The central neurocytoma was first recognized as a distinct entity in 1
982. The original description was of a low grade, intraventricular neo
plasm composed of uniform cells showing neuronal differentiation, and
having a very favorable prognosis after surgery. Subsequently rare cas
es have been described showing malignant histological features but ret
aining the morphological characteristics that justify inclusion in thi
s category. The behavior of such cases has yet to be determined. We re
port a case of a longstanding intraventricular tumor showing neuronal
differentiation in which repeated recurrences following surgery have r
evealed evidence of increasing nuclear pleomorphism, mitotic activity
and glial differentiation. We suggest that this tumor broadens further
the clinical and pathological spectrum of central neurocytoma.