Central neurocytoma

Jai Grewal MD (Dr. Grewal of the Long Island Brain Tumor Center at Neurological Surgery, PC, received research funding from Northwest Biotherapeutics and consulting fees from Novocure.)
Harpreet K Grewal MD (Dr. Grewal of JGMDPC in Roslyn, New York, has no relevant financial relationships to disclose; Dr. Grewal's spouse received research funding from Northwest Biotherapeutics and consulting fees from Novocure.)
Edward J Dropcho MD, editor. (Dr. Dropcho of Indiana University Medical Center has no relevant financial relationships to disclose.)
Originally released March 5, 2002; last updated November 28, 2014; expires November 28, 2017

Overview

Central neurocytoma is a rare benign intraventricular tumor. It may be easily confused with other brain tumors such as oligodendroglioma and ependymoma. The author discusses the differential diagnosis, role of neuroimaging modalities, and long-term management of this neoplasm. In this article, imaging findings of rare extraventricular neurocytoma, including spinal cord tumor, are described. For this update, one reference is added about MRI findings of neurocytoma, which might help to differentiate it from other tumors preoperatively. Three other studies are referenced about treatment and prognosis of neurocytoma.

Key points

 

• Central neurocytoma is usually a benign intraventricular neuronal neoplasm with favorable prognosis.

 

• It often presents with signs of raised intracranial pressure or hydrocephalus.

 

• It may be confused with oligodendroglioma, ganglioglioma, and ependymoma.

 

• Surgical resection may be curative.

Historical note and terminology

Central neurocytoma is a rare primary brain neoplasm of neuronal differentiation that has close resemblance to oligodendroglioma and ependymoma. It is a discrete tumor composed of clusters of neurocytes that are small, round, mitotically inactive, uniform, and are dispersed on a fibrillar matrix (Brown et al 2001). The term "central neurocytoma" was coined by Hassoun and colleagues in 1982, who described it as a distinct, calcified, intraventricular tumor in 2 young patients. Central neurocytoma is so named because of its neuronal origin and midline location within the ventricular system. It usually occurs in young adults, in contrast to neuroblastoma, which is a more immature and malignant tumor predominantly affecting children (Hassoun et al 1982). The World Health Organization incorporated central neurocytoma as a separate entity in its new classification of brain tumors in 1993 after more than 125 cases were reported in literature (Kleihues et al 1993). It has been designated as grade I tumor in WHO classification; however, it has been suggested to label it as a grade II lesion if there is evidence for high proliferation index or histological atypia (Soylemezoglu et al 1997; Mackenzie 1999).

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