/pineoblastoma

pineoblastoma

Rare Disease

MONDO:0016722

Also Known As

PNET of pineal glandPNET of the pineal glandpineal PNETpineal gland PNETpineal gland primitive neuroectodermal neoplasmpineal gland primitive neuroectodermal tumorpineal gland primitive neuroectodermal tumourpineal primitive neuroectodermal neoplasmpineal primitive neuroectodermal tumorpineal primitive neuroectodermal tumourpineoblastomapineoblastoma (WHO grade IV)pineoblastoma, malignantprimitive neuroectodermal neoplasm of pineal glandprimitive neuroectodermal neoplasm of the pineal glandprimitive neuroectodermal tumor of pineal glandprimitive neuroectodermal tumor of the pineal glandprimitive neuroectodermal tumour of pineal glandprimitive neuroectodermal tumour of the pineal gland

Definition

Pineoblastoma is a rare, malignant type of supratentorial primitive neuroectodermal tumor (sPNET), found mainly in children (less than 10% of cases are reported in adults), and located in the pineal region of the brain but that can metastasize along the neuroaxis. As it is the most aggressive of the pineal parenchymal tumors, it is usually associated with a poor prognosis.