Appendix cancer (also known as PMP) basically comes in two forms (1) low grade or (2) high grade. Low grade means that it usually stays within the abdomen, high grade (more aggressive) can infiltrate other nearby organs, or travel to other organs via bloodstream or lymphatic system. There have been disagreements and confusion over many years regarding the description of the types and variants of this disease.
“True” PMP is low grade where a cyst in the appendix bursts. Within the appendix there are cells which produce mucous, so if they leak into the abdominal cavity when the cyst bursts, they can multiply causing an accumulation of mucous (mucin), known as pseudomyxoma peritonei (PMP), also known as “jelly-belly”. Because the mucin spreads to and covers other organs in the abdomen it is often classed as Stage 4.
The gold standard treatment for this disease is cytoreductive surgery (CRS) – often referred to a MOAS (mother of all surgeries). This can be a very long surgery (up to sixteen hours) where the surgeons try to remove all visible signs of mucinous tumour which sticks to all the internal abdominal organs. Often, they remove the gallbladder, spleen, all or some of the large bowel, and sometimes some of the small bowel. Women often have their uterus and ovaries removed too. The liver and diaphragm are usually “scraped” to remove the mucin from their surfaces.
When as much mucin has been removed as possible, then heated chemotherapy (HIPEC) is introduced to the abdomen for around 90 minutes to bathe all the organs. The idea being to kill off all small residual mucinous tumours. Sometimes the chemo can be repeated for a few days after the surgery. Heating the chemo makes it more effective.
High grade has more of the features of a “usual” cancer. It can spread to nearby and distant organs. It is more sensitive to conventional systemic chemo although MOAS and HIPEC can still be used. Often the treatment is removal of the appendix with the complete right side of the colon, removal of any other visible tumour, and removal of lymph nodes. After a period of recovery, systemic chemo may be given. Again, if mucin producing cells leak into the abdomen, then PMP can develop.
To simplify it, I often think of “true PMP” as benign – however, one of the arguments related to this is – “well if it spreads to other organs how can it not be described as cancer”. Likewise, the disease may be benign but the condition is malignant in that if untreated (unless extremely slow-growing), the build-up of mucin with eventually lead to compression of the abdominal organs and eventually death.
Referrals for Appendix Cancer (AC) and Pseudomyxoma Peritonei (PMP)
AC / PMP is often diagnosed following surgery for suspected ‘other conditions’ (appendicitis, ovarian cancer etc.).
Following an AC/PMP diagnosis, it is essential that you are referred to an appendix cancer specialist. Many general surgeons and general oncologists consider these diseases to be similar to bowel cancer and treat them in the same way. While that MAY be appropriate, it isn’t always.
The two centres of excellence in the UK are:
When you are in ‘the eye of the storm’ following an AC/PMP diagnosis it is understandable that you are willing to be guided entirely by your medical team. However, if they do not refer you to one of the centres of excellence, PLEASE INSIST ON A REFERRAL!
More information about CRS/HIPEC
Contributor: James Caldwell – PMP Survivor, Treasurer of Cancer Patients Europe
“I was diagnosed with a high-grade mucinous adenocarcinoma of the appendix in early 2014 that had spread to my right ureter and bladder. I had a right hemi-colectomy and a bladder reconstruction followed by eight cycles of Xelox (Oxaliplatin and Capecitabine (Xeloda)). No evidence of disease since.”
Support Group Pseudomyxoma Survivor