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.

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This study reviews the clinicoradiological features of cranial and sacrospinal chordomas and identifies factors affecting survival. Nineteen patients seen between January 1980 and December 2000 with histopathological diagnosis of chordomas were retrospectively reviewed with reference to clinical presentation, imaging features, treatment modalities and post-therapy status. Eight had tumours in the skull base while 11 patients had spinal and sacrococcygeal lesions. Surgical resection was performed in 16 patients whose subsequent natural history was used to identify clinical indicators that may influence survival. Completeness of resection, age, gender and postoperative irradiation were subjected to analysis using the Cox proportional hazard models. Kaplan-Meir survival curves illustrate the survival distributions. Diplopia and facial pain are prime clinical presentations in cranial lesions, while extremity weakness and a sacrogluteal mass are common complaints in the sacrospinal group. Lesional calcifications are present in 40% while an osteolytic soft tissue mass is detectable by CT in all cases. Heterogeneous signals and internal septations on T2-weighted MRI are predominant features. In sacrospinal tumours, complete excision with adjuvant radiotherapy achieves the best results with a disease-free survival of more than 5 years. The clinical and imaging findings in this study are in accordance with those of other series. Except for complete surgical excision followed by radiotherapy in the subset of patients with sacrospinal tumours, none of the other clinical indicators show a statistical significant influence on survival.