IntroductionParagangliomas (PGLs) are rare and highly vascular tumors of neural crest origin that comprise only 0.6% of all head and neck tumors. 1 Formerly known as glomus tumors, or chemodectomas, PGLs develop from the extraadrenal chromaffin and chief cells of autonomic paraganglia in various locations throughout the body, usually in association with the walls of blood vessels or specific nerves. 2,3 Although these generally benign lesions most often present by exerting local effects on surrounding structures, a smaller subset of tumors can retain their neuroendocrine functionality and present with signs and symptoms of excess circulating catecholamines. These hypersecretory or functional PGLs can further complicate treatment by requiring a significant amount of autonomic pharmacotherapy to decrease the hemodynamic risks associated with any physical manipulation of the tumor itself.Of the various reported locations for PGL to develop in the head and neck, the carotid body has been cited as the most common site, followed in order of frequency by the jugulotympanic region, vagal body, and less common sites like the larynx, pharynx, and ciliary ganglion. 1 The infratemporal fossa (ITF) represents an especially rare location for a PGL to emerge, with only a handful of cases in the literature describing such a presentation. 4,5 Given the complex anatomy of the anterior skull base and the critical neurovascular structures within the ITF, successful resection of such a highly vascular tumor within this space presents numerous perioperative and intraoperative challenges. This report describes the management of a rare hypersecretory PGL of the infratemporal fossa.
Case ReportA 44-year-old white man was referred to the Head and Neck Surgery service for evaluation of a right skull base mass, after Keywords ► functional paraganglioma ► infratemporal fossa ► maxillary swing ► glomus tumor Abstract Background Paragangliomas are rare neural crest tumors that can manifest in the head and neck as either functional or more commonly as nonfunctional lesions. Paragangliomas of the infratemporal fossa are exceedingly rare, with no more than a handful of documented cases. Like other tumors of this space, surgical management is challenging on account of complex anatomy and nearby critical structures. Methods A 44-year-old man presented with a right infratemporal fossa functional paraganglioma. Following preemptive embolization and autonomic pharmacotherapy, his tumor was successfully resected via a transfacial maxillary swing approach. Results This case highlights the surgical management of a functional paraganglioma of the infratemporal fossa while demonstrating the effectiveness of a transfacial maxillary swing approach for both exposure and resection. Conclusion Although this report summarizes much of the literature on paragangliomas, there is still much to uncover regarding the fundamental features and genetic etiology of these lesions.