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Clinical utility of chromogranin A for surveillance of succinate dehydrogenase B- and D-related paraganglioma

Thompson, M, Parameswaran, V and Burgess, J ORCID: 0000-0003-4748-9571 2018 , 'Clinical utility of chromogranin A for surveillance of succinate dehydrogenase B- and D-related paraganglioma' , Annals of clinical biochemistry , doi:

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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:
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Copyright 2018 the authors

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