © 2010; Jaypee Brothers Medical Publishers (P) Ltd.


Abstract

Background

Medulloepithelioma is rare intraocular tumor seen predominantly in children, and arising mainly from undifferentiated nonpigmented epithelium of the ciliary body.

Methods

We present a typical neglected case of malignant nonteratoid medulloepithelioma of the eye with extensive metastases to the facial soft tissue parotid and cervical lymph nodes.

Results

An aggressive surgical approach with orbital exenteration and comprehensive disease clearance with total parotidectomy and neck dissection augmented by postoperative radiotherapy was utilized after initial unsuccessful chemotherapy.

Conclusions

The present case highlights the natural history of untreated medulloepithelioma of the eye. Colossal growth at the primary site with extensive regional lymph node metastases develops without distant metastases. Complete surgical excision supplemented with postoperative radiotherapy should be considered even in the setting of advanced locoregional disease.


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