Head and Neck Pathology

, Volume 11, Issue 1, pp 88–95 | Cite as

Update from the 4th Edition of the World Health Organization Classification of Head and Neck Tumours: Paragangliomas

  • Michelle D. WilliamsEmail author
  • Arthur S. Tischler
Original Paper
Part of the following topical collections:
  1. World Health Organization Classification Update


Updated editions of The World Health Organization Classification of Tumours Pathology & Genetics for both Head and Neck Tumours and Tumours of Endocrine Organs took place in 2016 based on consensus conferences. These editions present unification of concepts in paragangliomas and highlight expanding knowledge of their etiology. There is a major emphasis in the new bluebooks on familial/syndromic paragangliomas, representing ~40% of all head and neck paragangliomas. Ancillary use of immunohistochemical evaluation, specifically of SDHB, allows the pathologist to screen for a large subset of these potentially hereditary cases. In addition, similarly to other neuroendocrine tumors, paragangliomas are now considered to represent a continuum of risk, and are assessed in terms of risk stratification. Tumors with SDHB mutations pose the highest risk for metastasis. There is currently no validated or endorsed histologic grading system. Paragangliomas remain tumors of undetermined biologic potential and should not be termed benign.


Paraganglioma Carotid body tumor SDHx SDHD Paraganglioma syndromes 


Compliance with Ethical Standards

Conflict of interest

The authors have no conflicts of interest to declare.

Ethical Approval

This article does not contain any studies with human participants or animals performed by any of the authors.


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© Springer Science+Business Media New York 2017

Authors and Affiliations

  1. 1.Department of Pathology, Head and Neck SectionUniversity of Texas MD Anderson Cancer CenterHoustonUSA
  2. 2.Department of Pathology and Laboratory MedicineTufts Medical CenterBostonUSA

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