Surgical management of schwannomas in schwannomatosis: a comprehensive analysis of clinical outcomes and determinants of local recurrence.

OBJECTIVE

Schwannomatosis is a rare genetic predisposition to develop multiple nonintradermal schwannomas affecting the spine and peripheral nerves, excluding vestibular schwannomas. Schwannomatosis-associated schwannomas can present with pain or motor deficits, prompting resection. In this study, the authors reported the clinical characteristics, surgical interventions, and postoperative outcomes of patients with schwannomatosis-related schwannomas treated with resection.

METHODS

This was a retrospective cohort study of adult patients with schwannomatosis who had undergone resection of a symptomatic peripheral or spinal schwannoma at the authors' institution from September 2003 to June 2023. Data were extracted from electronic health records and provider notes.

RESULTS

A cohort of 85 patients (57.6% women) had a mean follow-up of 68.4 ± 58.2 months after surgery. The mean age at the time of surgery was 44.6 ± 16.5 years. Pain was the most common presenting symptom (94.1%), followed by motor weakness (36.4%) and sensory deficits (32.9%). Just under two-thirds of the patients (64.7%) had undergone prior surgery for schwannoma, 20 (36.4%) of whom had a prior resection in the same exact location. Gross-total resection (GTR) was achieved in 88.2% of cases and en bloc resection in 69.4%. There were significant improvements in pain, motor weakness, and sensory deficits at the last postoperative follow-up visit. There was local tumor recurrence in 14 patients (16.5%), and the overall mortality rate was 4.7%. Univariate and multivariate analyses identified GTR as the strongest predictor of a decreased risk of local recurrence (HR 0.09, 95% CI 0.02-0.48, p = 0.005).

CONCLUSIONS

Patients with schwannomatosis commonly presented with neurological symptoms of pain, sensory deficits, or motor weakness associated with their schwannomas. Resection significantly improved their preoperative symptoms, and GTR was associated with a decreased risk of local recurrence.