[Metastasis of a lumbosacral ependymoma with very long disease-free survival: a case report and review of the literature]

Abstract

Ependymoma is rare glial tumour of the central nervous system and is considered to be low-grade. The lumbosacral location of spinal ependymoma is the most common. Prognosis of ependymomas is dependent on tumour location, histological subtype and differentiation, extent of the tumour and of the completeness of the surgical resection. One of the characteristics of this kind of tumour is to present the possibility of a seeding of the entire cerebrospinal axis, by the way of cerebrospinal liquid. We describe the case of a young male patient operated by incomplete resection of a lumbar ependymoma. Six months later, the patient's symptoms reappeared and an external radiotherapy at curative doses and chemotherapy were delivered. Evolution of the remaining tumour was diagnosed 6 years after at the primary site and operated by large incomplete resection. A second session of radiotherapy was therefore administered. Twenty-four years after this episode, cervical pain and gait troubles appear. Complete imaging study concluded to a cervical extramedullary intradural tumour and to the persistence of the primary lumbosacral tumour. Macroscopical complete resection of the cervical tumour was performed and pathological findings concluded to a metastasis of his lumbar ependymoma. External radiotherapy was delivered on this site with a total dose of 50 Gy. Eight years after this episode, the patient is alive without evidence of distant disease. The primary lumbosacral ependymoma is stable. Ependymomas are often recurrent at the primary site, but can seed on the entire cerebrospinal axis. Awareness of such aberrant tumoral behaviour, even after such a long disease free interval, may warrant more careful follow-up of patients with this diagnosis.