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A two-decade experience of head and neck paragangliomas in a whole population-based single centre cohort

  • Head and Neck
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Abstract

Paragangliomas are rare neuroendocrine tumours arising from neural crest-derived tissue. In the head and neck region typical locations are the carotid bifurcation, vagal nerve or jugulotympanic region. Paragangliomas are normally benign, and malignant transformation is rare. During the past decade the understanding of the genetic and molecular aetiology has had an important clinical impact on the management of PGs. This is a retrospective review of all histologically verified paragangliomas diagnosed and managed at an academic tertiary care referral centre between 1990 and 2010. Data on age, sex, symptoms, tumour location, management and follow-up were recorded. There were 64 patients with 74 tumours. Thirty-six per cent of the tumours were located in the carotid body region, 48 % in the jugulotympanic region and 15 % in the vagal nerve. One tumour was located in the dorsal neck. Most (95 %) of the patients were treated primarily with surgery and with curative intent. Definitive radiation therapy was primarily given to two patients. Recurrent or residual tumours were treated with surgery in three patients and with radiation therapy in nine patients. The typical long-term post-operative sequel was vocal cord paralysis. Local recurrence was found in 6 % of patients. Symptoms and findings related to paragangliomas are variable and management should be individualized. Surgery remains the primary choice of the current treatment options, but often is challenging and warrants a multidisciplinary approach. We present an algorithm on the management of head and neck paragangliomas based on current knowledge.

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Acknowledgments

The following are acknowledged: The Helsinki University Central Hospital Research Funds: Pekka Ranta, Elina Nikanne, Tommi Torkkeli, Jouko Nieminen, Erkki Hopsu, Kristofer Nyman and Matti Hynynen.

Conflict of interest

This research was funded by The Helsinki University Central Hospital Research Funds.

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Authors and Affiliations

  1. Department of Otorhinolaryngology-Head and Neck Surgery, Helsinki University Central Hospital and University of Helsinki, P.O. Box 220, 00029 HUS, Helsinki, Finland

    T. Anttila, V. Häyry, T. Nicoli, A. Mäkitie & L. J. Bäck

  2. Department of Pathology and Oral Pathology, Haartman Institute and HUSLAB, University of Helsinki and Helsinki University Central Hospital, P.O. Box 21, 00014, Helsinki, Finland

    J. Hagström

  3. Department of Clinical Genetics, HUSLAB, Helsinki University Central Hospital, P.O. Box 400, 00029 HUS, Helsinki, Finland

    K. Aittomäki

  4. Genome-Scale Biology Research Program, University of Helsinki, Helsinki, Finland

    K. Aittomäki

  5. Department of Vascular Surgery, Helsinki University Central Hospital, P.O. Box 340, 00029 HUS, Helsinki, Finland

    P. Vikatmaa

  6. Department of Neurosurgery, Helsinki University Central Hospital, P.O. Box 266, 00029 HUS, Helsinki, Finland

    M. Niemelä

  7. Department of Oncology, Helsinki University Central Hospital, P.O. Box 180, 00029 HUS, Helsinki, Finland

    K. Saarilahti

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  1. T. Anttila
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Correspondence to T. Anttila.

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Anttila, T., Häyry, V., Nicoli, T. et al. A two-decade experience of head and neck paragangliomas in a whole population-based single centre cohort. Eur Arch Otorhinolaryngol 272, 2045–2053 (2015). https://doi.org/10.1007/s00405-014-3161-9

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