Have you ever had stomach discomfort, a feeling of fullness, or persistent constipation? Sometimes we think these are normal things, but in rare cases, there may be another reason behind these symptoms. Today we are going to talk about a very rare, but very important cancer condition. That is a condition called `Pseudomyxoma Peritonei`, which we also call `(PMP)` for short.
What is Pseudomyxoma Peritonei (PMP)?
Simply put, `Pseudomyxoma Peritonei` is a very rare type of cancer. What happens in this is that the inside of your abdominal cavity, that is, the stomach and its surrounding area (`(peritoneal cavity)`) is filled with cancer cells. These cancer cells produce large amounts of a jelly-like, slimy substance called ``mucin`` . Imagine, it's like the inside of your stomach is filled with jelly! This is why some people also call this disease ``jelly belly.``
The name "Pseudomyxoma Peritonei" means something like "false mucinous tumor of the peritoneal cavity." Doctors call this a "false tumor" because it's not like other cancers that start in one place and spread. It actually starts with a small lump called a ``polyp'' that forms in your appendix, the appendix .
When this polyp comes out of the appendix, the cancer cells that make that jelly-like substance I mentioned start to spread throughout the stomach. Over time, this can affect your digestive system.
What are the symptoms we experience when we develop PMP?
The symptoms of PMP often come on very suddenly. You may not notice them at first, or you may think they are something normal, like constipation or indigestion. But if these symptoms persist or worsen, you should be concerned.
Here are some of the main symptoms:
- Abdominal distension: The abdomen appears noticeably swollen and enlarged.
- Abdominal pain: This jelly-like substance in the stomach can feel like a general discomfort or pain.
- Constipation: Bloating can block the large intestine and make it difficult to pass stool.
- Difficulty getting pregnant: For women in particular, it can be difficult to conceive a child due to the pressure caused by this jelly-filled uterus or inflammation of the reproductive organs.
- Hernias: Increased pressure inside the abdomen can cause one of your organs to push out through the muscles. In men, an inguinal hernia is a common symptom of PMP.
- The food is tasteless.
- Nausea.
Sometimes, a doctor may catch this `PMP` condition early on during your annual check-up or while testing you for another condition. But most people only find out about it when their stomach problems persist and get worse.
Why does this PMP occur? What is the reason?
In fact, experts still don't know exactly what chain of events leads to PMP. But they do know that it starts with a small growth called a polyp in the appendix. From there, the cancer cells that make the mucin travel into the stomach.
Remember, `PMP` is a very, very rare disease . It affects about one to four people in a million per year. So don't be unnecessarily scared by hearing about this.
How do doctors find out if they have PMP?
When you go to see a doctor, they'll ask you about your symptoms. How long have you had them, and whether they're getting worse? Then they'll do a physical exam. They'll also order blood tests and imaging tests.
Blood tests
Your doctor may order a complete blood count (CBC). They may also order blood tests to look for tumor markers. These are substances that can indicate that you have cancer in your body.
Imaging tests
Tests can be done to see if your abdominal organs are enlarged, filled with fluid (ascites), or have a buildup of mucin, like a "jelly belly":
- Abdominal ultrasound.
- A CT scan.
- An MRI scan.
In addition, a doctor may do a needle biopsy, which involves taking a small sample of fluid or tissue from inside your abdomen. A medical pathologist will examine the sample under a microscope (cytology). Sometimes a laparoscopy may be done to look inside your abdomen and take tissue samples.
What are the treatments for PMP? Can it be cured?
Treatment depends on many factors, including your condition and your overall health. In some cases, doctors can treat and even cure Pseudomyxoma Peritonei.
Most often, this treatment involves surgery. This involves removing all the organs that contain cancer cells. You may also be given chemotherapy (intraperitoneal chemotherapy) during the surgery. But the surgeon will only do this if the surgery can remove all or most of the cancer or the mucin. That decision is made at the time of surgery.
There are different types of surgeries to treat PMP.
For example, if you have mucin in your small intestine or large intestine, your doctor will perform a bowel resection . There are also other types of surgery that remove specific organs:
- Cholecystectomy : Removal of your gallbladder.
- Hysterectomy with bilateral salpingo-oophorectomy : Removal of your fallopian tubes, ovaries, and uterus.
- Liver capsulectomy: Removing part of the surface of your liver.
- Omenectomy: Removal of the fatty layer in front of the stomach .
- Peritonectomy : Removal of the membrane that lines the inside of your abdomen and pelvis (peritoneum).
- Splenectomy : Removal of your spleen.
What complications can occur during surgery?
When you have surgery to remove organs, there is always a chance of complications such as excessive bleeding , infection, or anastomotic leak . Major surgeries can also cause life-threatening complications. It is said that about one to two percent (1-2%) of patients die after major surgery. Therefore, it is important to discuss all of these with your doctor.
Are there other treatments that don't involve surgery?
Sometimes your doctor may recommend other non-surgical treatments, such as:
- Active surveillance : If you have early-stage PMP, or if it is a very aggressive form, and the risk of surgery is greater than the risk of the disease, your doctor may suggest active surveillance. This involves your doctor regularly monitoring your health. They may do imaging tests and blood tests to see if the PMP is spreading.
- Palliative care : If you are unable to have surgery, your doctor may recommend treatments such as chemotherapy or radiation therapy that can control the PMP condition and reduce symptoms.
Currently, doctors are also researching small bowel transplantation as a treatment for people with PMP in the small intestine.
What kind of future can someone with a PMP expect?
In some cases, surgery to remove all the cancer cells in the abdomen can help control the disease for a long time . Other treatments can help you live with the disease for a longer period of time.
When talking about the ``survival rate``, the most important thing to remember is that this is a very rare disease. This means that very few people develop it. Researchers are learning what they know about it from very small groups of people. If you have this disease, it is best to ask your doctor about this ``survival rate`` research and how it affects your condition.
How do I take care of myself? (Self-care)
If you have had surgery to remove cancer cells or to remove certain organs, it is important to remember that your body needs time and rest to heal. For example, if you have surgery to remove your spleen, your doctor will explain how this will affect you.
If you choose palliative care, you may have to undergo cancer treatments such as chemotherapy and radiation therapy for the rest of your life. It is important to stay strong and follow your doctor's instructions carefully.
What time should I see the doctor?
If you have `PMP`, you should definitely inform your doctor in the following cases:
- If complications occur after treatment. For example, if there is excessive bleeding after surgery, or if there are signs of infection in the surgical wound.
- If the side effects of treatment are worse than you expected.
- If there are symptoms of `Pseudomyxoma Peritonei` reappearing after surgery.
Finally, the most important message (Take-Home Message)
Pseudomyxoma Peritonei (PMP) is a very rare and challenging disease. Experts still don't know exactly why it develops. You may have it for a long time and not notice any major changes in your body. When symptoms do appear, they can be as simple as constipation or bloating.
So, it can be a shock to learn that your symptoms are caused by such a rare disease. It can be overwhelming to be told that the treatment will involve surgery to remove one or more organs in your abdomen. It can be hard to understand all of this at once.
But don't worry. Your doctors understand that you may have many questions – perhaps even the same questions several times. As you learn about your treatment options and consider your next steps, they want to hear everything that's on your mind. That's your right. Being in a good mental state also helps a lot with treatment.
` Pseudomyxoma Peritonei, PMP, cancer, abdominal cavity, mucin, jelly belly, appendix, surgery
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