Incidence, survival and stage at diagnosis of small intestinal neuroendocrine tumours in Queensland, Australia, 2001‐2015

Abstract

Aim: Multiple studies have observed increasing incidence of small intestinal (SI) neuroendocrine tumours (NETs). The aim of this study was to describe incidence, mortality and survival of SI NETs by sub-site and stage at diagnosis. Methods: Data on patients diagnosed with SI NETs between 2001 and 2015 were sourced from the Queensland Oncology Repository. Staging algorithms utilising several data sources were used to calculate stage at diagnosis (localised, regional or metastatic disease). Results:We identified 778 SI NETs and of those 716 (92%) had either a documented or derived stage. Incidence doubled from 0.68 per 100 000 to 1.42 per 100 000 over the 15-year period. Most common site was ileum (49.1%) and 84.2% were of carcinoid morphology type. Stage at diagnosis was calculated for 91.7% of patients with 28.3% presenting with regional involvement and 23.9% with distant metastasis. Risk factors associated with metastatic disease were jejunal and SI site not otherwise specified, neuroendocrine carcinoma histology and residing in a rural area. Increasing incidence of localised disease and a corresponding reduction in metastatic disease was observed over time. Five-year cause-specific survival for patients diagnosed between 2001 and 2005 was 82.5%, increasing to 93.8% from 2011 to 2015. Survival was lowest for those with metastatic disease (74.2%). Survival increased between 2001 to 2005 and 2011 to 2015 for each disease stage. Conclusions: SI NET incidence in Queensland doubled between 2001 and 2015. Survival was high and improved over time.

Survival of patients with small bowel neuroendocrine neoplasms in Auckland, Aotearoa New Zealand

“…This study shows that survival is highly dependent on stage at presentation with patients presenting with metastatic disease having significantly worse outcomes compared to patients with local or locoregional disease. Over a third of patients in this study presented with metastatic disease, a finding consistent with the literature which suggests 20–45% present with metastatic disease 5,9–12 . In these patients there has been debate regarding the upfront resection of the primary tumour without curative intent.…”

“…The incidence of NENs is increasing worldwide 5,6 . The Surveillance, Epidemiology, and End Results program has shown a 6.4‐fold increase in NENs in the USA since its inception in 1973 7 .…”

“…The Surveillance, Epidemiology, and End Results program has shown a 6.4‐fold increase in NENs in the USA since its inception in 1973 7 . This increase is likely multifactorial with factors including a true increase in incidence, an increase in detection, and a change over time in histological classification 5 . Incidence of SI‐NENs has increased in Queensland, Australia by 222% from 0.36 cases per 100 000 between 1986 and 1995 to 1.16 cases per 100,00 between 2006 and 2015 8 …”

“…In general NETs are slow growing tumours with 5‐year overall survival of 70–80% 5 . This is despite 20–45% of patients having metastatic disease at the time of diagnosis 5,9–12 .…”

“…4 The incidence of NENs is increasing worldwide. 5,6 The Surveillance, Epidemiology, and End Results program has shown a 6.4-fold increase in NENs in the USA since its inception in 1973. 7 This increase is likely multifactorial with factors including a true increase in incidence, an increase in detection, and a change over time in histological classification.…”