Primary Peritoneal Cancer: Symptoms, Diagnosis, Treatment, and Clinical Trials
Primary peritoneal cancer, or PPC, is a rare type of cancer that attacks primarily women and is often considered to be a gynecologic cancer. It’s a very aggressive type of cancer and can spread quickly to other parts of the body. In makeup, it’s very similar to ovarian cancer, but even women who have had their ovaries removed can develop peritoneum cancer.
Primary peritoneal cancer is often referred as “the silent killer.” PPC often causes no symptoms, or either mild symptoms, when it first starts. Because the symptoms of PPC are often so vague, they are often dismissed without seeking a medical opinion. Once primary peritoneal cancer causes symptoms that can no longer be ignored, it has usually reached at least stage III. Because of this, the cancer has been assigned only two stages: III and IV.
Though primary peritoneal cancer is rare, it received considerable news coverage when a popular celebrity was diagnosed with PPC. This is the type of cancer that famous Saturday Night Live comedienne Gilda Radner had.
What is the peritoneum?
The peritoneum is the thin membrane that lines the abdominal cavity. It’s the largest space in the human body. It’s made up of epithelial cells and has a vast network of vascular and lymphatic capillaries. This lining produces a fluid that lubricates the internal organs, making them slide around easily when the body moves. If it weren't for this slippery fluid, our internal organs would "stick together" whenever they came in contact with each other.
The peritoneum is actually made up of two layers. The outer layer is attached to the abdominal wall and is called the parietal peritoneum. The inner layer encases the internal organs and is called the visceral peritoneum. The area between the two layers contains the aforementioned lubricating fluid and is referred to as the peritoneal cavity.
Who’s at risk for primary peritoneal cancer?
Doctors aren’t sure what causes peritoneal or peritoneum cancer. Risk factors include advanced age, a familial history of breast cancer, and being female. PPC usually affects women between the ages of 40 and 60. Men can have peritoneal cancer, but the occurrence is extremely rare.
Estimates for the number of people affected by PPC vary widely. In the United States, estimates for women diagnosed with peritoneum cancer each year range from about 2,000 up to 10,000. In the European Union, the figure is 0.3 people in 10,000 who are diagnosed with primary peritoneal cancer. Remember though, that PPC is difficult to diagnose in its early stages, so the true number of people with peritoneum cancer is probably much larger.
What are the symptoms of PPC?
Unfortunately, peritoneal cancer does not usually cause symptoms until the disease is in the advanced stage. Even then they’re sometimes vague or difficult to describe. When symptoms do present, they often include:
- Abdominal bloating
- Loss of appetite
- Weight loss or gain
- Unusual vaginal bleeding
- Frequent urination
- Abdominal swelling from fluid
- Abdominal cramps and pressure
- Abdominal pain, usually in the lower abdomen
How is peritoneal cancer diagnosed?
If you’re experiencing symptoms compatible with peritoneal cancer, see your doctor as soon as possible. Chances are that you're suffering from some other disorder, but it's better to be sure. He’ll ask you general health questions, as well as specific questions about your symptoms. He’ll use both a physical examination and tests as diagnostic tools.
Some of the procedures include:
Pelvic exam: The doctor will insert a lubricated gloved hand into the vagina in an effort to feel and assess the ovaries, the uterus, the bladder, the fallopian tubes, the rectum, and the vagina itself. The physician will be able to determine the size and shape of the organs and thereby note any abnormalities.
Rectal exam: Your physician might need to insert a lubricated gloved finger into your rectum to better evaluate parts of the peritoneum or organs.
Lower GI series: For this, you’ll be given an enema containing barium – a white chalky substance that shows up on x-ray. The procedure is uncomfortable but is not painful.
Blood tests: A CA125 test may be ordered. This measures the amount of a specific protein in your blood that is often elevated with ovarian or peritoneal cancer. It should be noted that other forms of cancer and even benign conditions can elevate the CA 125 level.
Computed Tomography (CT) scan: This painless procedure generally takes from 20-30 minutes. You will be given some sort of contrast. It can be in the form of a drink, an enema, or an intravenous injection. Since it contains iodine, be sure to tell your doctor if you’re allergic or if you have asthma. A momentary flushed or hot sensation is normal, but it passes soon.
