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Primitive Neuroectodermal Tumors of the Central Nervous System

Background

Primitive neuroectodermal tumors (PNET) are neoplasms of which medulloblastoma is the prototype. These are small cell, malignant embryonal tumors showing divergent differentiation of variable degree along neuronal, glial, or rarely mesenchymal lines.

The most recent WHO categorization of embryonal tumors is as follows:

Medulloblastoma

CNS primitive neuroectodermal tumor (PNET)

CNS neuroblastoma

CNS ganglioneuroblastoma

Medulloepithelioma

Ependymoblastoma

Atypical teratoid/rhabdoid tumor

Only tumors of the CNS are discussed here. Peripheral primitive neuroectodermal tumors are regarded as distinct entities.

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