Bilateral carotid body and cervical sympathetic chain paragangliomas: A case report and review of the literature

Abstract

Background: Paragangliomas are rare neuroendocrine tumors which develop at the site of extra-adrenal paraganglia of the autonomic nervous system, originating in the neural crest. Case presentation: We report the extremely rare finding of 4 concurrent paragangliomas arising bilaterally and simultaneously involving the carotid bodies and superior cervical ganglia of the sympathetic trunk in a 53-year-old man. These tumors were large enough to cause unilateral sympathetic chain dysfunction resulting in Horner's syndrome, in addition to oropharyngeal compression causing obstructive sleep apnea. The patient's clinical course, surgical treatment, and management considerations are discussed here. Conclusion: The morbidity associated with Horner's syndrome is relatively low; therefore, resection of these tumors is less controversial. However, the surgical access to large masses in the parapharyngeal space is more difficult and, in some cases, requires mandibulotomy to ensure that critical neurovascular structures are preserved during the resection. Keywords: Paraganglioma, Parapharyngeal space, Cervical sympathetic chain, Carotid body tumor, Horner's syndrome