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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.
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