Treatment for pseudomyxoma peritonei

The main treatments for pseudomyxoma peritonei (PMP) are surgery and chemotherapy together.  It's called cytoreductive surgery (CRS) with hyperthermic intraperitoneal chemotherapy (HIPEC).

Your treatment depends on several factors. These include where the cancer is, and your general health.

You might not start treatment straight away. Your doctor closely monitors your cancer in case you need treatment in the future. This is called watch and wait.

 If you need treatment you might have:

• surgery with chemotherapy into the abdomen  - this is cytoreductive surgery (CRS) with hyperthermic intraperitoneal chemotherapy (HIPEC)
• surgery to remove as much cancer as possible - this is debulking surgery
• chemotherapy

Watch and wait

Your doctor might decide to closely monitor your cancer if it’s small and slow growing and you don’t currently need treatment. Your doctor will check up on you regularly. Watch and wait can also sometimes be called active surveillance. They do this with blood tests and scans .

You might find it hard to cope with this and struggle with feeling that no action is being taken.

Surgery combined with chemotherapy into the abdomen

Where possible, you’ll have surgery combined with chemotherapy. You have the chemotherapy directly into your abdomen. It's called cytoreductive surgery (CRS) with hyperthermic intraperitoneal chemotherapy (HIPEC).

You have to be well enough to have this intensive surgery. And the surgeon needs to be able to remove the disease without affecting your vital organs .

It involves:

• the surgeon removing any tissue affected by PMP
• having heated chemotherapy drugs put directly into your abdomen during the surgery (HIPEC)

Debulking surgery

Debulking surgery aims to remove as much of the cancer as possible. It does not remove the cancer completely.

Debulking surgery helps to make a diagnosis and get samples of the tumour. It can also remove mucin. It won’t cure PMP but might ease your symptoms. It could also mean that you can then have cytoreductive surgery with HIPEC.

Debulking surgery might mean removing part of your bowel. The surgeon might remove your womb and ovaries if you are a woman.

Unless the surgeon can remove the whole cancer, it's very likely to come back. Because of this, you might have debulking surgery more than once.

Chemotherapy

You might have chemotherapy if you can’t have surgery. You have this into a vein or take it as a tablet. This is a systemic treatment which means it works throughout your body.  

 You are more likely to have chemotherapy if:

• your cancer is causing symptoms
• CT scans show your cancer is growing quickly

You might have either mitomycin C into a vein with or without capecitabine as tablets. Doctors sometimes use other drugs.

It is important that you discuss the pros and cons of having treatment with your specialist. If your cancer is slow growing, the doctor might suggest that they monitor you. They will only suggest chemotherapy if your cancer is growing quickly or causing symptoms.  

There hasn't yet been enough research into the benefits of systemic chemotherapy for PMP.

Specialist centres for pseudomyxoma peritonei

The National Institute for Health and Care Excellence (NICE) has produced guidance for PMP. The guidance is on cytoreductive surgery with intraperitoneal chemotherapy. They recommend that people with PMP have treatment in a specialist centre.

There are 2 designated UK specialist treatment centres. This is because pseudomyxoma peritonei is very rare. The centres are:

• the Peritoneal Malignancy Institute at Basingstoke and North Hampshire NHS Foundation Trust
• the Colorectal and Peritoneal Oncology Centre at The Christie NHS Foundation Trust in Manchester

Many people with pseudomyxoma peritonei have treatment at another hospital first. This is because PMP wasn't suspected. Even if you have already had surgery, your specialist might suggest further surgery. This might be followed by intraperitoneal chemotherapy.

Research into treatment for pseudomyxoma peritonei

Researchers around the world are trying to improve treatments for pseudomyxoma peritonei. But because this type of cancer is so rare, it is difficult to do trials.

Doctors are looking at:

• drugs to relieve symptoms when PMP comes back or surgery is not possible

• chemotherapy for PMP

Where to get support and information

It can be very difficult to find support if you have a rare type of cancer. You are likely to find support from people at your local cancer support group. They will share your experience of being diagnosed with cancer and possibly of having major surgery. But you may want to try to contact other people who have the same condition as you. 

If you want to find people to share experiences with online, you could use Cancer Chat, our online forum.

Pseudomyxoma Survivor

Pseudomyxoma Survivor is a registered charity, run by patients and caregivers. They offer a befriending service called Pseudomyxoma Buddies, as well as provide practical and emotional support to people affected by PMP.