Abstract
BackgroundThe 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.MethodsEpidemiological 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.ResultsAn 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.ConclusionIncidence 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.
Highlights
The determination of the incidence and prevalence of rare diseases is important for economists and health-care providers
Based on the statistics obtained from the Norwegian Radium Hospital, the incidence of Pseudomyxoma peritonei (PMP) in Norway was calculated for the years 2009–2018 based on the date of first surgical intervention (Table 2)
We propose a novel method for period prevalence calculation applied to PMP, which is a slowgrowing rare cancer
Summary
The determination of the incidence and prevalence of rare diseases is important for economists and health-care providers. A novel method was 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. 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. Incidence and prevalence are essential tools for assessment of the financial and human cost of a disease
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Free) 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
