How Pseudomyxoma Peritonei Is Treated

Table of Contents
View All
Table of Contents

Pseudomyxoma peritonei (PMP) is a rare, slow-growing form of abdominal cancer. Curing this disease is a common goal of treatment. Treatments are also geared toward alleviating symptoms.

PMP starts as a small growth from the appendix that then enters the peritoneum (abdominal cavity). This causes the spread and accumulation of mucus-producing tumor cells, which generate abundant amounts of mucin (mucus).

Treatment is required to remove the mucin and mucus-producing tumor cells from the abdomen and pelvis. Without treatment, obstruction of the intestines and poor nutritional intake, which can be life-threatening, can occur.

The primary recommended treatment for PMP is cytoreductive surgery (reducing the amount of cancer cells) followed by hyperthermic intraperitoneal chemotherapy (HIPEC). Debulking surgery and other treatments, such as watchful waiting, may also be used.

This article will discuss the various treatments for PMP and what you can expect from each.

Doctor discusses treatment options with person with a rare cancer

DMEPhotography / Getty Images

Surgeries and Specialist-Driven Procedures

The primary form of treatment for PMP is surgery coupled with chemotherapy. Your overall health and age when you are diagnosed will both play a role in the treatments that are recommended for you.

Since there are side effects associated with most PMP treatments, your input and goals are very important and will be taken into account when a treatment plan is created.

Cytoreductive Surgery with HIPEC

The goal of cytoreductive surgery is to eliminate PMP by removing mucus and mucus-producing tumor cells. The length and extent of the surgery will be determined by the spread of tumor cells in the abdomen. You may be in surgery for up to 12 hours.

Your surgeon will strive to locate and remove all PMP cancer cells from your abdomen and pelvis. Based on the extent and location of the tumor cells, your surgeon may remove some or all of these tissues and organs:

  • Peritoneum (lining of the abdominal cavity and organs)
  • Omentum (the fold connecting the peritoneum to abdominal organs)
  • Gallbladder
  • Spleen
  • Bowel
  • Uterus
  • Ovaries

The spleen is instrumental in fighting infections. If your spleen is removed, you will be placed on antibiotics and this treatment may be ongoing if you are deemed at high risk for infection. You may also require several vaccinations prior to surgery.

After the removal of tissues and organs, your surgeon or another specialist on your cancer team will place heated chemotherapy liquid into the peritoneal space to wash it clean of any remaining cancer cells. This part of the procedure is known as hyperthermic intraperitoneal chemotherapy (HIPEC).

The chemotherapy drugs commonly used during HIPEC include mitomycin C and oxaliplatin. Heating these drugs enables them to better penetrate any remaining tumors. The chemotherapy liquid will remain in your body for 60 to 90 minutes and then be drained.

If needed, you will be given additional HIPEC treatments during the next five days after surgery. HIPEC is administered through a tube into your abdomen. You will remain in the hospital during this time, and possibly for days or weeks afterward.   

During the surgery, you may require a stoma. If so, your surgeon will make an opening through the abdominal wall, which will connect to a section of bowel. This redirects your bowel movements to a disposable bag that is worn externally over the stoma.

The stoma may be permanent or temporary. If you don’t need the stoma long term, this procedure will be reversed during a small operation about three to six months after cytoreductive surgery.  

Debulking Surgery

Debulking surgery is an alternative to cytoreductive surgery. It may be recommended if your doctor feels that cytoreductive surgery won’t be sufficient for removing all the PMP cancer cells. It may also be recommended if you’re not healthy enough to withstand the rigors of cytoreductive surgery.

Debulking surgery aims to alleviate symptoms by removing mucin and cancer cells. It may not include HIPEC and is not a potential cure.   

During debulking surgery, you may have internal organs, such as part of your bowel, removed. In some instances, debulking surgery may be done before cytoreductive surgery. It may also be required more than once.  

Having a permanent stoma is a common side effect of debulking surgery.

Chemotherapy

In some instances, chemotherapy may be administered without surgery. If you’re not healthy or strong enough for a surgical procedure, your healthcare provider may recommend this option to you.

Chemotherapy without surgery may be done to reduce symptoms. It may also be used to slow down or stop the spread of cancer.

Mitomycin C is often used intravenously to treat PMP without surgery. You may also be given capecitabine, another chemotherapy drug, orally in tablet form.

Watchful Waiting

PMP is indolent (slow growing) and may not cause symptoms for a long time. Often, this disease is uncovered during testing or treatment for a different health condition.

If you have PMP but don’t have symptoms or discomfort, your healthcare provider may recommend watchful waiting to you. They may also recommend watchful waiting if the side effects from treatment outweigh the benefits.  

During watchful waiting, you will be monitored regularly to check for the progression of the disease. Tests to expect include:

  • Computed tomography (CT) scans to check for mucin pouches and tumor growth
  • Blood tests to check for tumor markers (measurable substances that may indicate tumor presence)
  • Physical exams to check for symptoms such as abdominal distension (bloating)

If your disease shows signs of progression, your oncologist or another member of your cancer team will create a treatment plan for you. 

Novel Treatments

Your healthcare provider may recommend lesser-used PMP treatments in some instances. The reasons for this vary but may include your inability to withstand traditional treatments.

Novel approaches may also be recommended if your healthcare provider thinks traditional treatments won’t be effective enough to cure your disease or alleviate symptoms sufficiently. 

Pressurized Intraperitoneal Aerosol Chemotherapy (PIPAC)

PMP does not usually reach the small bowel. However, if the cancer is widespread and affects large blood vessels or the small bowel surface, your healthcare provider may recommend PIPAC to you. The goal of PIPAC is to prolong survival in PMP patients who are not candidates for surgery and chemotherapy.

PIPAC is still in the investigational stage, but has been performed on thousands of patients since its inception in 2013.

PIPAC is a minimally invasive procedure that may be repeated every six to eight weeks. During a laparoscopy, chemotherapy is administered into the peritoneal cavity in a mist form by a highly pressurized injector and a nebulizer. The pressure helps the mist penetrate into tumor tissue.

Clinical Trials

Clinical trials for PMP may explore alternative treatments that prolong life or alleviate symptoms. You can talk to your healthcare provider about clinical trials that are currently recruiting people with PMP. You can also keep an eye on upcoming trials at ClinicalTrials.gov.

Summary

Pseudomyxoma peritonei is a rare and slow-growing form of abdominal cancer. The primary treatment for this condition is surgery combined with chemotherapy. Novel treatments are sometimes recommended for people who are not candidates for traditional PMP treatment.

In people with no symptoms, watchful waiting is often recommended. At this time, no alternative treatments have been proven effective for alleviating or curing PMP.

A Word From Verywell

PMP treatments can be rigorous and challenging to go through. It may help to remember that the cure rate after treatment is around 65%.

Taking care of yourself while you're undergoing treatment and afterward is essential. Eating healthy food, getting enough rest, and other forms of self-care will help keep you grounded and focused on the future.

If it feels like a fit, consider connecting with a pseudomyxoma "buddy" through the Pseudomyxoma Survivor community and support system.

5 Sources
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read our editorial process to learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
  1. National Organization for Rare Diseases. Pseudomyxoma peritonei.

  2. Macmillan Cancer Support. Pseudomyxoma peritonei (PMP).

  3. Cancer Research UK. Treatment for pseudomyxoma peritonei.

  4. Pseudomyxoma Survivor. Pressurized intraperitoneal aerosol chemotherapy (PIPAC): a novel treatment for patients with PMP in whom extensive surgery is not possible.

By Corey Whelan
Corey Whelan is a freelance writer specializing in health and wellness conntent.