Abstract
Long-term outcomes for pediatric patients treated for spinal ependymoma are unknown. 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). 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. 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.
Highlights
The spinal canal is a more common location in adults, followed by a supratentorial presentation, whereas posterior fossa ependymomas are more uncommon in adults.[7]
We retrospectively analyzed a non-selected, consecutive cohort consisting of children and adolescents who underwent primary resection of a spinal ependymoma during the period from 1938 to 2019 at the Department of Neurosurgery, Oslo University Hospital, Oslo, Norway
In recent years immunohistochemistry and molecular markers have been the focus in efforts to understand the biology of these tumors
Summary
Ependymoma is the third most common central nervous system tumor in children, after low-grade astrocytoma and medulloblastoma.[1,2,3,4] Supratentorial ependymomas are less common than posterior fossa presentation in the pediatric age group, but a spinal presentation is exceedingly rare.[5,6] Opposed to this, the spinal canal is a more common location in adults, followed by a supratentorial presentation, whereas posterior fossa ependymomas are more uncommon in adults.[7] In 1977, Mork and Loken presented the results in 101 ependymoma cases of all ages operated between 1953 and 1974 at our institution.[8] Out of 53 spinal cases, there were 6 children (0e19 years old) They pointed out that all patients treated for myxopapillary spinal ependymomas were alive, and seemed to have a good prognosis. Long-term outcomes for pediatric patients treated for spinal ependymoma are unknown
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