Plasma chromogranin A levels are increased in a small portion of patients with hereditary head and neck paragangliomas.
BACKGROUND
The majority of patients with head and neck paragangliomas (HNPGL) have biochemically silent tumours. Chromogranin A (CgA) is a tumour marker for neuroendocrine tumours.


OBJECTIVE
To assess the role of CgA as a tumour marker in patients with hereditary HNPGL.


METHODS
We included 95 consecutive patients with hereditary HNPGL for screening of plasma CgA levels and catecholamine excess by measurement of 24-h urinary excretion of (nor)metanephrine, (nor)adrenaline, VMA, dopamine and 3-methoxytyramine. In all patients with catecholamine excess, abdominal/intrathoracic paragangliomas were excluded by (123) I-MIBG scintigraphy, MRI and/or CT.


RESULTS
Plasma CgA levels were increased in only 15 of 95 patients (16%). Thirty-three of the 95 patients (35%) had increased urinary excretion rates of catecholamines. Six of these 33 patients (18%) had increased plasma CgA levels. Nine of the 62 patients (15%) with a biochemically silent tumour, i.e. no increased urinary excretion of catecholamines or their metabolites, had increased CgA levels. Increased plasma CgA levels were positively correlated with urinary excretion rates of noradrenaline (r = 0·68, P = 0·005) and normetanephrine (r = 0·68, P = 0·005). There was a positive correlation between maximal HNPGL diameter and plasma CgA levels in the 57 patients with a single HNPGL (r = 0·57, P = 0·001).


CONCLUSIONS
Plasma CgA levels are increased in only a small portion of patients with hereditary HNPGL and have limited additional value to the combination of radiological and routine biochemical assessment of patients with HNPGL. Increased plasma CgA levels are associated with increased noradrenergic activity and tumour size in patients with a single HNPGL.
Department of Endocrinology and Metabolic Diseases, Leiden University Medical Center, Leiden, The Netherlands. n.van_duinen@lumc.nl
Nicolette
N
van Duinen
Ido P
IP
Kema
Johannes A
JA
Romijn
Eleonora P M
EP
Corssmit
D000293:Adolescent; D000328:Adult; D000368:Aged; D053379:Chromogranin A / Q000097:blood*; D005260:Female; D006258:Head and Neck Neoplasms / Q000097:blood*; D006801:Humans; D008297:Male; D008875:Middle Aged; D010235:Paraganglioma / Q000097:blood*; D055815:Young Adult
D053379:Chromogranin A