Management of paragangliomas. Clinical and angiographic aspects

Abstract

Head and neck paragangliomas are highly vascular tumors with variable clinical behavior. The goal of this paper is to determine specific clinical and radiological findings and extract from these findings a treatment algorithm. Twenty-three patients with paragangliomas were referred from different surgical centers for angiography and pre-operative embolization. Clinical records were analyzed retrospectively, and focused on impairment of cranial nerves. Angiographic features of paragangliomas, such as arterial supply, tumor flow characteristics, and venous drainage, were evaluated to find characteristic angioarchitectural patterns. All but one patient presented with a single tumor. All eight jugular and four of five vagal paragangliomas caused a lower cranial nerve deficit. Tympanic paragangliomas presented with hearing loss and tinnitus. The ascending pharyngeal artery was the main feeder and contributed to the supply in every tumor. Jugular and vagal paragangliomas typically compromise the patency of the jugular vein with resulting antegrade or retrograde flow through collateral venous systems. Surgical resection of vagal and jugular paragangliomas was especially performed when unifocal paragangliomas were present. In all of these patients, the tumor caused a cranial nerve deficit. The supply from an enlarged ascending pharyngeal artery is typical for paragangliomas. The venous drainage pattern of jugular and vagal paragangliomas allows differentiation from other vascular lesions at the skull base.

Figure 1

65-year-old woman with a right sided vagal paraganglioma. Because a complete jugular foramen syndrome is present, no additional risk of cranial nerve deficit is present when embolizing in the region of the jugular foramen. A) Selective injection of the right ascending pharyngeal artery (arrow) in lateral projection shows filling of a small tumoral compartment. The vertebral artery (small arrow) is filled retrogradely through the odontoid arcade (arrowhead). B) Selective injection of the right vertebral artery in AP projection shows same tumor opacification through the anastomotic channel (arrowhead).

Figure 2

56-year-old man with a right sided jugular paraganglioma. A) Selective injection of the ascending pharyngeal artery (arrow) in lateral projection shows only slight tumor opacification (arrowhead). B) Selective injection of the occipital artery in lateral projection shows the enlarged neuromeningeal trunk (arrow) giving the main supply to the lesion.

Figure 3

46-year-old woman with a right-sided tympanic paraganglioma. Selective injection of the ascending pharyngeal artery in lateral projection shows the tympanic branch (small arrow) feeding a small tympanic paraganglioma (arrowhead).

Figure 4

59-year-old man with suspected right-sided jugular paraganglioma. A) Selective injection of the ascending pharyngeal artery (arrow) in lateral projection shows a highly vascular lesion in the left temporal bone (arrowhead). No real parenchymal stain is seen. Rapid venous drainage in a patent jugular vein is present (small arrow). B) Same in antero-posterior projection, the venous drainage pattern suggests an osteodural arteriovenous malformation.

Figure 5