Incidence of Appendiceal Malignancies in Sweden Between 1970 and 2012

There is limited knowledge about the epidemiology of the major histological subtypes of appendiceal malignancy: adenocarcinoma and neuroendocrine neoplasm of the appendix (A-NEN). The aims of this national cohort study were to assess the prevalence, incidence and trends of appendiceal malignancies in Sweden.

All individuals who underwent appendicectomy and all diagnosed with appendiceal malignancy from 1970 to 2012 were identified from the National Patient Register and the Swedish Cancer Registry. Demographic data of the background population were obtained from Statistics Sweden. The incidence rate (IR) and the prevalence of appendiceal malignancy per performed appendicectomy were calculated.

We identified 3774 patients with appendiceal malignancy. IR of A-NEN was 5.8/10⁶ person-years with a peak of 8.4/10⁶ at age 20-30 years, whereafter it plateaued at a somewhat lower level. IR for adenocarcinoma was 3.7/10⁶ person-years, starting at a very low level among the youngest and increasing to 15.4/10⁶ at age 80-89 years. The IR of adenocarcinoma increased from 2.6/10⁶ in 1970-1979 to 5.4/10⁶ in 2010-2012. The IR of A-NEN was stable during the study period. The prevalence per appendicectomy was low for both types of malignancies among the young but increased with age, most dramatically for adenocarcinoma. There was a trend during the study period towards more extensive surgery.

Adenocarcinoma is most common and increasing in the elderly, whereas A-NEN affects all ages with a peak in young age. This peak probably reflects removal of occult A-NEN due to the higher appendicectomy frequency in the young.