In the UK, it’s predicted that half of the people born after 1960 will be diagnosed with some kind of cancer at some point in their life, and someone in the UK is diagnosed with cancer every two minutes. However, pseudomyxoma peritonei (PMP) is a very rare type of cancer.
The incidence rate was previously approximated at 1–2 people per million per year, hence the “one in a million” tag for people with PMP. This incidence hadn’t been challenged, and the prevalence hadn’t been estimated until a team from Norway and a team from the UK looked at epidemiological data to look at the incidence rate based on the number of patients having a first surgical intervention¹. These statistics are based on those findings.
Cases
About 215 cases a year are diagnosed in the UK.¹
Survival
Of those that have had cytoreductive surgery² and HIPEC³, 63% survive pseudomyxoma peritonei for 10 years or more.¹
Cured?
98.7% of recurrences occur in the first 10 years after diagnosis. After that, a patient maybe considered cured.¹
Incidence
The incidence of pseudomyxoma (PMP), is estimated to be 3.2 people per million each year.¹
Written by the Pseudomyxoma Survivor editorial team.
Updated March 24, 2023.
Estimating the Prevalence of Pseudomyxoma Peritonei in Europe Using a Novel Statistical Method
¹Patrick-Brown TDJH, Carr NJ, Swanson DM, Larsen S, Mohamed F, Flatmark K. 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. PMID: 32488520; PMCID: PMC7752784.
Complete cytoreduction
Complete cytoreduction is the complete surgical tumour removal. This is a long operation which takes about 10 hours to complete and includes:
- removal of the right hemicolon
- spleen
- gall bladder
- greater omentum and lesser omentum
- stripping of the peritoneum from the pelvis and diaphragms
- tripping of tumour from the surface of the liver
- removal of the uterus and ovaries in women
- removal of the rectum in some cases
This is also referred to as a full peritonectomy. The goal is to remove tumour nodules down to 2.5mm.
HIPEC
Hyperthermic Intraperitoneal Chemotherapy
Heated chemotherapy, delivered directly into the abdomen, after complete cytoreduction. If the surgeons are able to remove the all the tumours, you may be given HIPEC.
Hyperthermic means that the chemotherapy solution used during the procedure is heated to a higher-than-normal temperature, typically around 41-43°C (105.8-109.4°F). This is usually Mitomycin C. This is put directly in your abdomen while you are in theatre. This elevated temperature is maintained throughout the duration of the procedure, usually around 90 minutes, to enhance the effectiveness of the chemotherapy drugs and to target cancer cells in the abdominal cavity. HIPEC will penetrate tumour nodules up to 2.5mm in size. The combination of chemotherapy drugs and hyperthermia can help to destroy cancer cells (increased cytotoxicity) while minimizing damage to healthy tissue.
Chemotherapy given into the abdomen during surgery is called intraoperative chemotherapy.
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What is the PCI score?
Doctors use the PCI score to help guide treatment options for Pseudomyxoma Peritonei (PMP) and other cancers that affect the lining of the abdomen.
What are the benefits of HIPEC?
HIPEC stands for “Hyperthermic Intraperitoneal Chemotherapy” and follows cytoreductive surgery (CRS).
My dad has pseudomyxoma peritonei. Should I have my appendix out?
If a blood relative has been diagnosed with PMP, you may be wondering if you should have your appendix removed as a preventative measure.