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Poster display session

2299 - Carcinoid syndrome: patient outcomes from a European Neuroendocrine Tumour Society (ENETs) Centre of Excellence


10 Sep 2017


Poster display session


Neuroendocrine Tumours


Paolo d'Arienzo


Annals of Oncology (2017) 28 (suppl_5): v142-v157. 10.1093/annonc/mdx368


P.D. d'Arienzo1, E. Amir2, A.R. Lewis3, L. Magdalani3, W. Mansoor3, R.A. Hubner3, J.W. Valle4, M.G. McNamara5

Author affiliations

  • 1 Medical Sciences, Sant'Anna School of Advanced Studies, 56127 - Pisa/IT
  • 2 Dmoh, Princess Margaret Hospital, M5G 2M9 - Toronto/CA
  • 3 Medical Oncology, The Christie NHS Foundation Trust, M20 4BX - Manchester/GB
  • 4 Medical Oncology, The University of Manchester / The Christie, Manchester/GB
  • 5 Medical Oncology, The University of Manchester / The Christie, M204BX - Manchester/GB


Abstract 2299


Carcinoid syndrome (CS), characterised by flushing, diarrhoea, wheeze and fibrotic valvulopathy, arises in patients (pts) with advanced NETs due to serotonin and kallikrein secretion.


Sequential pts with advanced well-differentiated gastroenteropancreatic NETs (GEP-NETs) treated at The Christie (1998-2017) with ≥1 carcinoid symptom(s) and raised serum/urinary 5-hydroxyindoleacetic acid (5-HIAA) were identified. Ratio of 5-HIAA/upper limit normal (ULN) was calculated. Progression-free (PFS) and overall survival (OS) were estimated (Kaplan-Meier method) and prognostic factors identified (Cox proportional hazards model).


Of 882 pts, 139 (16%) had CS: median (med) age 64 yrs, 55% male, 80% performance status (PS) 0-1, 13% PS 2; 65% had small bowel primary, 10% large bowel, 4% pancreas, 0.7% gastric, 21% unknown primary (consistent with GEP-NET origin). Tumour grade (G) was 1 in 45%; G2 in 29%; symptoms included diarrhoea (91%), flushing (89%), wheeze (22%), and carcinoid heart disease (CHD; 35%). Fifty-seven (41%) had primary resection, and 121 (87%) had liver metastases. In first line, 66% received a somatostatin analogue (SSA), 20% debulking surgery, 14% other. Med baseline 5-HIAA levels were 8.45 x ULN (urinary: 10.56 x ULN, serum: 6.07 x ULN). Med follow-up was 45.7 months (mo). Med PFS and OS were 27.0 (95%CI 17.2-33.9) and 65.4 (95%CI 50.4-76.4) mo. On univariate analysis, small bowel primary (P = 0.045), liver metastases (P = 0.03), Ki-67 (P 


Baseline 5-HIAA ratio, but not change from baseline to 6 months, was prognostic for PFS and OS. Treatment optimisation is pivotal.

Clinical trial identification

Legal entity responsible for the study

Audit Department - The Christie NHS Foundation Trust, Manchester




All authors have declared no conflicts of interest.

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