Incidence and prognosis of patients with small intestinal neuroendocrine tumors in a population based nationwide study

S Snorradottir; A Asgeirsdottir; S Rögnvaldsson; J G Jonasson; E S Björnsson

doi:10.1016/j.canep.2022.102197

Incidence and prognosis of patients with small intestinal neuroendocrine tumors in a population based nationwide study

Cancer Epidemiol. 2022 Aug:79:102197. doi: 10.1016/j.canep.2022.102197. Epub 2022 Jun 16.

Authors

S Snorradottir¹, A Asgeirsdottir¹, S Rögnvaldsson¹, J G Jonasson², E S Björnsson³

Affiliations

¹ Faculty of Medicine, University of Iceland, Reykjavik, Iceland.
² Faculty of Medicine, University of Iceland, Reykjavik, Iceland; Department of Pathology, Landspitali University Hospital of Iceland, Reykjavik, Iceland.
³ Faculty of Medicine, University of Iceland, Reykjavik, Iceland; Division of Gastroenterology and Hepatology, Department of Internal Medicine, Landspitali University Hospital of Iceland, Reykjavik, Iceland. Electronic address: einarsb@landspitali.is.

PMID: 35716441
DOI: 10.1016/j.canep.2022.102197

Abstract

Background & aims: Small intestinal neuroendocrine tumours (SI-NETs) are the most frequent malignant tumours of the small intestine. Population based studies on SI-NETs are scarce. We aimed to examine the incidence, presentation of disease and prognosis of SI-NET and to determine patient prognosis in those undergoing emergency or elective surgery.

Methods: This was a retrospective population-based study. Information on all patients diagnosed with neuroendocrine tumours of the small intestine (excluding duodenum) from the beginning of the Icelandic Cancer Registry and the pathology departments in the country (1966-2017). Detailed phenotypic information was obtained from medical records on symptoms at diagnosis, treatment, recurrence and survival.

Results: A total of 113 patients with SI-NETs were identified, 3 patients were excluded due to lack of data and/or diagnostic error, leaving 110 patients for final analysis. The incidence of SI-NET was 0.78/100,000 and did not increase during the study period. A total of 42 % (n = 46) of patients were diagnosed incidentally. Long-term prognosis, after a landmark of 12 months, was better in patients who were diagnosed incidentally (HR 0.52; p = 0.03). Overall 89 % (n = 98) of cases underwent surgical resection of the primary tumor, 31 % (n = 30) patients acute or semi-acute surgery and 69 % (n = 68) elective surgery. Emergency surgery was associated with a 6-fold risk of death in the first 12 months after surgery (HR: 5.99; p = 0.01) and associated with more severe surgical complications. However, there was no difference in the long-term risk of death after the first 12 months (HR: 1.39; p = 0.27).

Conclusions: The incidence of SI-NETs has not changed significantly in the last decades. Incidentally diagnosed SI-NET was associated with a favorable long-term prognosis. Emergency surgery in patients with SI-NET was associated with a significantly worse short-term risk of mortality compared to those who underwent elective surgery.

Keywords: Carcinoid; Epidemiology; Small intestinal neuroendocrine tumors; Surgery.

MeSH terms

Humans
Incidence
Intestinal Neoplasms* / epidemiology
Intestinal Neoplasms* / surgery
Neuroendocrine Tumors* / diagnosis
Neuroendocrine Tumors* / epidemiology
Neuroendocrine Tumors* / surgery
Prognosis
Retrospective Studies