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. 2017 Mar;11(1):88-95.
doi: 10.1007/s12105-017-0786-1. Epub 2017 Feb 28.

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

Michelle D Williams  1 , Arthur S Tischler  2
Affiliations

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

Michelle D Williams et al. Head Neck Pathol. 2017 Mar.

Abstract

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.

Keywords: Carotid body tumor; Paraganglioma; Paraganglioma syndromes; SDHD; SDHx.

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Conflict of interest statement

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.

Figures

Fig. 1
Fig. 1
Immunohistochemical evaluation for SDHB expression is a screening tool for possible genetic association. a H&E evaluation highlights the mixed cellular population present in paragangliomas including the rich vascular network around the zellenballen tumor structure. b Normal SDHB expression is granular in the cytoplasm and is identified in both the PGL cells, as well as, in the background tissues (endothelial cells and inflammatory cells). c Loss of SDHB expression signifies a mutation in one of the SDHx family genes, however may only be reported when expression is present in the internal control cells (i.e. endothelial cells and inflammatory cells). d Careful evaluation of SDHB expression is needed as PGL cells with SDHB loss (circles) may be obscured when there is a high inflammatory component (right) where SDHB will be retained. Discuss each panel in the text in order
See this image and copyright information in PMC

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