Central neurocytoma: clinical, immunohistologic, and biologic findings of a human neuroglial progenitor tumor

Abstract

Background: Central neurocytomas are rare brain tumors recognized by their typical radiologic and histologic features. In general, a good prognosis is achieved by total removal. The histogenesis is still under debate, but a neuronal origin is widely assumed.

Methods: This study presents the clinical and immunohistologic findings of five patients and the results of cell culture experiments of two patients with central neurocytoma treated surgically between 1983 and 1993.

Results: The patient age at diagnosis ranged from 21 to 30 years (mean, 25 years). The male-to-female ration was 1:4. Raised intracranial pressure due to hydrocephalus was the main cause of the clinical manifestations. Total resection was achieved in two cases. Four patients received radiotherapy. One patient suffered a recurrence 1 year after surgery, requiring a second resection and radiotherapy. Follow-up studies took place between 1 and 10.5 years (mean, 7.1 years). To date, all patients are free of their tumors. Two patients suffered from permanent memory disturbances after surgery. Immunohistochemistry confirmed the neuronal nature of the tumors. Cell-culture studies, which have been carried out for the first time, demonstrated concomitant expression of neuronal (synaptophysin) and glial (GFAP) markers.

Conclusion: Total removal is the therapy of choice. In tumor recurrence or limited surgery (e.g. due to severe affliction of the fornical structures), radiotherapy has shown to be efficacious. The cell-culture experiments give new insight on the histogenesis of central neurocytoma, indicating that the tumor arises from an undifferentiated precursor cell with the capacity of bipotential neuroglial differentiation.