Artikel
Management and outcome of NF2- and Schwannomatosis-associated non-Intracranial Schwannomas – influence of surgery, genetics, and localisation
Management und Outcome von NF2- und Schwannomatose-assoziierten nicht-intrakraniellen Schwannomen: Einfluss der Chirurgie, Genetik und Lokalisation
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Veröffentlicht: | 25. Mai 2022 |
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Gliederung
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Objective: To evaluate the neurological outcome after resection of peripheral non-intracranial schwannomas in Neurofibromatosis Type 2 (NF2) compared to Schwannomatosis (SWNT) patients.
Methods: We retrospectively reviewed the clinical and functional outcome (motor, sensory, pain) of 205 operated PNS (133 NF2 and 72 SWNT related tumors) in 85 patients (53 NF2 and 32 SWNT patients). Possible influencing factors (e. g. genetics) were also evaluated.
Results: Patients with NF2 were significantly younger (p < 0.001, median 25±11 years in NF2 and 45±1 years in SWNT) at the time of surgery. In both entities, total resection could be achieved for the majority of cases (NF2: n=109, ≙82%; SWNT: n=67, ≙93%), and persisting drug-resistant pain was the most common symptom (84%, n=173) as well as an indication for surgery (54%, n=110). Improvement of pain intensity was seen in 81% of all tumors. In 41 (18 NF2 and 23 SWNT) patients with available genetics, mosaic NF2 cases showed worse pain intensity scores (p=0.017) and SWNT cases with causative mutations poorer pre-and postoperative motor/sensory function and pain intensity outcome (p<0.05). In the direct comparison, NF2-associated PNS had more sensory impairment (p=0.027) whereas SWNT cases suffered from higher pain intensity (p=0.024) before surgery.
Conclusion: With careful patient selection and in experienced Center, surgery of PNS is an effective and low-risk treatment in both NF2 and SWNT associated cases. Patients with severe pain have a particular profit. Mutations seem to play an important role particularly for SWNT related tumors regarding the functional outcome and this possible influence needs to be further investigated in larger cohorts.