444P - Carcinoid syndrome: patient outcomes from a European Neuroendocrine Tumour Society (ENETs) Centre of Excellence

Date 10 September 2017
Event ESMO 2017 Congress
Session Poster display session
Topics Neuroendocrine Cancers
Endocrine Cancers
Presenter Paolo d'Arienzo
Citation Annals of Oncology (2017) 28 (suppl_5): v142-v157. 10.1093/annonc/mdx368
Authors P.D. d'Arienzo1, E. Amir2, A.R. Lewis3, L. Magdalani3, W. Mansoor3, R.A. Hubner3, J.W. Valle4, M.G. McNamara5
  • 1Medical Sciences, Sant'Anna School of Advanced Studies, 56127 - Pisa/IT
  • 2Dmoh, Princess Margaret Hospital, M5G 2M9 - Toronto/CA
  • 3Medical Oncology, The Christie NHS Foundation Trust, M20 4BX - Manchester/GB
  • 4Medical Oncology, The University of Manchester / The Christie, Manchester/GB
  • 5Medical Oncology, The University of Manchester / The Christie, M204BX - Manchester/GB



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.