The National Institute for Health and Clinical Excellence (NICE) has issued full guidance to the NHS in England, Wales, Scotland and Northern Ireland on Complete cytoreduction for pseudomyxoma peritonei (Sugarbaker technique).
As part of the NICE's work programme, the current guidance was considered for review but did not meet the review criteria as set out in the IP process guide. The guidance below therefore remains current.
This is a radical treatment for pseudomyxoma peritonei.
Pseudomyxoma peritonei is rare, occurring in about one person per million per year. It is a slowly progressive tumour arising from the appendix or bowel, which spreads throughout the peritoneal cavity and produces a large amount of mucus. The condition is considered borderline malignant.
Disease progression is usually slow, some people surviving for several years after diagnosis. However, most people will develop symptoms caused by the bulk of the tumour.
The Sugarbaker technique was developed by Paul Sugarbaker at the Washington Cancer Institute. It involves complete surgical tumour removal (also known as complete cytoreduction) combined with intraoperative heated chemotherapy, followed by postoperative intraperitoneal chemotherapy. The operation 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 diaphragm
- stripping of tumour from the surface of the liver
- removal of the uterus and ovaries in women
- removal of the rectum in some cases
The standard surgical approach is debulking, in which the surgeon attempts to remove as much tumour as possible, and usually removes the right hemicolon, and uterus and ovaries in women. Disease recurrence is very common. People often need several debulking operations.
People with pseudomyxoma peritonei may also be treated using a 'watch and wait' policy, involving surgery only when unacceptable symptoms or life-threatening complications such as intestinal obstruction arise.