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Epidemiology and Genetics and Pattern of Spread

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Tumors of the Jugular Foramen

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Abstract

According to the World Health Organization paragangliomas are classified as neuroendocrine tumors. Head and neck paragangliomas comprise 0.3 % of all paragangliomas and 60 % are carotid body tumors. Jugulotympanic paragangliomas present an estimated annual incidence of 1 case per 1.3 million people. Jugular foramen schwannomas constitute approximately 2.9–4 % of all intracranial schwannomas and primary meningiomas of the jugular foramen are extremely rare. The genetic basis of development of paragangliomas, schwannomas, meningiomas, chordomas, and chondrosarcomas and mutation of different genes has been described. Familial forms of paragangliomas present multicentric tumors in almost 80 % of the cases. Transmission of paragangliomas is by an autosomal dominant gene. Schwannomatosis and neurofibromatosis are frequently associated with schwannomas and meningiomas in the jugular foramen. The knowledge of invasion routes of Jugular foramen paragangliomas with involvement of the internal carotid artery, middle ear, neck, and intradural helps to plan surgical approaches and predict the surgical difficulties.

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Authors and Affiliations

  1. Chairman of the Neurosurgical Department, Neurological Institute of Curitiba, Curitiba, Brazil

    Ricardo Ramina MD, PhD

  2. Chairman of the Neurosurgical Department, Eberhard Karls University, Tübingen, Germany

    Marcos Soares Tatagiba MD, PhD

Authors
  1. Ricardo Ramina MD, PhD
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  2. Marcos Soares Tatagiba MD, PhD
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Ramina, R., Tatagiba, M.S. (2017). Epidemiology and Genetics and Pattern of Spread. In: Tumors of the Jugular Foramen. Springer, Cham. https://doi.org/10.1007/978-3-319-43368-4_3

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  • DOI: https://doi.org/10.1007/978-3-319-43368-4_3

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