PMP Research Foundation

To that end, we’ve put together a list of questions commonly asked by those diagnosed with Pseudomyxoma Peritonei (PMP) and related Peritoneal Surface Malignancies (PSM) - and their caregivers.

If you don’t find an answer to your question here, feel free to email us at info@pmpcure.org

For a glossary of commonly used terms in discussing PMP, please click here: PMP Glossary

Please be mindful that the following is provided for informational purposes only and should not be construed as medical advice. Only you and your PMP specialist can determine your treatment plan.

1. What does PMP stand for?

PMP stands for Pseudomyxoma Peritonei. PMP is a rare type of cancer characterized by a mix of tumor and mucin in the abdominal cavity. PMP has different classifications based upon the malignancy of the tumor mass and the slow or aggressive growth pattern of the tumor and mucin itself. These classifications are currently being standardized. For the moment, PMP Is a group of diseases that may best be regarded as "peritoneal surface malignancies" (PSM).

2. What diseases do you mean by "peritoneal surface malignancies?"

Of the number of possible origins of peritoneal surface malignancies, our organization supports the following diseases:

Appendiceal (Appendix) cancer.
Dissemenated Peritoneal Adenomucinosis (DPAM) of any origin.
Peritoneal Mucinous Adenocarcinoma
Peritoneal Mucinous Carcinomatosis (PMCA) of any origin.

3. How common is PMP and related PSM?

PMP was once thought to be diagnosed in about 1000 people world-wide each year. It's said that the lifetime odds of being diagnosed with PMP are about 1 in 1 million. However, specialists now believe it is more common than was once thought. For certain, PMP and PSM requires earlier & more accurate diagnostic tools.

      PMP Is listed among the rare or "orphan" diseases acknowledged by the National Association of Rare Disorders. NORD website. An orphan disease is generally considered to have a prevalence of fewer than 200,000 affected individuals in the United States.

      An orphan disease has such a low prevalence in the general population that a doctor in a busy general practice would not expect to see more than one case a year. It also means that pathologists see the disease infrequently and may misinterpret cell pathology, resulting in an incorrect diagnosis.

      Clearly, improved awareness and diagnostic methods in the medical community will benefit patients through earlier detection and treatment of PMP/PSM.

4. What's known about the cause of PMP?

There are a number of theories about the origins of PMP. The most commonly espoused theory is that an inflamed appendix leaks into the abdominal cavity and causes cells to implant there and produce the mucin and tumor.

However, there have been a growing number of exceptional cases where PMP starts from an ovarian mature teratoma, scrotal mass, urachus, and/or other sites.

More research needs to be conducted to understand this process.

5. Is there a typical profile of the person who gets PMP?

PMP affects men and women almost equally. Age of diagnosis ranges from late teens to late in life, with most individuals diagnosed in their 40's and 50's.

There does not appear to be a genetic (familial) link for PMP at this time.

The factors that predispose an indvidual to being diagnosed with PMP are unknown.

6. What are the common presenting symptoms?

Many patients lack clearly defined symptoms until the disease has reached an advanced stage. Following is a list of common symptoms:

Increased abdominal girth
Bloating
Pain/discomfort in the abdominal region (can be a dull ache or sharp pains similar to appendicitis)
Hernia symptoms (PMP is often initially diagnosed as a hernia, especially in men).
In women, symptoms of an ovarian cyst or tumor (PMP is often initially misdiagnosed as ovarian cancer).
Ascites (fluid) in the abdominal cavity.

7. How is PMP diagnosed?

For most patients, the disease is discovered during surgery for a different reason. In those cases a sample tissue can be obtained for a pathology study. Following is a list of the diagnostic tests commonly used in diagnosing PMP:

scan of the chest and the abdomen conducted by a radiologist who is trained in interpreting PMP scans. Note that CT scans are not very accurate, and for many patients they do not show any sign of tumor. Having a radiologists who is not trained to see PMP decreases your chances for an early diagnosis.

Discovery or exploratory laparoscopy: a thin tube with a camera at its tip is inserted through key holes in the abdomen to visualize the abdominal cavity and obtain sample tissue. Again, a specialist needs to conduct this procedure as they would follow appropriate protocols to prevent tumor seeding in the abdominal wall, in case it exists.
Tumor markers: Most commonly CEA, CA-125 & CA 19-9. Others may be ordered by your specialist. Unfortunately, these markers are not accurate or relative indicators for everyone. Certain patients will have no elevation in their tumor markers despite having extensive, aggressive tumor.

More research needs to be conducted to determine earlier, more accurate diagnostic tests for PMP and related PSM.

8. What is the best available practice in managing PMP/PSM?

     The standard of care followed by PMP/PSM specialists is a combination therapy that consists of a specialized cytroreductive ("debulking") surgery and localized heated chemotherapy (HIPEC).

     For many patients this therapy is conducted to increase longevity or to relieve some of the symptoms that affect their quality of life. For other patients this combined therapy carries good odds for long term survival, with a possibility of eliminating the disease.

     Cytoreductive surgery seeks to remove all visible tumor by removing the peitoneum and all organs affected by the tumor. Surgery is typically quite extensive and requires a very experienced specialist. After surgery is completed, the abdominal cavity is washed with heated chemotherapy (HIPEC) to remove non-visible or microscopic tumor cells.

