Primitive neuroectodermal tumors of the brain in children

TLDR

Primitive neuroectodermal tumors are predominantly undifferentiated neoplasms arising in the cerebrum of young individuals. The study examined 23 cases of PNETs that shared common clinical and pathological features. The tumors were grossly cystic and compressive, microscopically malignant with prominent mesenchymal components, occasional glial/neuronal differentiation, and an aggressive course averaging 18 months from symptom onset to death.

Abstract

Predominantly undifferentiated tumors occurring in the cerebrum of young individuals are referred to as "primitive neuroectodermal tumors." Twenty-three of these cases were collected which had certain common features. Grossly they tended to be cystic and compressed adjacent brain. Microscopically they were malignant-appearing and demonstrated a prominent mesenchymal component. Sixteen cases showed focal evidence of glial and/or neuronal-appearing differentiation. It is suggested that the mesenchyme is also an aspect of tumor differentiation. The biologic behavior of these tumors was malignant, with an average duration of 18 months from onset of symptoms to death.