Pseudomyxoma Peritonei

I was given a case report written by one of the doctors on mom's diagnosis upon discharge and was advised to bring along the report to T&E Unit (Trauma & Emergency) should mom needed immediate medical attention.

Mom's diagnosis: Pseudomyxoma Peritonei, sounds so alienated to me. I started doing a search on the internet to get more information about this new term.

What pseudomyxoma peritonei is

Pseudomyxoma peritonei is a very rare type of cancer that usually begins in your appendix as a small growth, called a polyp. Or, more rarely, it can start in other parts of the bowel, the ovary or bladder. This polyp eventually spreads through the wall of your appendix and spreads cancerous cells to the lining of the abdominal cavity (the peritoneum). These cancerous cells produce mucus, which collects in the abdomen as a jelly like fluid called mucin. The cause of this type of cancer is not known. Most cancers are caused by a number of different factors working together.

How pseudomyxoma peritonei spreads

Pseudomyxoma peritonei does not act like most cancers and does not spread through the bloodstream or the lymphatic system. It spreads along the internal surfaces of the abdomen, rather than into nearby tissues of the bowel or liver. The mucus collects in the peritoneum and causes symptoms. It may be many years before you have any symptoms from this type of cancer.

Symptoms of pseudomyxoma peritoneiSymptoms can include:
* Abdominal or pelvic pain
* Not being able to become pregnant
* Abdominal swelling and bloating
* Changes in bowel habits

Some people will not have any of these symptoms, so pseudomyxoma peritonei can be difficult to diagnose. In women, this type of cancer can sometimes be confused with ovarian cancer, because ovarian cancer may also cause a swollen abdomen. Some types of ovarian cancer cells also produce mucin. Often, it is only after an operation to look into the abdomen (a laparotomy) that pseudomyxoma peritonei is properly diagnosed. Once diagnosed, a CT scan can help the doctor find out how much of the abdomen is affected by the cancer.

Treatment for pseudomyxoma peritonei

You are most likely to have
* Surgery
* A specialist type of chemotherapy called IP chemotherapy

Surgery

You are most likely to have surgery to treat this type of cancer. There are two types of surgery
* Debulking surgery
* Complete tumour removal (Sugarbaker technique)

Debulking surgery is the most common. The aim is to take out as much of your tumour as possible. This might mean removing your womb and ovaries if you are a woman, and sometimes part of your bowel. Unless you have intensive surgery, it is hard to remove all of the tumour. So the cancer is likely to come back. Then you need to have more surgery, and sometimes several debulking operations. But each operation becomes more difficult to do. If you have a lot of abdominal surgery, you are also likely to develop complications, such as ‘adhesions’, where the abdominal tissues stick together and cause problems such as pain.

Recent research suggests that some people with pseudomyxoma peritonei may benefit from a very intensive type of surgery that involves trying to remove all traces of the cancer from the abdominal area. Your doctor may call this intensive treatment the Sugarbaker technique after the doctor who developed it. A large section of your bowel, spleen, gall bladder are removed and, if you are female, your ovaries and womb.This intensive surgery is not suitable for everyone. The surgery takes a very long time and you will be in intensive care for about 5 days. You will need to be fed by a tube, and will stay in hospital for about a month. About 3 out of every 10 people (30%) have serious complications after treatment.

About 1 out of 5 patients (20%) need a stoma after surgery. In about half of these people the stoma is only needed for 3 to 6 months. And it is not always possible to know how far your cancer has spread until the operation takes place. So the surgeon cannot guarantee to remove the tumour completely. Chemotherapy into a vein or as tablets does not work well for this type of cancer. Because the disease has a poor blood supply the drugs can’t get to the tumour cells if you have them as a drip or tablets. But after surgery to remove all the cancer, doctors may use a different way of giving chemotherapy called intraperitoneal chemotherapy. This means putting the chemotherapy drugs straight into your abdomen, where they can directly contact with the cancer cells. To have intraperitoneal chemotherapy, you have a heated solution of mitomycin C put into your abdomen during and after your operation.

After your surgery, you may also have another chemotherapy drug called 5FU put into your abdomen for 4 days. NICE (The National Institute for Health and Clinical Excellence) has produced guidance on this intensive surgery with intraperitoneal chemotherapy. They are uncertain about its risks and benefits and recommend that it is only carried out in very specialised centres. And the risks and benefits must be discussed with patients before they consent to treatment.