Frederick A Jakobiec1,2,3,4, Fouad R Zakka1,2,3,4, Thanos Papakostas1,5,3,4, Shizuo Mukai1,5,3,4. 1. Department of Ophthalmology, David G. Cogan Laboratory of Ophthalmic Pathology, Boston, MA, USA. 2. David G. Cogan Laboratory of Ophthalmic Pathology, Boston, MA, USA. 3. Massachusetts Eye and Ear Infirmary, Boston, MA, USA. 4. Harvard Medical School, Boston, MA, USA. 5. Retina Service, Massachusetts Eye and Ear Infirmary, Boston, MA, USA.
Abstract
PURPOSE: To investigate the source of fibrous astrocytes and neuroblasts in a small ciliary body medulloepithelioma appearing as a leukocoria in a 3-week-old baby girl. METHODS: Histopathologic and immunohistochemical studies included Alcian blue, periodic acid-Schiff, and antisera for the detection of S100 protein, CD99, glial fibrillary acidic protein (GFAP), CRX, NeuN, neurofilaments, synaptophysin, desmin, and myogenin. RESULTS: A small, nonteratoid ciliary body medulloepithelioma with collections of Alcian blue+ mucoplysaccharides was present in the enucleated globe. The retinal mass displayed multilaminar dysplastic rosettes that were CRX+, NeuN-, and synaptophysin-. Intraretinal neurofilaments and scattered NeuN+ neurocytes were also identified. At the base of the retinal mass ribbons and pseudopapillae of CRX+, NeuN- medullary epithelium were found. The latter developed from an S100+ and weakly CD99+ monolayer of premedullary epithelium. GFAP+ fibrous astrocytes and NeuN- neuroblasts streamed from the medullary epithelium. CONCLUSIONS: A multilaminar medullary epithelium and a precursor monolayer of premedullary epithelium were both identified. Neuroblasts and fibrous astrocytes were determined to arise separately from the medullary epithelium. The early stage of tumorigenesis afforded by a small tumor provided the opportunity to discover morphologic and immunohistochemical evidence for these differentiations.
PURPOSE: To investigate the source of fibrous astrocytes and neuroblasts in a small ciliary body medulloepithelioma appearing as a leukocoria in a 3-week-old baby girl. METHODS: Histopathologic and immunohistochemical studies included Alcian blue, periodic acid-Schiff, and antisera for the detection of S100 protein, CD99, glial fibrillary acidic protein (GFAP), CRX, NeuN, neurofilaments, synaptophysin, desmin, and myogenin. RESULTS: A small, nonteratoid ciliary body medulloepithelioma with collections of Alcian blue+ mucoplysaccharides was present in the enucleated globe. The retinal mass displayed multilaminar dysplastic rosettes that were CRX+, NeuN-, and synaptophysin-. Intraretinal neurofilaments and scattered NeuN+ neurocytes were also identified. At the base of the retinal mass ribbons and pseudopapillae of CRX+, NeuN- medullary epithelium were found. The latter developed from an S100+ and weakly CD99+ monolayer of premedullary epithelium. GFAP+ fibrous astrocytes and NeuN- neuroblasts streamed from the medullary epithelium. CONCLUSIONS: A multilaminar medullary epithelium and a precursor monolayer of premedullary epithelium were both identified. Neuroblasts and fibrous astrocytes were determined to arise separately from the medullary epithelium. The early stage of tumorigenesis afforded by a small tumor provided the opportunity to discover morphologic and immunohistochemical evidence for these differentiations.
Authors: Andrey Korshunov; Frederick A Jakobiec; Charles G Eberhart; Volker Hovestadt; David Capper; David T W Jones; Dominik Sturm; Anna M Stagner; Deepak P Edward; Ralph C Eagle; Alan D Proia; Arend Koch; Marina Ryzhova; Anastasia Ektova; Ulrich Schüller; Olga Zheludkova; Peter Lichter; Andreas von Deimling; Stefan M Pfister; Marcel Kool Journal: Neuropathology Date: 2015-07-16 Impact factor: 1.906
Authors: Frederick A Jakobiec; Matthew F Rose; Danielle Trief; Anna M Stagner; Ivana Kim; Evangelos S Gragoudas Journal: Clin Exp Ophthalmol Date: 2014-10-22 Impact factor: 4.207
Authors: Frederick A Jakobiec; Marcel Kool; Anna M Stagner; Stefan M Pfister; Ralph C Eagle; Alan D Proia; Andrey Korshunov Journal: Am J Ophthalmol Date: 2015-03-05 Impact factor: 5.258
Authors: Swathi Kaliki; Carol L Shields; Ralph C Eagle; Geeta K Vemuganti; Ana Almeida; Fairooz P Manjandavida; Kaustubh Mulay; Santosh G Honavar; Jerry A Shields Journal: Ophthalmology Date: 2013-06-21 Impact factor: 12.079
Authors: Frederick A Jakobiec; Fouad R Zakka; Robert D'Amato; Margaret M Deangelis; David S Walton; Rajesh C Rao Journal: J AAPOS Date: 2011-12 Impact factor: 1.220
Authors: Frederick A Jakobiec; Danielle Trief; Alia Rashid; Matthew F Rose; Don Minckler; Deborah Vanderveen; Shizuo Mukai Journal: Am J Ophthalmol Date: 2014-08-28 Impact factor: 5.258
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