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. Grading of Medulloepithelioma Ocul Oncol Pathol 2016;2:190–193 DOI: 10.1159/000443963 191 Table 1. Clinical and histopathological details of published adult cases of intraocular medulloepithelioma First author [Ref.], year Age, years Sex Diagnosis Treatment Followup Recurrence Adjuvant Soudakoff, 1936a 28 M Malignant nonteratoid Enucleation 13 mo No No Sayed [10], 1962 35 F Malignant nonteratoid Enucleation 6 mo No NR Zimmerman, 1972a 32 F Malignant teratoid Enucleation NR No No Broughton [8], 1978a 41 NR Benign NR NR NR NR Carrillo, 1979a 43 F Malignant teratoid Enucleation 5 y No No Floyd, 1982a 79 M Benign nonteratoid Enucleation NR NR No Litricin, 1985a 50 M Malignant teratoid Enucleation NR No No Shields, 1996a 58 F Malignant nonteratoid Exenteration and RT 2 y Parotid metastasis NR Husain, 1998a 62 M Malignant nonteratoid Neoadjuvant RT and enucleation 6 mo No No Jumper, 1999a 45 M Malignant Exenteration and adjuvant RT NR NR NR Davidorf, 2002a 23 F Medulloepithelioma BT and resection 18 mo No No Font [11], 2005 27 M Malignant nonteratoid Resection and enucleation 6 y No No Sosinska-Mielcarek [9], 2006 44 M Malignant teratoid Enucleation 15 mo Intracranial invasion CT/RT Pushker [12], 2008 38 F Malignant non-teratoid Enucleation NR NR NR Meel [13], 2010 40 M Malignant non-teratoid Enucleation 6 mo LN metastasis CT/RT Singh [14], 2010 40 M Malignant non-teratoid Enucleation 7 mo LN metastasis CT/RT Ali [15], 2013 67 F Malignant non-teratoid Enucleation 5 y No No mo = Months; NR = not reported; y = years; RT = radiotherapy; BT = brachytherapy; CT = chemotherapy; LN = lymph node. a All referenced in Sosinska-Mielcarek et al. [9]. a b c