Suprasellar Primitive Neuroectodermal Tumor in an Adult

Primitive neuroectodermal tumors (PNET) of the central nervous system (CNS) are a heterogeneous group of embryonal malignancies that are composed of undifferentiated or poorly differentiated neuroepithelial cells. Supratentorial PNET is the second most common CNS embryonal malignancy in children, bu...

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Veröffentlicht in:Journal of neuro-ophthalmology 2016-09, Vol.36 (3), p.299-303
Hauptverfasser: Espino Barros Palau, Angelina, Khan, Khurrum, Morgan, Michael L, Powell, Suzanne Z, Lee, Andrew G
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Sprache:eng
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Zusammenfassung:Primitive neuroectodermal tumors (PNET) of the central nervous system (CNS) are a heterogeneous group of embryonal malignancies that are composed of undifferentiated or poorly differentiated neuroepithelial cells. Supratentorial PNET is the second most common CNS embryonal malignancy in children, but it is rare in adults. We report the case of a 31-year-old woman with bilateral vision loss and a bitemporal hemianopia. Neuroimaging revealed a suprasellar mass, and pathology was consistent with PNET. After surgical debulking of the tumor followed by radiation therapy and chemotherapy, the patient had significant visual recovery and remained stable over 14 months of follow-up.
ISSN:1070-8022
1536-5166
DOI:10.1097/WNO.0000000000000312