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Spinal ependymomas in NF2: a surgical disease?
M Kalamarides1,2,3, W Essayed4, J P Lejeune5,6
1Department of Neurosurgery, Groupe Hospitalier Pitié -Salpêtrière, 47-83 boulevard de l'Hôpital, 75013, Paris, France. michel.kalamarides@aphp.fr.
Journal of Neuro-Oncology
|December 1, 2017
Summary
Conservative management of spinal ependymomas in Neurofibromatosis Type 2 (NF2) leads to deterioration in 27% of cases. Surgical intervention in selected NF2 patients, especially at specialist centers, shows improved outcomes compared to conservative approaches.
Area of Science:
- Neurosurgery
- Oncology
- Genetics
Background:
- Spinal ependymomas in Neurofibromatosis Type 2 (NF2) were traditionally managed conservatively due to assumptions of low morbidity.
- Modern advancements in NF2 tumor management necessitate re-evaluation of surgical roles for spinal ependymomas.
Purpose of the Study:
- To compare outcomes of conservative treatment versus surgical intervention for spinal ependymomas in NF2 patients.
- To assess the morbidity associated with spinal ependymomas in NF2 and the efficacy of different management strategies.
Main Methods:
- Retrospective review of 70 patients with spinal ependymomas (tumor length >1.5 cm) across two NF2 centers (Manchester, UK and Paris/Lille, France).
- Manchester center adopted conservative management, while the French center offered surgical intervention as an option.
- Primary outcome measure was acquired neurological deficit assessed by the Modified McCormick Outcome Score.
Main Results:
- Conservative management resulted in a 27% deterioration rate, reflecting the natural history of NF2 spinal ependymomas.
- Surgical intervention showed a 23% postoperative deterioration rate, with only 11% (2/18) deteriorating in specialist NF2 centers.
- A significantly improved outcome was observed in the center that actively pursued surgical intervention (P=0.012).
Conclusions:
- Spinal ependymomas in NF2 do cause significant morbidity.
- Surgery is a viable option for preventing or improving neurological deficits in selected, growing, or symptomatic NF2 spinal ependymomas.
- Surgical consideration is recommended, particularly when tumor burden is not overwhelming and bevacizumab is not the primary treatment.