Magnetic Resonance Imaging (MRI): This is a painless procedure, but it often takes an hour to complete. During that time, you’ll have to lie completely still. You’ll slide into an open ended “tunnel.” Some patients, especially those with claustrophobia, might be given a mild sedative to help them relax. There are also open MRIs, which are less claustrophobic. If you suffer from claustrophobia, ask your doctor about the possibility of an open MRI.
Ultrasound: This is a non-invasive procedure that allows the inside of the abdominal cavity to be seen and assessed for peritoneal cancer and other problems. If you’ve been pregnant in the last 30 years or so, you’ve probably had one of these. A conductive jelly is spread onto the abdomen, and an instrument is passed along the belly. The procedure is painless.
Vaginal ultrasound: With this procedure, a small sound wave device is placed in the vagina to create an image of the internal organs and the peritoneum. The device is about the size of a tampon. The procedure is painless but might be uncomfortable.
Laparoscopic biopsy: In this procedure, a surgeon will make a small incision into the perineum. A small amount of peritoneal tissue will be removed so that it can be tested for peritoneum cancer. You’ll have to be put to sleep for this, so a short hospital stay is required.
Fluid aspiration: Peritoneum cancer sometimes causes fluid to accumulate in the abdominal cavity. In this procedure, a local anesthetic is used to numb the skin, then a small amount of this fluid is drawn and tested to determine if peritoneal cancer cells are present.
What are the treatments for cancer of the peritoneum?
Because primary peritoneal cancer is almost always in either stage III or stage IV before it's discovered, advanced treatment methods are usually used. Primary treatment includes:
Surgery: You will be admitted in to the hospital and put to sleep under general anesthesia. This surgery should be done only by a gynecological oncologist. He or she will remove as much of the cancerous tissue as possible. This might include the ovaries, the uterus, and some abdominal fat called the omentum. The surgeon will also be able to detect the initial source of the peritoneum cancer.
Chemotherapy: Chemotherapy involves the administration of drugs that have cancer cell-destroying properties. Most of these drugs work by inhibiting the abnormal cells from dividing and multiplying. You will likely receive these drugs intravenously, as an outpatient. The chemotherapy drugs may be used before and after surgery. This treatment may be administered once a week, once every two weeks, or once every three weeks.
Radiotherapy (radiation therapy): This includes high-energy radiation that can be delivered in two ways. One is through a beam or radiation outside the body, and the other is placed inside the body. Both types of radiotherapy destroy cancerous cells and shrink any tumors.
Palliative care: If patients are too sick or weak to undergo chemotherapy, supportive care is given to help make them more comfortable. This might include a procedure called paracentesis - the draining away of excess abdominal fluid. Other palliative measures usually include pain management and a special diet. Anyone with this type of cancer needs a strong support group of family members, friends, and/or professional counselors.
Follow up for patients with cancer of the peritoneum
It’s imperative that you keep all your appointments with your oncologist so that he will know if the peritoneum cancer spreads. He can also evaluate any complications from the cancer and from the therapy. Be sure to tell your health care team of any new symptoms or side effects from therapies or medications.
Clinical trials for primary peritoneal cancer
Clinical trials studying the effects of new anti-cancer drugs used in the advanced treatment of primary peritoneal cancer, along with other cancer treatment options, are ongoing. Currently, there’s one being conducted at Memorial Health University Medical Center in Savannah, Georgia, studying the use of ChemoFx. One in New York, at Mt. Sinai Medical Center is testing the effectiveness of Paclitaxel or Xyotax.
If you’re interested in participating in a PPC clinical trial, talk to your physician about the risks and possible benefits of such cancer treatment options. If he thinks you might be a good candidate, you’ll need to get more information about the specific trial and be approved by the study group.
Remission of primary peritoneal cancer
If your PPC cancer goes into remission, it’s important for your doctor to keep abreast of your condition. You will be asked to return for checkups once every few months. Unfortunately, this type of cancer often recurs. Even patients who have experienced remission after primary treatment will most likely have to undergo further surgeries and additional advanced treatment with chemotherapy. Hopefully, the new clinical trials and research will soon discover a cure for primary peritoneal cancer.
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