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In this chapter, we provide a focused review on a highly challenging subset of head and neck paragangliomas, that is, those arising from the skull base (jugulotympanic region). Presently, these tumors can be cured only with surgery, which must be performed in highly specialized skull base surgical centers. We review here the clinical presentation, diagnostic workup, classification, and surgical management of these rare but important tumors, together with currently available evidence concerning genetics, developmental origin, and pathology., (Copyright: The Authors.)

Carotid body and vagal paragangliomas, although considered indolent tumors, represent a challenge for the treating physician. This is mainly because of their peculiar localization, in close proximity with important anatomical structures. In addition, there is no chemotherapy available for these tumors, the role of radiation therapy is debated, and the only successful therapy is surgery. However, to achieve the best treatment goals, it is fundamental for the professional caregiver to master not only the clinical and surgical procedures but also the genetic backgrounds and the histopathological features of these tumors. We provide in this chapter a comprehensive review of the above mentioned aspects, with the aim to address the complexity of these tumors with a multidisciplinary approach., (Copyright: The Authors.)