Tumors of the Cervical Sympathetic Chain - Diagnosis and Management

Figure 1. Anatomy of the Carotid Space. The sympathetic chain is located posteromedial to the great vessels and cranial nerves IX, X, XI, and XII. All of these structures are potentially at risk with growth or removal of sympathetic tumors. Objectives: Tumors originating from the cervical sympathetic chain are uncommon but important entities in the differential diagnosis of prestyloid parapharyngeal (carotid) space masses. We sought to evaluate the presentation of these tumors and the outcomes of surgical treatment. Study Design: Retrospective chart review Methods: We report our experience with 24 patients from 1994 to 2011. Clinic notes, operative and pathology reports, and radiologic images and reports were used to create the study database. Results: The most common presenting symptoms were dysphagia (29%, n=7), neck mass (n=7, 29%), and throat fullness (n=4, 17%). Two patients (8%) presented with Horner’s syndrome. Although radiologic images showed classic lateral displacement of the carotid arteries in 10 (42%), in 9 (38%) patients the radiologic findings demonstrated splaying of the carotid arteries similar to carotid body tumor and in 5 (20%) the findings were indeterminate. Three patients were observed, two due to small size and patient preference and one because of multiple bilateral cranial nerve involvement. Twenty-one patients underwent surgical removal, with pathology revealing 10 paragangliomas, 10 schwannomas, and 1 neurofibroma. Three patients (14%) had cranial nerve weaknesses (two vagal and one spinal accessory). Although most patients had some degree of Horner’s syndrome postoperatively, this was symptomatic in 12 (57%). With a mean follow-up of 20 months there have been no recurrences. Conclusions: This represents the largest original series of tumors of the sympathetic chain to date. Although anterolateral displacement of the carotids on imaging is suggestive of a sympathetic tumor, absence of these findings does not rule out this entity. Cervical sympathetic tumors can be safely managed with operative intervention with less than a 15% incidence of cranial nerve weakness.