Article
Management and outcome of operated NF2 associated spinal ependymomas
Management und Outcome von operierten NF-2 assoziierten spinalen Ependymomen
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Published: | June 4, 2021 |
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Objective: To evaluate the clinical effects of surgery for symptomatic and/or progressive Neurofibromatosis Type 2 (NF2) associated spinal ependymomas.
Methods: We retrospectively reviewed clinical reports and T1- and T2-weighted magnetic resonance images in 180 patients with NF2. 109/180 patients (61%) did not bear spinal ependymomas, 7 operated cases were excluded due to insufficient data.
Neurological function before and after surgery was classified using an established scoring system including the 5 categories sensibility (incorporating pain), motor, bladder/bowel and gait function. Preservation of neurological function was determined as maintenance of preoperative scoring class.
Results: 13/64 patients with spinal ependymomas (20%) required surgery either due to rapid growth (2/13, 15%), worsening of neurological function (8/13, 62%) or combination of both (3/13, 23%).
The most common symptoms leading to surgery were gait disturbances (7/13, 54%), followed by sensory and motor deficits and pain (4/13, 31%) and bladder and bowl impairments (3/13, 23%). 10/13 patients exhibit two or more symptoms before surgery.
Within the first 24-months of follow-up after surgery, the clinical status was stable in 6/13 (46%), improved in 3/13 (23%) and worsened in 4/13 (31%).
Conclusion: 40% of NF2 patients harbour spinal ependymomas and the majority of those is asymptomatic and does neither require treatment nor biopsy for securing the diagnosis, as these tumours totally behave different compared to sporadic forms. In cases with developing neurological symptoms mostly combined with rapid tumour progression, surgical intervention appears to be a promising and low-risk approach in experienced hands to maintain or improve the neurological function in about 70% of cases.