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Retinal anaplastic pleomorphic xanthoastrocytoma unassociated with phakomatosis
PURPOSE: To present a rare anaplastic form of retinal pleomorphic xanthoastrocytoma (PXA) unassociated with phakomatosis. METHODS: A 9-year-old girl, presented with a rapidly growing unilateral intraocular white mass unresponsive to intra-arterial chemotherapy, underwent enucleation with the clinica...
Autores principales: | , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Elsevier
2018
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6611984/ https://www.ncbi.nlm.nih.gov/pubmed/31317107 http://dx.doi.org/10.1016/j.joco.2018.09.005 |
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author | Navahi, Roshanak Aliakbar Babaheidarian, Pegah Sedaghat, Ahad Naseripour, Masood Aghai, Gholamhoseyn Mardani, Azam Ahangarani, Mohammadreza |
author_facet | Navahi, Roshanak Aliakbar Babaheidarian, Pegah Sedaghat, Ahad Naseripour, Masood Aghai, Gholamhoseyn Mardani, Azam Ahangarani, Mohammadreza |
author_sort | Navahi, Roshanak Aliakbar |
collection | PubMed |
description | PURPOSE: To present a rare anaplastic form of retinal pleomorphic xanthoastrocytoma (PXA) unassociated with phakomatosis. METHODS: A 9-year-old girl, presented with a rapidly growing unilateral intraocular white mass unresponsive to intra-arterial chemotherapy, underwent enucleation with the clinical suspicion of retinoblastoma versus malignant astrocytoma. RESULTS: Histopathology revealed pleomorphic cells with rosenthal fibers, mitosis, and necrosis. Immunohistochemistry confirmed the diagnosis of anaplastic pleomorphic xanthoastrocytoma (aPXA). The patient had no signs of phakomatosis. CONCLUSION: Retinal PXA may occur in patients without phakomatosis and rarely progress toward malignant transformation. |
format | Online Article Text |
id | pubmed-6611984 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2018 |
publisher | Elsevier |
record_format | MEDLINE/PubMed |
spelling | pubmed-66119842019-07-17 Retinal anaplastic pleomorphic xanthoastrocytoma unassociated with phakomatosis Navahi, Roshanak Aliakbar Babaheidarian, Pegah Sedaghat, Ahad Naseripour, Masood Aghai, Gholamhoseyn Mardani, Azam Ahangarani, Mohammadreza J Curr Ophthalmol Article PURPOSE: To present a rare anaplastic form of retinal pleomorphic xanthoastrocytoma (PXA) unassociated with phakomatosis. METHODS: A 9-year-old girl, presented with a rapidly growing unilateral intraocular white mass unresponsive to intra-arterial chemotherapy, underwent enucleation with the clinical suspicion of retinoblastoma versus malignant astrocytoma. RESULTS: Histopathology revealed pleomorphic cells with rosenthal fibers, mitosis, and necrosis. Immunohistochemistry confirmed the diagnosis of anaplastic pleomorphic xanthoastrocytoma (aPXA). The patient had no signs of phakomatosis. CONCLUSION: Retinal PXA may occur in patients without phakomatosis and rarely progress toward malignant transformation. Elsevier 2018-10-11 /pmc/articles/PMC6611984/ /pubmed/31317107 http://dx.doi.org/10.1016/j.joco.2018.09.005 Text en © 2018 Iranian Society of Ophthalmology. Production and hosting by Elsevier B.V. http://creativecommons.org/licenses/by-nc-nd/4.0/ This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/). |
spellingShingle | Article Navahi, Roshanak Aliakbar Babaheidarian, Pegah Sedaghat, Ahad Naseripour, Masood Aghai, Gholamhoseyn Mardani, Azam Ahangarani, Mohammadreza Retinal anaplastic pleomorphic xanthoastrocytoma unassociated with phakomatosis |
title | Retinal anaplastic pleomorphic xanthoastrocytoma unassociated with phakomatosis |
title_full | Retinal anaplastic pleomorphic xanthoastrocytoma unassociated with phakomatosis |
title_fullStr | Retinal anaplastic pleomorphic xanthoastrocytoma unassociated with phakomatosis |
title_full_unstemmed | Retinal anaplastic pleomorphic xanthoastrocytoma unassociated with phakomatosis |
title_short | Retinal anaplastic pleomorphic xanthoastrocytoma unassociated with phakomatosis |
title_sort | retinal anaplastic pleomorphic xanthoastrocytoma unassociated with phakomatosis |
topic | Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6611984/ https://www.ncbi.nlm.nih.gov/pubmed/31317107 http://dx.doi.org/10.1016/j.joco.2018.09.005 |
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