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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...

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Autores principales: Navahi, Roshanak Aliakbar, Babaheidarian, Pegah, Sedaghat, Ahad, Naseripour, Masood, Aghai, Gholamhoseyn, Mardani, Azam, Ahangarani, Mohammadreza
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2018
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.
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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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