What is pseudomyxoma peritonei?
Pseudomyxoma peritonei (PMP) is a very rare condition that generally develops from the appendix, where a ruptured mucinous tumor spreads throughout the abdominal cavity and produces a large amount of mucus.
In some cases, the condition can also arise from ovarian tumors or, more rarely, other abdominal organs. PMP affects about 1.5 person per million each year of all ages in Japan, both men and women.
Through our PMP patients’ network, you can meet, discuss, and support other families and patients living with this disease.
A large amount of mucinous fluid with the consistency of jelly in the abdominal cavity causes swelling of the abdomen. Disease progression is different for each patient, but is usually slow. Patients are frequently unaware of their condition until the abdominal cavity is filled with mucus produced by tumorous cells.
If left untreated, it will eventually take over the abdominal cavity and cause compression of the abdominal organs. The mucus can solidify into masses which may cause an intestinal obstruction. This condition can result in malnutrition and eventually life-threatening complications.
In the past, pseudomyxoma peritonei was considered an incurable disease, and no effective treatment was available except for non-effective chemotherapy or palliative debulking surgery.
However, since the late 1990’s, an effective treatment aiming towards a complete cure has been developed.
It is a comprehensive treatment with complete cytoreductive surgery (CRS) combined with hyperthermic intraperitoneal chemotherapy (HIPEC). Neoadjuvant or adjuvant chemotherapy is also performed if needed.
As this intensive surgery requires a high level of expertise to ensure safety and efficacy, it is recommended that you receive this treatment at a specialist center with highly experienced doctors and medical staff.
• Specialist centers in Japan •
- — Member hospitals of the NPO to Support Peritoneal Dissemination —
- Department of Surgery, Kusatsu General Hospital (Shiga Pref.)
- Peritoneal Surface Metastages Center, Kishiwada Tokushukai Hospital (Osaka)
- First Department of Surgery, Department of Obstetrics and Gynecology, Cancer care promotion center, University of Fukui (Fukui Pref.)
- Department of Surgery, Kishiwada City Hospital (Osaka)
- Division of Colorectal Surgery, National Center for Global Health and Medicine (Tokyo)
At Kusatsu General Hospital in Shiga and Kishiwada Tokushukai Hospital in Osaka, a total of about 500 patients with peritoneal dissemination including pseudomyxoma peritonei are treated each year. These hospitals are considered to be among the largest treatment centers in the world. You can also meet a specialist at Ikeda Hospital in Shizuoka (consultation only).
National Center for Global Health and Medicine (Division of Colorectal Surgery) also offers complete macroscopic tumour removal combined with heated intraoperative intraperitoneal chemotherapy (HIPEC), which has been approved as Advanced Medicine in Japan.
Kishiwada Tokushukai Hospital and National Center for Global Health and Medicine are authorized as hospitals that accept foreign paients as of 2016.
If you or your close family member has been diagnosed with PMP, it is recommended that you go to one of the above specialist centers for a second opinion. They can provide you with more options that will allow you to choose the most suitable treatment for you.
Situations in other countries
This comprehensive treatment has been promoted and proved to be successful in many countries on the initiative of the Peritoneal Surface Oncology Group International (PSOGI) launched in 1998.
As of 2015, there are about 200 specialist centers in Europe and about 100 in the United States that offer this comprehensive treatment. The same treatment is also provided in Canada, as well as countries in Central and South America and Africa. In some European countries, especially France and Great Britain, this treatment is regarded as the standard of care for PMP.
Throughout Asia, including Japan, there are fewer specialist centers (only 12 hospitals as of 2015), but progress is being made. In Korea, for example, this treatment has been covered by National Health Insurance since October 2014.