Multifocal Paraganglioma Including the Cervical Sympathetic Chain

Area of Science:

• Endocrinology
• Neurosurgery
• Oncology

Background:

• Multifocal head and neck paragangliomas are rare, posing diagnostic and therapeutic challenges.
• Paragangliomas can arise from various locations, including the carotid body, sympathetic chain, glomus jugulare, and glomus tympanicum.
• A strong family history of paragangliomas suggests a potential genetic predisposition.

Purpose of the Study:

• To report a unique case of synchronous multifocal paragangliomas involving the head, neck, and sympathetic chain.
• To review the literature on multifocal paragangliomas, particularly those involving the sympathetic chain.
• To shed light on the evaluation and treatment of patients with complex multifocal paraganglioma presentations.

Main Methods:

• Case report of a 69-year-old male patient with four synchronous paragangliomas.
• Literature review of multifocal head and neck paragangliomas.
• Preoperative embolization followed by surgical resection of specific tumors.

Main Results:

• The patient presented with synchronous tumors of the right carotid body, right cervical sympathetic trunk, right glomus jugulare, and left glomus tympanicum.
• Surgical resection of the sympathetic trunk and carotid body tumors was successful.
• Postoperative outcomes included transient Horner's syndrome and cranial nerve XII weakness, with good recovery of voice and recurrent laryngeal nerve function.

Conclusions:

• This case represents a novel presentation of multifocal paragangliomas involving the sympathetic chain, carotid body, glomus jugulare, and glomus tympanicum.
• The successful management highlights the importance of tailored surgical approaches and multidisciplinary care.
• Further research is needed to understand the genetic basis and optimal treatment strategies for such rare multifocal paraganglioma syndromes.