Intraventricular pleomorphic xanthoastrocytoma: a case report

Abstract

Pleomorphic xanthoastrocytoma (PXA), an uncommon glial neoplasm, typically affects adolescents and young adults and accounts for less than 1% of all astrocytic neoplasms. The authors present a case of PXA located entirely in the third ventricle in a 24-year-old male patient. The patient presented with a 6-month history of headaches, progressive decline in cognitive function and profound behavioral disturbances. He was admitted to the hospital with signs of increased intracranial pressure. Magnetic resonance imaging showed a well-enhanced solid tumor, located entirely inside the third ventricle. The tumor was totally removed via a right fronto-pterional trans-lamina terminalis approach and neuropathology report confirmed PXA diagnosis. No further treatment was indicated. To the best of our knowledge, this is the first case report of a solid PXA confined to the third ventricle. KEywoRds: Pleomorphic xanthoastrocytoma, Intraventricular, Immuno-histochemical ÖZPleomorfik ksantoastrositom (PXA) nadir bir gliyal neoplazidir ve tipik olarak adolesanlar ve genç yetişkinlerde görülüp, tüm astrositik neoplazmların %1'inden azını oluşturur. Yazarlar 24 yaşında bir erkek hastada tamamen üçüncü ventrikülde bulunan PXA olgusu sunmaktadır. Hasta 6 aylık başağrısı, bilişsel işlevde giderek azalma ve belirgin davranış bozuklukları öyküsüyle geldi. Artmış intrakraniyal basınç bulgularıyla hastaneye yatırıldı. Manyetik rezonans görüntüleme tamamen üçüncü ventrikül içinde bulunan ve iyi kontrast tutan bir solid tümör gösterdi. Tümör sağ fronto-pterional trans-lamina terminalis yaklaşımı ile tamamen çıkarıldı ve nöropatoloji raporu PXA tanısını doğruladı. Başka bir tedavi gerekmedi. Bildiğimiz kadarıyla bu üçüncü ventrikülle sınırlı solid bir PXA için ilk olgu raporudur.

Intraventricular Pleomorphic Xanthoastrocytoma: A Case Report and Systemic Review

“…Infratentorial PXAs are rare, but cases in the cerebellum, spinal cord, and even the retina in children have been reported. Based on the up-to-date publications, there are 11 cases with confirmed image data for intraventricular PXAs, of which five were classified as grade 2 and six were classified as grade 3 (Table 1) [10][11][12][13][14][15][16][17][18][19]. In the present case, the tumor was located not only in the cerebral ventricles but also consisted of two masses, which is exceedingly rare for PXA.…”

Angiomatous pleomorphic xanthoastrocytoma: a case report and literature review

“…It usually develops in the superficial cortex, especially in the temporal lobes, and meningeal involvement is common [ 2 , 3 ]. Uncommon sites of PXA include the cerebellum [ 4 , 5 ], ventricle [ 6 , 7 ], spinal cord [ 8 , 9 ], sella [ 10 ], retina [ 9 , 11 ] and pineal gland [ 11 – 14 ]. Patients usually present with a prolonged history of seizure.…”