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Abstract

Background:Patients with mutations of succinate dehydrogenase B (SDHB) and D (SDHD) are at high risk of paraganglioma (PGL) necessitating surveillance. Chromogranin A (CgA) has been proposed as a biochemical marker of PGL. We sought to determine the diagnostic utility of CgA in a population based SDHx sample.Methods:Tasmania is an island state with one tertiary referral centre for endocrine neoplasia. We performed cross sectional analysis of all adult SDHB (n=52) and SDHD (n=10) patients undergoing PGL surveillance between 2011 and 2017. CgA was referenced against the outcome of PGL surveillance with a minimum of 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) and plasma metanephrines (metanephrine and normetanephrine).Results:CgA correctly predicted the result of PGL surveillance more often in patients with SDHB compared to those with SDHD (77% vs 22%, p=0.003). In the SDHB group, CgA demonstrated a sensitivity of 67% and specificity of 79% compared to 22% and 0% in the SDHD group. CgA identified one of three PET/CT-visualised SDHB-related PGLs with normal plasma metanephrines at the expense of nine false positive results. A normal CgA demonstrated a negative predictive value of 92% for SDHB-related PGL. In patients with SDHB, plasma normetanephrine and metanephrine offered superior specificity (100%, p=0.01 and 100%, pConclusion:CgA does not provide additive benefit to standard surveillance for predicting the presence of SDHB- or SDHD-related PGL, but has a useful negative predictive value when normal in patients with SDHB mutation.

Item Type:	Article
Authors/Creators:	Thompson, M and Parameswaran, V and Burgess, J
Keywords:	Chromogranin A, paraganglioma, SDHx
Journal or Publication Title:	Annals of clinical biochemistry
Publisher:	Royal Soc Medicine Press Ltd
ISSN:	0004-5632
DOI / ID Number:	https://doi.org/10.1177/0004563218811865
Copyright Information:	Copyright 2018 the authors
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