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Pseudomyxoma peritonei (or PMP) is an extremely rare disease. Estimates vary and it’s often quoted as the ‘1 in 1 million disease’. However, it is now thought that PMP is diagnosed in between 3-4 people per million each year¹. Possibly due to prior misdiagnosis or misclassification, some treatment centres are reporting that incidence may be as high as 10 per million per year.

PMP is identified by Eurordis as a rare or “orphan” disease (one which affects less than 1 in 2000 people).

The incidence of PMP is so low that most GPs will never see a case in their working life which makes it much more difficult for symptoms to be picked up. Most pathologists see few cases and may identify the cells incorrectly resulting in an incorrect diagnosis and consequently inappropriate or no treatment.

It’s important to know how many people have a disease because it helps us understand how much money and resources we need to treat it. For a rare disease like pseudomyxoma peritonei, it’s hard to know how many people have it because there may not be a good system to keep track of it. But based on some estimates, there are still a lot of people in Europe who have it. This means we need to make sure we have enough doctors and resources to help treat them.

@eurordis, #pseudomyxomaperitonei


(1) Estimating the incidence and prevalence of pseudomyxoma peritonei in Europe

¹Patrick-Brown, T.D.J.H., Carr, N.J., Swanson, D.M. et al (2020). Estimating the Prevalence of Pseudomyxoma Peritonei in Europe Using a Novel Statistical Method. Ann Surg Oncol (2020). Retrieved on May 28, 2022, from doi:10.1245/s10434-020-08655-8.

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