PMID- 23219067 OWN - NLM STAT- MEDLINE DCOM- 20130404 LR - 20220331 IS - 1879-1891 (Electronic) IS - 0002-9394 (Print) IS - 0002-9394 (Linking) VI - 155 IP - 3 DP - 2013 Mar TI - Reactive retinal astrocytic tumors (so-called vasoproliferative tumors): histopathologic, immunohistochemical, and genetic studies of four cases. PG - 593-608.e1 LID - S0002-9394(12)00616-2 [pii] LID - 10.1016/j.ajo.2012.09.002 [doi] AB - PURPOSE: To evaluate the cellular nature of and diagnostic terminology used in connection with acquired retinal "vasoproliferative tumors." DESIGN: Retrospective clinicopathologic study. METHODS: Clinical records and microscopic slides of 4 enucleated globes were reviewed. Special stains and immunohistochemical probes for CD31, CD34, p53, glial fibrillary acidic protein (GFAP), CD163, and Ki67 (cell replication) were employed; ultrastructural and fluorescence in situ hybridization (FISH) analyses were performed. RESULTS: Tumors were located inferotemporally in middle-aged patients. They were uniformly composed of compacted elongated, GFAP-positive spindle cells (due to intermediate filaments identified ultrastructurally) with a Ki67 index of less than 1%. Rosenthal fibers and eosinophilic granular bodies were observed. Hyalinized periodic acid-Schiff-positive vessels were widely separated. CD31 and CD34 revealed a sparse microvasculature. Tumor-associated exudate spread predominantly subretinally. The retinal pigment epithelium had undergone extensive placoid fibrous metaplasia with focal ossification. P53 upregulation, BRAF-KIAA gene rearrangement, and IDH1R132H mutation typically associated with low-grade astrocytic neoplasms were absent. CONCLUSIONS: Retinal "vasoproliferative" tumors have been mischaracterized, because they actually display a paucity of microvessels. Proliferating fibrous astrocytes with a very low proliferation index predominate, without immunohistochemical or genetic evidence favoring a neoplasm. Subretinal exudate appeared capable of provoking widespread fibrous metaplasia of the pigment epithelium that was mainly responsible for secondary retinal damage. The term "reactive retinal astrocytic tumor" is proposed as more appropriate for this entity. In carefully selected progressive lesions, consideration should be given to earlier surgical intervention before extensive subretinal exudate accumulates and pigment epithelial proliferation with fibrous metaplasia ensues. CI - Copyright (c) 2013 Elsevier Inc. All rights reserved. FAU - Poole Perry, Lynn J AU - Poole Perry LJ AD - Department of Ophthalmology, Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston, MA, USA. FAU - Jakobiec, Frederick A AU - Jakobiec FA FAU - Zakka, Fouad R AU - Zakka FR FAU - Reichel, Elias AU - Reichel E FAU - Herwig, Martina C AU - Herwig MC FAU - Perry, Arie AU - Perry A FAU - Brat, Daniel J AU - Brat DJ FAU - Grossniklaus, Hans E AU - Grossniklaus HE LA - eng GR - P30 EY006360/EY/NEI NIH HHS/United States GR - P30 EY06360/EY/NEI NIH HHS/United States PT - Case Reports PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't DEP - 20121206 PL - United States TA - Am J Ophthalmol JT - American journal of ophthalmology JID - 0370500 RN - 0 (BRAF-KIAA1549 fusion protein, human) RN - 0 (Biomarkers, Tumor) RN - 0 (Neoplasm Proteins) RN - 0 (Oncogene Proteins, Fusion) RN - EC 1.1.1.41 (Isocitrate Dehydrogenase) RN - EC 1.1.1.42. (IDH1 protein, human) SB - IM CIN - Am J Ophthalmol. 2013 Jul;156(1):202-3. PMID: 23791372 CIN - Am J Ophthalmol. 2013 Jul;156(1):203. PMID: 23791373 MH - Adult MH - Aged MH - Astrocytes/metabolism/ultrastructure MH - *Astrocytoma/genetics/metabolism/pathology MH - Biomarkers, Tumor/*metabolism MH - Eye Enucleation MH - Female MH - Gene Rearrangement MH - Humans MH - Immunohistochemistry MH - In Situ Hybridization, Fluorescence MH - Isocitrate Dehydrogenase/*genetics MH - Male MH - Neoplasm Proteins/*metabolism MH - Oncogene Proteins, Fusion/*genetics MH - Polymerase Chain Reaction MH - *Retinal Neoplasms/genetics/metabolism/pathology MH - Retrospective Studies MH - Terminology as Topic PMC - PMC4470612 MID - NIHMS698650 EDAT- 2012/12/12 06:00 MHDA- 2013/04/05 06:00 PMCR- 2015/06/17 CRDT- 2012/12/11 06:00 PHST- 2012/06/28 00:00 [received] PHST- 2012/08/30 00:00 [revised] PHST- 2012/09/04 00:00 [accepted] PHST- 2012/12/11 06:00 [entrez] PHST- 2012/12/12 06:00 [pubmed] PHST- 2013/04/05 06:00 [medline] PHST- 2015/06/17 00:00 [pmc-release] AID - S0002-9394(12)00616-2 [pii] AID - 10.1016/j.ajo.2012.09.002 [doi] PST - ppublish SO - Am J Ophthalmol. 2013 Mar;155(3):593-608.e1. doi: 10.1016/j.ajo.2012.09.002. Epub 2012 Dec 6.