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Retinal anaplastic pleomorphic xanthoastrocytoma unassociated with phakomatosis
Roshanak Aliakbar Navahi1, Pegah Babaheidarian2, Ahad Sedaghat1
1Eye Research Center, Rassoul Akram Hospital, Iran University of Medical Sciences, Tehran, Iran.
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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