Intravagal paragangliomas: report of four cases

Abstract

The intravagal paraganglioma is a very rare tumor of the head and neck that accounts for only 5% of the paragangliomas in that area. A painless mass in the high neck with extension into the peripharyngeal space is the most common characteristic of this tumor. Malignant paragangliomas with invasion of the cervical lymph nodes and carotid artery have been reported, but the presence of metastasis, rather than the histological findings, is the only parameter for classifying them as malignant tumors. Despite the numerous descriptions of the efficacy of radiation therapy, the histological findings of irradiated specimens have shown little effect of radiation therapy on the chief cells. The only curative therapy for intravagal paragangliomas is the total resection of the tumor. Using the supra-adventitia dissection plane, we were able to achieve total resections in four cases of complex intravagal paraganglioma. The surgical management of these cases was complex because of the following: 1) misdiagnosis as a carotid body tumor, 2) previous radiation therapy and surgical procedure, 3) association with glomus jugulare, and 4) a giant tumor with invasion of the temporal bone and encasement of the internal carotid artery. We report the surgical management of intravagal paragangliomas and the role of radiation therapy, hormonal secretion, and rehabilitation care.