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What is a central neurocytoma?

Central neurocytoma is a tumor of young adults composed of uniform round cells with neuronal differentiation, typically occurring in the lateral ventricles in the region of the foramen of Monro. Central neurocytoma corresponds to WHO Grade II.  

Who is affected by central neurocytoma tumors?

Central neurocytoma represents less than 1% of all intracranial tumors, with a peak in young adults between 20 and 40 years of age. Both sexes are affected equally.

Where in the brain are central neurocytomas typically located?

Central neurocytoma is most commonly found in the anterior portion of the lateral ventricle (50%), followed by a combined presentation in the lateral and 3rd ventricles (15%), or biventricular location (13%). Only 3% of cases occur in the 3rd ventricle alone.

What are the typical symptoms of a patient with central neurocytoma? 

Patients with central neurocytoma typically present with signs and symptoms of increased intracranial pressure. These may include headache, or decreased responsiveness or even coma.  

What do central neurocytomas look like on MRI scans? 

Typically these tumors show enhancement.  Angiography may reveal a homogeneous vascular blush, persisting late in to the venous phase.

How does these tumors appear under the view of a surgeon who is operating?

These intraventriciular tumors are usually grayish and friable, with variable degrees of calcification and intratumoral hemorrhage.

How do these tumors appear under the microscope?

These tumors have uniform round cells with neuronal differentiation, with adjacent fibrillary areas mimicking neuropil. Synaptophysin is the most reliable diagnostic marker. The low proliferation rate is generally low. In rare cases, anaplastic histological features may be seen.

Are there specific genes that are altered that make up central neurocytomas?

The molecular genetics of central neurocytoma have not been particularly elucidated.

What is the typical prognosis if a person is diagnosed with a central neurocytoma?

Usually these tumors have a relatively benign clinical course.  An excellent treatment for tumor growh control is surgical removal of the tumor.  The management of patients with subtotal resection remains unclear, though it appears that these patients have shorter local control and survival rates. Interestingly, aggressive histological appearance does not seem to predict poor clinical outcome.

 

Images of a central neurocytoma before complete removal.