Diagnostic Approach,Treatment,and Outcomes of Cervical Sympathetic Chain Schwannomas

Abstract

Objective

This review examined the diagnostic approach, surgical treatment, and outcomes of cervical sympathetic chain schwannomas (CSCS) to guide clinical decision making.

Data Sources

Medline, EMBASE, and Cochrane databases.

Review Methods

A literature review from 1998 to 2013 identified 156 articles of which 51 representing 89 CSCS cases were evaluated in detail. Demographic, clinical, and outcomes data were extracted by 2 independent reviewers with high interrater reliability (κ = .79). Cases were mostly international (82%), predominantly from Asia (50%) and Europe (27%).

Conclusions

On average, patients were 42.6 years old (SD = 13.3) and had a neck mass ranging between 2 to 4 cm (52.7%) or >4 cm (43.2%). Nearly 70% of cases were asymptomatic at presentation. Presurgical diagnosis relied on CT (63.4%), MRI (59.8%), or both (19.5%), supplemented by cytology (33.7%), which was nearly always inconclusive (96.7%). US-treated cases were significantly more likely to receive presurgical MRI than internationally treated cases but less likely to have cytology (P < .05). Presurgical diagnosis was challenging, with only 11% confirmatory accuracy postsurgically. Irrespective of mass size, extracapsular resection (ie, complete resection with nerve sacrifice) was the most frequently (87.6%) performed surgical procedure. Common postsurgical adverse events included Horner’s syndrome (91.1%), first bite syndrome (21.1%), or both (15.7%), with higher prevalence when mass size was >4 cm. Adverse events persisted in 82.3% of cases at an average 30.0 months (SD = 30.1) follow-up time.

Implications for Practice

Given the typical CSCS patient is young and asymptomatic and the likelihood of persistent morbidity is high with standard surgical approaches, less invasive treatment options warrant consideration.