Multifocal Paraganglioma Including the Cervical Sympathetic Chain.

Abstract

Multifocal head and neck paragangliomas are exceptionally rare. We present a patient with 4 synchronous tumors including a sympathetic chain lesion. This is a case report and literature review conducted from September 2021 to the present. Our 69-year-old male patient presented to the clinic with multifocal paraganglioma involving the right carotid body, right cervical sympathetic trunk, right glomus jugulare, and left glomus tympanicum. After preoperative embolization, successful surgical resection was performed for the right sympathetic trunk and right carotid body tumor. Postoperatively, the patient had a right Horner's syndrome but a strong voice and intact recurrent laryngeal nerve function. He also had first-bite syndrome and right cranial nerve XII weakness which resolved. Of interest, patient also has a strong family history of paragangliomas: his sister has malignant paragangliomas with lymph node involvement and his mother and nephew have non-malignant paragangliomas. To our knowledge, multifocal tumors including synchronous carotid body, sympathetic chain, glomus jugulare, and glomus tympanicum lesions occurring in 1 person have not been reported until now. As a result of this study, there is new light shed on the treatment and evaluation of patients who present with multifocal paraganglioma involving the sympathetic chain.