Christopher K H Burris1, Vasilios P Papastefanou2, Caroline Thaung3, Gordon Hay4, Marianne Grantham5, Victoria M L Cohen2. 1. Department of Ophthalmology, University of Wisconsin-Madison, Madison, Wis., USA. 2. Department of Ocular Oncology, St. Bartholomew's Hospital, London, UK; Department of Ocular Oncology, Moorfields Eye Hospital, London, UK. 3. Department of Eye Pathology, UCL Institute of Ophthalmology, London, UK. 4. Department of Ocular Oncology, St. Bartholomew's Hospital, London, UK. 5. Department of Molecular Medicine, Royal London Hospital, London, UK.
Abstract
BACKGROUND: Medulloepithelioma is a rare congenital neoplasm derived from precursors of the nonpigmented ciliary epithelium of the ciliary body. The average patient age at clinical presentation is 3.8 years. CASE PRESENTATION: We present the case of a 78-year-old male with progressive lens subluxation and ocular hypertension who was found to have a ciliary body mass. After enucleation for presumed ciliary body melanoma, histopathology showed a nonteratoid medulloepithelioma. Cytogenetic analysis revealed abnormalities in chromosomes 3 and 8. CONCLUSION: Medulloepithelioma is often initially misdiagnosed. Though congenital in nature, it can exhibit rapid growth, have chromosomal abnormalities, and must be considered in all age groups.
BACKGROUND: Medulloepithelioma is a rare congenital neoplasm derived from precursors of the nonpigmented ciliary epithelium of the ciliary body. The average patient age at clinical presentation is 3.8 years. CASE PRESENTATION: We present the case of a 78-year-old male with progressive lens subluxation and ocular hypertension who was found to have a ciliary body mass. After enucleation for presumed ciliary body melanoma, histopathology showed a nonteratoid medulloepithelioma. Cytogenetic analysis revealed abnormalities in chromosomes 3 and 8. CONCLUSION: Medulloepithelioma is often initially misdiagnosed. Though congenital in nature, it can exhibit rapid growth, have chromosomal abnormalities, and must be considered in all age groups.
Authors: K Sisley; I G Rennie; M A Parsons; R Jacques; D W Hammond; S M Bell; A M Potter; R C Rees Journal: Genes Chromosomes Cancer Date: 1997-05 Impact factor: 5.006
Authors: Felix Sahm; Frederick A Jakobiec; Jochen Meyer; Daniel Schrimpf; Charles G Eberhart; Volker Hovestadt; David Capper; Sander Lambo; Marina Ryzhova; Ulrich Schüller; Olga Zheludkova; Ella Kumirova; Peter Lichter; Andreas von Deimling; David T W Jones; Stefan M Pfister; Marcel Kool; Andrey Korshunov Journal: Genes Chromosomes Cancer Date: 2016-02-04 Impact factor: 5.006