     Some specialists recommend post-operative localized chemotherapy for a approximately three to five days, providing the patient tolerates it well. In many cases, a specialist will recommend preventive systemic chemo post-operatively.

Click here to read a good explanation of cytoreductive surgery: http://www.appendix-cancer.com/Cytoreduction%20Surgery.htm

Click here for more HIPEC info:http://health.ucsd.edu/cancer/patcare/hipec

9. Can you provide more details about Cytoreductive Surgery/HIPEC?

Surgery for PMP involves “debulking” the peritoneal cavity and surrounding affected organs of all visible cancer and mucin. Commonly, organs such as the spleen, gall bladder, female organs, & omentum are removed. Areas of the small and large intestine are often “resected” (cut and stitched or cauterized back together).
Depending on the degree of invasiveness of the cancer, an ileostomy may be performed. This may be temporary or permanent depending on the patient's individual circumstances.
Debulking surgery typically lasts 8-15+ hours. Surgery is then immediately followed by the perfusion of heated chemotherapy (typically Mitomycin C) which is used to kill cancer not always visible to the human eye. This may add another 90-120 minutes to the surgical procedure.
Some surgeons continue intra-abdominal chemotherapy for a period of 3-5 days post-surgery. Others feel this is not imperative and conclude the surgery with the one HIPEC procedure.
Cyto-reductive surgery is major surgery and recovery takes time. Without complications, hospital stays are in the range of 10 to 21 days.
Some people can return to work in about eight weeks, others may require several more months to regain their vitality.
Full recovery is estimated to take from 6 to 12 months depending upon a patient’s age and general physical condition.
Statistics indicate that the risk of morbidity (complications from surgery) is approximately 2% and the risk of mortality (death) is 2%.
Years ago a PMP patient termed cyto-reductive surgery “MOAS” standing for “Mother of All Surgeries.”

For more answers to questions about surgery, we defer to the PMP specialist who has conducted the greatest amount of surgeries and written most extensively on the subject, Dr. Paul Sugarbaker. Please note that this is a lot of information to take in, but it is crucial for your own survival that you learn the most you can about your condition and treatment therapies. Click here for Link to Sugarbaker Oncology Associates- handbook

10. Can any good physician / surgeon treat PMP?

We highly recommend you seek out a very specialized type of surgeon with the following qualifications:

A surgeon who has conducted at least 100 cytoreductions in order to learn all of the intricacies of this delicate and complex procedure.
Operates with a specialized team of surgeons, medical oncologists, anesthesiologists, radiologists, pathologists and oncology nurses.
Operates in a facility that offers HIPEC. Evidence from past practices suggest that cytoreduction without HIPEC does not increase your survival rate and accordingly does not increase the chance at a cure.
A physician who will be experienced enough in treating PMP to fully explain what to expect from this therapy and what post-op complications you may have according to your particular case.

We highly recommend that you seek out a specialist in treating PMP. There are perhaps a dozen practitioners in the U.S. and a half dozen more in countries such as Italy, Japan and Australia.

11. What questions should I ask my PMP Specialist?

We have prepared a list of questions you may wish to ask the physicians you consult with. Please remember, a surgeon with extensive experience in PMP/PSM's is highly recommended.

Click here for: Questions for Specialists.

12. Is systemic chemotherapy &/or radiation an effective treatment for PMP?

There is presently no consensus in the medical community about the use of chemotherapy &/or radiation in treating PMP/PSM. Some individuals appear to benefit from systemic chemotherapy while others do not.

Because PMP/PSM is atypical in that it doesn't typically spread via the lymph system, and because of its mucin component, the most effective therapy appears to be surgery and intraperitoneal chemotherapy as described in answer #8 above.

More clinical research needs to be conducted to establish the efficacy of systemic chemotherapy and radiation for PMP/PSM.

13. Is PMP considered curable?

For most patients PMP is, at the present time considered treatable, but not curable. There are, however, a growing number of individuals (mostly detected early and with favorable pathology) who have undergone cyto-reductive surgery and have experienced years of disease-free survival.

More information about some long term survivors can be found at an informative and helpful website at www.pmppals.org

14. What are the latest survival statistics?

Survival depends heavily on the type of PMP diagnosed, the stage at which the PMP was diagnosed, the degree of cyto-reduction achieved during surgery, the general health of the patient, and more.
For those with severe disease progression and signet cells, unfortunately the survival odds may be bleak. Less than 5% of these patients may survive five years.
For those with the less malignant, slow growing form of PMP, survival odds are typically beyond 5 years.
According to Dr. Paul Sugarbaker (see Specialists page for more information), 70% of individuals with low malignant, slow-growing (aka "low grade") cancer cells (often called DPAM) who have had optimal debulking surgery can be expected to live for 20+ years.

Remember, statistics are not representative of any one individual's experience - they are simply a measure of the past. You are always best discussing your particular prognosis with your PMP/PSM specialist. Don't become discouraged by statistics. Someone has to be at the far end of the curve... why not you?

15. What can I do to help PMP research move toward a cure?

Our mission is to fund research to prolong the length and quality of life for every person diagnosed with PMP in the future.

Please help us with our mission by generously donating funds. We want those diagnosed with PMP/PSM in 5 years to have greater hope than many of us living with PMP today.

To Donate Now, click here: Donate Now