Outcome after treatment of spinal ependymoma in children and adolescents: long-term follow-up of a single consecutive institutional series of 33 patients treated over the period of eight decades.

Abstract Methods We performed a retrospective analysis of outcome data from 33 children and young adults (0-22 years) who were operated on for a spinal ependymoma at our institution during the last eight decades (1938-2019). Findings. Nineteen patients are alive with follow-up period up to 60 years. Twelve of them are tumor-free, while seven are alive with disease. Fourteen patients are dead, nine of them due to recurrent and/or progressive disease 1-56 years (median 11 years) after the initial surgery. Four of the deceased patients were treated before 1948, three of them with excellent long-term survival for 62-66 years after the initial surgery.Tumor recurrence was observed in half of the patients, both local at the site of the primary tumor resection as well as widespread intraspinal presentations. Recurrences were observed within months but also occurred after up to 20 years after initial treatment. After the implementation of MRI in 1987, details of recurrent disease became more easily demonstrated. Repeated resections were performed when the symptomatic spinal disease was in progress (n=11). Furthermore, two patients have intracranial tumor manifestations, one of them underwent resection of a suprasellar tumor in 1991. Four deceased patients experienced aggressive extraspinal progressive disease requiring multiple surgeries, including pulmonary metastasis in one of them. Conclusion Pediatric spinal ependymomas can be treated with favorable results and functional outcome may be good even after more than half a century of follow-up. However, unexpected and late recurrences may occur, and life-long follow-up is therefore recommended.