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- W2977697072 abstract "Pleomorphic Xanthoastrocytoma (PXA) describes a relatively rare and often focal glioma most commonly affecting children and young adults. Lesions are preferentially supratentorial and superficially located with a close association and occasional involvement of the leptomeninges, although infratentorial masses have been described. The World Health Organization (WHO) categorizes such tumors as Grade II, and surgical excision carries a favorable prognosis. Reportedly, PXA may be associated with Neurofibromatosis Type I (NF1), although such tumors are not a hallmark of the classic phenotype. In this report we describe a novel case of a pineal region PXA in a patient with a history of NF1. A 17-year-old male with a history of NF1 presented with complaints of bifrontal headache. Imaging of the head was ordered. Magnetic resonance imaging (MRI) was significant for a heterogeneous tectal mass suspicious for glioma extending to the level of the 4th ventricle with cerebral aqueduct displacement but no obstructive hydrocephalus. The patient underwent suboccipital craniotomy for an infratentorial supracerebellar approach to the pineal region and resection of tumor. Histopathology confirmed a low-grade variable neoplasm consistent with PXA. Post-operative imaging demonstrated gross total resection. There is no evidence of residual or recurrence at 3months follow-up. To the best of our knowledge, there are 8 prior reported cases of pineal region PXA with such lesions rarely occurring in individuals with NF1. These masses present similarly to dysplastic tumor types, and should be considered as a rare etiology of NF1 associated tectal masses." @default.
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- W2977697072 date "2016-11-01" @default.
- W2977697072 modified "2023-09-25" @default.
- W2977697072 title "RARE-54. FIRST CASE OF PLEOMORPHIC XANTHOASTROCYTOMA OF THE PINEAL REGION IN A PATIENT WITH NEUROFIBROMATOSIS TYPE I" @default.
- W2977697072 doi "https://doi.org/10.1093/neuonc/now212.713" @default.
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