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Benign central neurocytoma (CN) is a rare neuronal tumour of the central nervous system recognised since the early eighties. More than 300 articles have been published in the literature, mostly comprising of case reports and short series from individual specialties. These tumours, though normally benign, are more often likely to recur after surgery than previously thought. A multi-modality team involvement, therefore, has become increasingly necessary for their optimum management. In this article, the authors from various neurosciences sub-specialties, with a specific interest and experience in managing CN, review the epidemiology, clinical features, pathological findings, radiological characteristics and surgical treatment, with an emphasis on the latest developments in their histology, molecular biology and adjuvant treatment modalities for recurrent or residual disease.

A cerebellar tumour, first diagnosed as an anaplastic oligodendroglioma, received radiation therapy (45 Gy) following gross total resection. The second histological study revealed a liponeurocytoma, a benign tumour not requiring adjuvant therapy. This case emphasizes the importance of considering this diagnosis to prevent unnecessary irradiation of such rumours.

Total excision is usually the recommended treatment for central neurocytomas. The role of radiation therapy in their management is controversial, and is usually reserved for partially resected or recurrent tumours. Long-term outcome was studied in patients with central neurocytoma who were treated with radiation therapy. By retrospective chart review, eight patients with intraventricular tumours, who had undergone radiation therapy following stereotactic biopsy, were identified. The diagnosis of neurocytoma was confirmed based on positive immunostaining with synaptophysin. Clinical and radiological follow-up was obtained. Of eight patients, one died 5 years after treatment from shunt dysfunction and 1 had disseminated intracranial disease 15 months after treatment. Six patients were symptom-free at a mean follow-up period of 78 months and had good local control as demonstrated by CT. Since this tumour can be confused with an oligodendroglioma or an ependymoma on routine histopathology (as happened in seven of our cases); immunohistochemical studies with synaptophysin should be routinely performed for intraventricular glial tumours. Our study supports the beneficial effect of radiation therapy in the management of these tumours.

Extra-ventricular neurocytoma (EVN) is rare, mainly described within the cerebral hemispheres or spinal cord. We report a case of sellar neurocytoma presenting with chiasmal compression.A 50-year-old woman presented with decreasing vision and a bitemporal field deficit. CT and MRI demonstrated an enhancing invasive sellar lesion extending into the sphenoid sinus and left cavernous sinus, with speckled calcification and compression of the optic chiasm. Pituitary function was normal apart from a mildly elevated prolactin level consistent with stalk effect.Endoscopic trans-sphenoidal resection of the lesion was performed revealing a firm, vascular tumour. Some residual tumour was left within the left cavernous sinus. Histology revealed a neurocytic tumour with nests, islands and strands of neuropil. Diffuse synaptophysin, chromogranin, neurofilament protein and CD56 positivity was seen. A histological diagnosis of EVN, WHO grade II was made and adjuvant radiotherapy was given for the residual tumour.This is only the second case of neurocytoma of the sella reported. It should be considered in the differential diagnoses of sellar lesions that appear radiologically atypical for pituitary adenomas.

Central neurocytomas (CNs) are characterized by an indolent growth and a low recurrence rate. Cell proliferation has been rarely studied in recurrent CNs despite the growing awareness of its prognostic value. We present a CN that recurred rapidly after a prolonged relapse-free interval showing a 4-fold increase of MIB-1 labelling index.

A 52-year-old woman displayed a brain-MRI depicting a left lateral ventricle mass, associated with cystic component, extending controlaterally. Histological examination revealed neuronal differentiation with neurocytes and small ganglionic cells and the tumour was graded as a ganglioneurocytoma. We report the first case of a cystic ganglioneurocytoma with an intraventricular location.

Central neurocytoma is a rare benign tumour of neuronal origin that has only recently been described. The majority of previously reported cases have originated from the septum pellucidum and the walls of the lateral ventricles. We describe a case of central neurocytoma arising from the thalamus. This site of origin has not been previously described. A review of the literature and discussion of the management follow.

Neurocytoma has only recently been recognized as a distinct pathological entity. Previously a rare tumor, it is now being recognized with increasing frequency. It typically presents in an intraventricular location in young adults. Many neurocytomas were originally diagnosed by light microscopy as oligodendrogliomas. More recently they have been shown to have ultrastructural and immunohistochemical features of neuronal differentiation. This paper presents two cases of intraventricular neurocytomas and reviews the recent literature.