Pseudomyxoma Peritonei is a tumor in the lining of the abdominal cavity that secretes large amounts of mucus. It consequently causes increasing abdominal girth, the so called "jelly-belly." Because it is a relatively rare condition, its treatment is best handled by specialists familiar with its treatments and the newer treatment regimens.
Difficulty: Moderately Easy
Check the review article on the website of the Sugarbaker oncology associates. It has a flow chart that graphically depicts the treatment options and how they should be pursued. It also links to a detailed discussion of cytoreductive surgery, the primary surgical means of removing the tumors.
Discuss treatment with your primary care physician (PCP). He or she will have an idea of what initial options are available for treatment. These will be dependant upon your insurance.
Begin your search for a specialist by following the recommendation of your PCP. In all likelihood, this will be an appointment with a local cancer specialist (oncologist).
Contact one of the national centers for cancer therapy like M.D. Anderson hospital at the University of Texas in Houston. This hospital has reported a surgical series of 45 patients with pseudomyxoma and obviously has experience. You may not need to go to a national center for treatment, but they may have resources for you with regard to choice and local physicians familiar with the disease.
Discuss treatment alternatives with your oncologist and your PCP. Ask the oncologist how many cases of pseudomyxoma he or she has seen. Also, discuss how sure everyone is of your diagnosis. There are a number of different tumors that can produce the same symptoms.
Seek a second opinion regardless of what the oncologist suggests. This is not an affront to the oncologist. For a rarer tumor like a pseudomyxoma, it is simply an appropriate precaution. If possible, get an opinion from a local medical school. All medical schools are referral centers and see greater numbers of uncommon problems.
Go through the same process if surgery is recommended--seeing the surgeon to whom you are referred but seeking a second opinion. Keep your PCP in the loop at all times. Your PCP must be the anchor of your treatment since only he or she knows you and understands all of your medical problems. In addition, as you move through these various stages of treatment, your PCP will be an invaluable source of support.
Go through whatever steps are needed to go to a national center like M.D. Anderson if you are uncomfortable after having pursued all your local options. While national centers are not always a best solution since followup is difficult and a patient is often rushed through treatment in an impersonal fashion, going to one may be necessary if you have discomfort with your local options.
Tips & Warnings
- As you go through your search for a specialist, make sure you collect copies of all pertinent reports, histories and physicals, pathology reports, lab reports, and consultations. Have an extra copy of the reports available in case a specialist needs them, but keep your own copies intact at all times. This greatly hastens your treatment.
- Do NOT discount trust as being an important part of your treatment. Trusting your surgeon and oncologist is a crucial part of successfully combating any disease but particularly one like pseudomyxoma which can have confusing options and one that is being explored with new treatments.