Author/Authors :
Aliakbar Navahi Roshanak Eye Research Center - Rassoul Akram Hospital - Iran University of Medical Sciences - Tehran, Iran , Sedaghat Ahad Eye Research Center - Rassoul Akram Hospital - Iran University of Medical Sciences - Tehran, Iran , Naseripour Masood Eye Research Center - Rassoul Akram Hospital - Iran University of Medical Sciences - Tehran, Iran , Aghai Gholamhoseyn Eye Research Center - Rassoul Akram Hospital - Iran University of Medical Sciences - Tehran, Iran , Babaheidarian Pegah Pathology Department - Rassoul Akram Hospital - Iran University of Medical Sciences - Tehran, Iran , Mardani Azam Iran University of Medical Sciences - Tehran, Iran , Ahangarani Mohammadreza Iran University of Medical Sciences - Tehran, Iran
Abstract :
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
