Disparities in the diagnosis, treatment and outcomes of a benign nerve sheath tumor: Implications for practice from an international systematic review

Objectives:To systematically evaluate the literature to determine global differences in the diagnosis, treatment and outcomes of Cervical Sympathetic Chain Schwannomas (CSCS), a benign nerve sheath tumor.

Methods: Using Medline, EMBASE, and Cochrane databases, 89 CSCS case reports/series were identified from 1997 to 2013.  Most cases were treated internationally (82%), predominantly in Asia (50%) and Europe (27%).  Data on diagnosis and treatment approach relative to outcomes were extracted by independent reviewers with high inter-rater reliability (κ=.79).  

Results:  On average, patients were 42.6 years old (SD=13.3) and had a 2 - 4 cm (52.7%) or >4 cm (43.2%) neck tumor.  Nearly 70% of cases were asymptomatic.  Pre-operative diagnosis relied on CT (63.4%), MRI (59.8%) or both (20%), supplemented by cytology (47.6%).  U.S.-treated cases were significantly more likely to receive MRI than internationally-treated cases, but less likely to have cytology/histopathology (p<0.05).  Pre-operative diagnosis accuracy was 11% as confirmed by post-operative histopathology.  Complete resection of tumor involving nerve sacrifice vs. preservation was the most common treatment (87.6%), irrespective of tumor size and treatment country.  Adverse events (AEs) including Horner’s syndrome (89.9%), First Bite syndrome (21.1%) or both (15.7%), were significantly more prevalent with >4 cm tumor size and complete resection (p<0.05), persisting in 81.8% of cases at follow-up (mean: 28.8 months, SD:27.1).

Conclusions: Globally, variations in diagnostic approach and treatment impacted patient outcomes.  Given the typical CSCS patient is young and asymptomatic and the likelihood of persistent morbidity is high with aggressive surgery, less invasive approaches to differentially manage CSCS are warranted.