Extensive anaplastic multi-centric ependymoma in a young adult: case report and literature review

Abstract

Abstract Background Ependymoma is a rare adult tumor that originates from ependymal cells of the ventricles and the spinal cord. The diagnosis and management can be very challenging. This study aims to bring out the hypothalamus as an atypical location of ependymoma and to underline the consequences of treatment delay in anaplastic ependymomas through a concrete case. Case presentation We present a case of 20 years with no previous medical history, suffering from progressive intracranial hypertension syndrome, tetraparesis, cerebellar ataxia, and a weight loss of 3 kg in 4 months. Cerebral and medullar Magnetic Resonance Imaging showed multiple processes including the temporal region, the cerebellum, the 4th ventricular floor, the interpeduncular cistern, the hypothalamus, and almost the entire spinal cord, in addition to a posterior intradural lesion. The hypothalamic localization was very suggestive of neurohistiocytosis. A stereotactic biopsy and immunohistochemical study confirmed the diagnosis of anaplastic ependymoma. Total resection was impossible and radiotherapy was delayed by inconclusive dosimetric scans. The patient's outcome was unfortunately quickly fatal. Conclusions Ependymomas should be evoked whatever the location in the central nervous system. Radiotherapy must be quickly discussed and started when the gross total resection is impossible. The locations, as well as the doses of irradiation, are now well-established and any delay in initiating radiotherapy only darkens the prognosis.