Central neurocytoma (CNC) is an extremely rare, ordinarily benign intraventricular brain tumour that typically forms from the neuronal cells of the septum pellucidum.
Treatment for a central neurocytoma typically involves surgical removal, with an approximate 1 in 5 chance of recurrence.
The tumor is composed of “uniform, small-to-medium-sized cells with rounded nuclei, finely stippled chromatin and inconspicuous nucleoli, along with scant cytoplasm.” CNC are characterized by perivascular pseudorosettes, circular/flower-like arrangements of cells with a small blood vessel at the centre, and polygonal small cells with a clear perinuclear halo, sometimes called the ‘fried egg’ appearance, and is clear or slightly eosinophilic.
[7] Two adjuvant therapeutic strategies are Stereotactic surgery (SRS) and fractionated convention radiotherapy (FCRT).
Gamma knife surgery is incredibly effective at treating neurocytoma and maintaining tumor control after the procedure when a complete excision has been performed.
[5] The majority of patients can be expected to be cured of their disease and become long-term survivors of central neurocytoma.
[11] Central neurocytomas are rare brain tumors that are located most of the times in the lateral ventricles near the Monro foramina.
[12] In 1985, Wilson had also described a rare case of "differentiated neuroblastoma" in the lateral ventricle that resembles oligodendroglioma on light microscopy.
Most cases described were of non-neuronal origin such as oligodendroglioma, ependymoma, meningioma, choroid plexus papilloma and giant cell.
Neurocytomas were probably historically misdiagnosed as intraventricular oligondedronglioma or clear cell ependymoma prior to this.
Many recent studies suggest that their location, biological potential and clinical behavior are observed be more variable than previously thought.
Recent studies indicate their uncommon location, aggressive biological behavior and frequent recurrences following after surgical resection have generated significant interest in various treatment modalities and also in their terminology, lineage potential and molecular regulation.
[15] Central neurocytomas predominantly form in young adults, most commonly during the second or third decade of life.