CENTRAL NEUROCYTOMA - CLINICAL, IMMUNOHISTOLOGIC, AND BIOLOGIC FINDINGS OF A HUMAN NEUROGLIAL PROGENITOR TUMOR

BACKGROUND Central neurocytomas are rare brain tumors recognized by th eir typical radiologic and histologic features. In general, a good pro gnosis is achieved by total removal. The histogenesis is still under d ebate, but a neuronal origin is widely assumed. METHODS This study pre sents the clinical and immunohistologic findings of five patients and the results of cell culture experiments of two patients with central n eurocytoma treated surgically between 1983 and 1993. RESULTS The patie nt age at diagnosis ranged from 21 to 30 years (mean, 25 years). The m ale-to-female ratio was 1:4. Raised intracranial pressure due to hydro cephalus was the main cause-of the clinical manifestations. Total rese ction was achieved in two cases. Four patients received radiotherapy. One patient suffered a recurrence 1 year after surgery, requiring a se cond 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 (synaptoph ysin) and glial (GFAP) markers. CONCLUSION Total removal is the therap y of choice. In tumor recurrence or limited surgery (e.g., due to seve re affliction of the fornical structures), radiotherapy has shown to b e efficacious. The cell-culture experiments give new in sight on the h istogenesis of central neurocytoma, indicating that the tumor arises f rom an undifferentiated precursor cell with the capacity of bipotentia l neuroglial differentiation.