Background:Central neurocytoma is an uncommon benign tumor of the central nervous system. A section of these tumors have unusual aggressiveness and are termed as “atypical central neurocytomas,” the definition of which is debated. Many studies in the available literature define them as tumors with elevated MIB-1 labeling index (MIB-1 LI) >2%, while some associate them with higher values of MIB-1 LI or those with histological atypical features. Newer parameters also have been identified and correlated with MIB-1 LI to differentiate atypical from benign neurocytoma cases. A recent analysis of the atypical neurocytoma cases with malignant behavior revealed their increased tendency of spread through the cerebrospinal fluid causing craniospinal axis dissemination. However, limited studies document the appropriate indications and usefulness of additional therapeutic modalities, such as upfront craniospinal irradiation (CSI) or adjuvant chemotherapy, in countering the aggressive behavior of such tumors.
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