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Pseudomyxoma peritonei, or PMP, is a rare type of cancer that affects the lining of the abdomen. It is estimated that PMP affects between 3-4 in every 1 million people each year, making it a rare disease. Despite its rarity, PMP can be a challenging disease to diagnose and treat.

PMP can affect people of any age, but it is most commonly diagnosed in people between the ages of 50 and 70. Some studies show that it is also more common in women than in men. However, the exact cause of PMP is unknown.

Recent research has shown that there may be a familial link to PMP in about 10% of cases. This means that in some families, multiple members may be affected by PMP. The research suggests that PMP may be caused by genetic mutations that are inherited from one or both parents. These mutations may increase the risk of developing PMP.

It is important to note that the other 90% of PMP cases are not related to genetics and do not have a known cause. This means that anyone can develop PMP, regardless of their family history or other risk factors.

The symptoms of PMP can be vague and non-specific, making it difficult to diagnose. Symptoms may include abdominal pain, bloating, and changes in bowel habits. These symptoms can be caused by many different conditions, which can make it difficult to diagnose PMP early.

Treatment for PMP typically involves surgical removal of the tumours, followed by heated intraperitoneal chemotherapy (HIPEC) to kill any remaining cancer cells. The extent of the disease and your overall health as the patient will determine the best course of treatment.

If you have a family history of PMP, it is important to talk to your doctor about your risk of developing the disease. Your doctor may recommend regular screening or other measures to help detect PMP early if it does develop.

 PMP is a rare disease that can affect anyone, but is more common in women between the ages of 50 and 70. While some cases may have a familial link, the majority of cases are not related to genetics and do not have a known cause. If you have any concerns about your risk of developing PMP, it is important to talk to your doctor. By staying informed and working with a specialized medical team, patients and their families can navigate the challenges of PMP and achieve the best possible outcomes. 

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Appendiceal Neoplasms and Pseudomyxoma peritonei: A population based study

Smeenk RM, van Velthuysen MLF, Verwaal VJ, Zoetmulder FAN (2007, May 23). Appendiceal Neoplasms and Pseudomyxoma peritonei: A population based study. European Journal of Surgical Oncology (EJSO). 20007. Retrieved April 5, 2023, from doi:10.1016/j.ejso.2007.04.002.

Epidemiology, histopathology, clinical outcomes and survival of 50 cases of appendiceal mucinous neoplasms: Retrospective cross-sectional single academic Tertiary Care Hospital experience

Aleter A, El Ansari W, Toffaha A, Ammar A, Shahid F, Abdelaal A (2021, March 23). Epidemiology, histopathology, clinical outcomes and survival of 50 cases of appendiceal mucinous neoplasms: Retrospective cross-sectional single academic Tertiary Care Hospital experience. Annals of Medicine and Surgery. Retrieved on April 5, 2023 from doi:10.1016/j.amsu.2021.102199.

Inherited cancer susceptibility gene sequence variations among patients with Appendix Cancer

Holowatyj AN, Washington MK, Tavtigian SV, Eng C, Horton C. Inherited cancer susceptibility gene sequence variations among patients with Appendix Cancer. JAMA Oncology. 2023;9(1):95. doi:10.1001/jamaoncol.2022.5425

Estimating the prevalence of Pseudomyxoma Peritonei in Europe using a novel statistical method

Patrick-Brown TD, Carr NJ, Swanson DM, Larsen S, Mohamed F, Flatmark K. Estimating the prevalence of Pseudomyxoma Peritonei in Europe using a novel statistical method. Annals of Surgical Oncology. 2020;28(1):252-257. doi:10.1245/s10434-020-08655-8

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What is the PCI score?

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.

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