Background:
Extraventricular neurocytoma (EVN) is a rare variant of central neurocytoma which arises outside of the ventricular system. Diffuse ganglioid differentiation is a characteristic seen in a subset of these tumors which has an uncertain prognostic significance. Typically, EVN presents in children and young adults. Given the rarity of this tumor, the natural history and response to treatments remain unclear.
Case description:
We present a case of EVN with diffuse ganglioid differentiation in a 70-year-old male which arose in the midline parasellar region and extended into the third ventricle. This is the oldest such patient reported. Despite prior reports that extremes of age are associated with more aggressive behavior, the tumor in this case did not exhibit such an aggressive course.
Conclusion:
In this report, we review the natural history and clinical course of this patient and summarize the literature regarding this rare pathological entity. Our patient responded well to therapy despite older age, ganglioid differentiation, and higher mitotic index.