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Incidence and prognostic value of serotonin secretion in pancreatic neuroendocrine tumours
Author(s) -
Zandee Wouter T.,
Adrichem Roxanne C.,
Kamp Kimberly,
Feelders Richard A.,
Velthuysen MarieLouise F.,
Herder Wouter W.
Publication year - 2017
Publication title -
clinical endocrinology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.055
H-Index - 147
eISSN - 1365-2265
pISSN - 0300-0664
DOI - 10.1111/cen.13364
Subject(s) - carcinoid syndrome , serotonin , medicine , chromogranin a , endocrinology , gastroenterology , incidence (geometry) , neuroendocrine tumors , immunohistochemistry , physics , receptor , optics
Summary Background Serotonin secretion occurs in approximately 1%‐4% of patients with a pancreatic neuroendocrine tumour ( PNET ), but the incidence is not well defined. The aim of this study was to determine the incidence of serotonin secretion with and without carcinoid syndrome and the prognostic value for overall survival ( OS ). Methods Data were collected from 255 patients with a PNET if 24‐hours urinary 5‐hydroxyindoleacetic acid excretion (5‐ HIAA ) was assessed. Patients were diagnosed with serotonin secretion if 24‐hours urinary 5‐ HIAA excretion was more than 3× the upper limit of normal ( ULN ) of 50 μmol/24 hours during follow‐up. The effect of serotonin secretion on OS was estimated with uni‐ and multivariate analyses using a Cox regression. Results Two (0.8%) patients were diagnosed with carcinoid syndrome, and another 20 (7.8%) had a serotonin‐secreting PNET without symptoms. These patients mostly had ENETS stage IV disease with high chromogranin A (CgA). Serotonin secretion was a negative prognostic factor in univariate analysis ( HR 2.2, 95% CI : 1.27‐3.81), but in multivariate analysis, only CgA>10× ULN ( HR : 1.81, 95% CI : 1.10‐2.98) and neuron‐specific enolase ( NSE ) > ULN ( HR : 3.51, 95% CI : 2.26‐5.46) were predictors for OS . Immunohistochemical staining for serotonin was positive in 28.6% of serotonin‐secreting PNET s (one with carcinoid syndrome) and negative in all controls. Conclusion Carcinoid syndrome is rare in patients with a PNET , but serotonin secretion occurs often. This is a negative prognostic factor for OS, but after correction for CgA and NSE , it is no longer a predictor and probably only a “not‐so innocent bystander” in patients with high tumour burden.