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 Eight Decades

Abstract

Background: Long-term outcomes for pediatric patients treated for spinal ependymoma are unknown.

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 8 decades (1938-2019).

Results: Nineteen patients are alive, with follow-up period up to 60 years. Twelve of them are tumor-free, and 7 are alive with disease. Fourteen patients are dead, 9 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, 3 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 magnetic resonance imaging 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, 2 patients have intracranial tumor manifestations, 1 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 1 of them.

Conclusions: 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. Nevertheless, unexpected and late recurrences may occur, and life-long follow-up is therefore recommended.

Keywords: Adult outcome; Pediatric spinal ependymoma.