On the Classification and Grading of Medulloepithelioma of the Eye

Medulloepithelioma is a rare congenital tumor of the ciliary body and iris. The current classification and grading of medulloepithelioma shows inconsistencies and does not reflect clinical behavior. This position paper discusses the backgrounds of the current classification and intends to initiate a discussion on an alternative classification and grading scheme. © 2016 The Author(s) Published by S. Karger AG, Basel Recently, I had to produce the pathology report of a case of intraocular medulloepithelioma, a rare congenital tumor of the ciliary body and iris. In the preparation of a histopathological diagnosis, my attention was brought to inconsistencies in the WHO classification scheme. The most recent WHO fascicle on the international histological classification of tumors of the eye and its adnexa [1] offers two separate classifying entities of medulloepithelioma: teratoid or nonteratoid, both in a benign and a Received: November 30, 2015 Accepted after revision: January 11, 2016 Published online: February 13, 2016 Robert M. Verdijk, MD, PhD Section of Ophthalmic Pathology, Department of Pathology Erasmus MC University Medical Center, Room No. Be228, PO Box 2040 NL–3000 CA Rotterdam (The Netherlands) E-Mail r.verdijk @ erasmusmc.nl © 2016 The Author(s) Published by S. Karger AG, Basel 2296–4681/16/0023–0190$39.50/0 www.karger.com/oop Th is article is licensed under the Creative Commons AttributionNonCommercial-NoDerivatives 4.0 International License (CC BYNC-ND) (http://www.karger.com/Services/OpenAccessLicense). Usage and distribution for commercial purposes as well as any distribution of modifi ed material requires written permission. D ow nl oa de d by : 54 .1 91 .4 0. 80 9 /1 7/ 20 17 8 :3 8: 58 A M