Retinal anaplastic pleomorphic xanthoastrocytoma unassociated with phakomatosis

To present a rare anaplastic form of retinal pleomorphic xanthoastrocytoma (PXA) unassociated with phakomatosis. 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 ret...

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Bibliographic Details
Published in:Iranian journal of ophthalmology 2019-06, Vol.31 (2), p.234-237
Main Authors: Navahi, Roshanak Aliakbar, Babaheidarian, Pegah, Sedaghat, Ahad, Naseripour, Masood, Aghai, Gholamhoseyn, Mardani, Azam, Ahangarani, Mohammadreza
Format: Article
Language:English
Subjects:
Anaplasia
Cancer
Chemotherapy
Eye diseases
Medical prognosis
Nervous system
Pleomorphic xanthoastrocytoma
Retina
Retinal tumor
Retinoblastoma
Tumors
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Summary:To present a rare anaplastic form of retinal pleomorphic xanthoastrocytoma (PXA) unassociated with phakomatosis. 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. 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. Retinal PXA may occur in patients without phakomatosis and rarely progress toward malignant transformation.
ISSN:2452-2325
2452-2325
DOI:10.1016/j.joco.2018.09.005