Clinical characteristics and long-term surgical outcome of spinal myxopapillary ependymoma: a French cohort of 101 patients.

Anne-Sophie Montero,Félix Berriat,Suzanne Tran,Clovis Adam,Bertrand Mathon,Fabrice Parker,Ahmed Idbaih,Alexandre Carpentier,Chiara Villa,Stephan Gaillard,Loïc Feuvret,Marc Polivka,Franck Bielle,Aymeric Amelot,Guillaume Lot

doi:10.1007/s11060-021-03717-7

Abstract

Myxopapillary ependymoma (MPE) is the most frequent tumor affecting the medullary conus. The surgical therapeutic management is still debated and only few studies have focused on the postoperative clinical outcome of patients. This study aimed to demonstrate long-term postoperative outcome and to assess the predictive factors of recurrence as well as the clinical evolution of these patients. From 1984 to 2019, in four French centers, 101 adult patients diagnosed with MPE were retrospectively included. Median age at surgery was 39years. Median tumor size was 50mm and lesions were multifocal in 13% of patients. All patients benefited from surgery and one patient received postoperative radiotherapy. Gross total resection was obtained in 75% of cases. Sixteen percent of patients presented recurrence after a median follow-up of 70months. Progression free survival at 5 and 10years were respectively estimated at 83% and 79%. After multivariable analysis, sacral localization, and subtotal resection were shown to be independently associated with tumor recurrence. 85% of the patients had a favorable evolution concerning pain. 12% of the patients presented a postoperative deterioration of sphincter function and 4% of motor function. Surgery alone is an acceptable option for MPE patients. Patients with sacral location or incomplete resection are at high risk of recurrence and should be carefully monitored.

Highlights

Myxopapillary ependymoma (MPE) is a rare tumor, with an estimated incidence of 1 out of one million inhabitants [1]
Surgery alone is an acceptable option for MPE patients
Patients with sacral location or incomplete resection are at high risk of recurrence and should be carefully monitored

Summary

Introduction

Myxopapillary ependymoma (MPE) is a rare tumor, with an estimated incidence of 1 out of one million inhabitants [1]. The 2006 WHO classification assigned to MPE a grade 1, but the grade will be changed to grade 2 in the classification [2, 3]. MPE occurred almost exclusively within the conus and the filum terminale [3]. MPE is the most frequent tumor of the conus medularis [3, 4]. The natural course of MPE remains largely unknown. Some articles on untreated MPE report an evolution towards tumor growth and neurological degradation within a few years [5, 6]. The average duration of symptoms preceding diagnosis ranges from 13 months to 8.3 years since they are slow growing [6,7,8].Most patients present a long history of non-specific symptoms such as low back pain with or without motor, sphincter or sensory signs [9]

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