Congenital cerebral primitive neuroectodermal tumor with astrocytic differentiation and extracranial metastases

A cerebral primitive neuroectodermal tumor with astrocytic differentiation and extracranial metastases in a 28-day-old infant is reported. The infant presented with a progressively enlarged head, cutaneous lesions in the neck, and enlarged cervical lymph nodes. A computed tomography brain scan demonstrated a giant thalamic tumor with subarachnoid dissemination and hydrocephalus. Biopsy material from the cervical lesions showed a picture of glioma with anaplastic astrocytes. The patient received a ventriculoperitoneal shunt operation and palliative chemotherapy, but died at 3 months of age. Autopsy was performed. Histological studies, which included immunohistochemical stains of the thalamic tumor, showed small, round, primitive, neoplastic cells with focal astrocytic differentiation.