Estimating the Prevalence of Pseudomyxoma Peritonei in Europe Using a Novel Statistical Method

Ann Surg Oncol. 2021 Jan;28(1):252-257. doi: 10.1245/s10434-020-08655-8. Epub 2020 Jun 2.


Background: The determination of the incidence and prevalence of rare diseases is important for economists and health-care providers. Pseudomyxoma peritonei (PMP) is a rare, slow-growing abdominal cancer that represents a substantial burden on both patients and health-care systems. The incidence rate was previously approximated at 1-2 people per million per year; this incidence has never been challenged, and the prevalence has not been estimated.

Methods: Epidemiological data from Norway and England were obtained and analysed to calculate a minimum incidence rate based on the number of patients having a first surgical intervention for PMP. A novel method was then used to determine a prevalence rate for PMP, incorporating incidence, death, and cure rates in a multi-year analysis that accounted for the increasing population of Europe over a 10-year period.

Results: An incidence rate of 3.2 people per million per year was calculated, with a corresponding estimated prevalence rate of 22 people per million per year. By this calculation, 11,736 people in Europe were estimated to be living with PMP in 2018.

Conclusion: Incidence and prevalence are essential tools for assessment of the financial and human cost of a disease. For rare diseases, such as PMP, the lack of accurate registries presents a particular challenge in determining such health-related statistical parameters. Based on our calculations, a significant number of people are living with PMP in Europe, underlining the need for appropriate resource allocation to ensure that adequate health-care measures are provided.

MeSH terms

  • Europe / epidemiology
  • Humans
  • Norway
  • Peritoneal Neoplasms* / epidemiology
  • Prevalence
  • Pseudomyxoma Peritonei* / epidemiology