17 August 2023 , 08:20
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Around 215 people are diagnosed with ‘Jelly Belly’ cancer every year in the UK, but symptoms of the rare disease are often misdiagnosed as another condition.
Officially titled pseudomyxoma peritonei (PMP), the tumour grows slowly and causes a build-up of mucin (a jelly-like substance) in the abdomen and pelvis. According to the Cleveland Clinic, the cancer begins as a polyp usually on the inside lining of the appendix, but it can also start in other organs such as the large bowel, an ovary or the bladder.
Dr. Sara Mesilhy, gastroenterologist at Royal College of Physicians, told the Mirror: “PMP is a very rare type of cancer that occurs in approximately two cases per million individuals per year. It starts as a small polyp and gradually spreads through the appendix wall, eventually housing cancerous cells in the lining of the abdominal cavity, known as the peritoneum.”
She added: “These cancerous cells produce mucus which collects in the abdomen as mucin, a jelly-like fluid. This gives it the "Jelly Belly" neoplasm name. The abdomen is full of this jelly substance.”
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Around 215 people are diagnosed with ‘Jelly Belly’ cancer every year in the UK (Getty Images/iStockphoto)How does PMP spread?
PMP doesn’t spread through blood or the lymphatic system, but it stays in the abdomen increasing in size. The Christie NHS Foundation Trust writes: “This can cause problems with moving and breathing and affects the body’s ability to absorb food properly.”
Dr. Mesilhy explained: “The other name for PMP is Low Grade Appendiceal Mucinous Neoplasm (LAMN). The reason for this name is that PMP is considered a borderline malignant condition. PMP doesn’t act like other cancers, as it only spreads to nearby organs, inside the abdomen.”
Symptoms of PMP
The average age of onset for PMP is 48 years, and in the start most people don’t have any symptoms. Dr Mesilhy said: “So, it can be difficult to diagnose before spreading to the abdomen. The slow nature of PMP might delay symptoms for up to several years. Because of this, it usually spreads beyond the appendix before diagnosis.”
When the cancer spreads and produces mucus that collects inside the abdomen, symptoms start to arise. Eventually accumulating, it presses on the bowel and other organs. Cancer Research UK highlights that PMP can often be confused with ovarian cancer, adding: “Ovarian cancer may also cause a swollen abdomen. Some types of ovarian cancer cells also produce mucin.”
The disease can be difficult to spot as some people won't have any symptoms, while others may experience a variety of red flags that can be easily misdiagnosed. According to Dr Mesilhy, these include:
Abdominal or pelvic pain
Not being able to become pregnant
Abdominal swelling and bloating
Changes in bowel habits
Hernia (a bulge in the tummy wall or groin)
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Loss of appetite
Feeling of fullness.
Meanwhile, Pseudomyxoma Survivor also reports general fatigue and bowel irritation as red flags of the disease. The condition is usually diagnosed after an operation to look into the abdomen, known as a laparotomy.
If you are experiencing any of the symptoms listed above, it is recommended that you visit a doctor as soon as possible.
What causes PMP?
The cause of PMP is unknown, and like many other tumours, this can develop in people who lead healthy lifestyles. According to the NHS, there is no clear association within families and it does not appear to be an inherited condition.
“According to multiple studies, it has been shown that PMP is not linked to any known genetic, familial, or environmental risk factors; which means that it doesn't run in families, and doesn't seem to be linked to anything in the environment,” says Dr. Mesilhy.
Abdominal swelling and bloating are common symptoms of PMP (Getty Images/iStockphoto)The doctor added: “Males and females are affected in equal numbers. The misconception that females develop it more frequently is wrong, this may come from mistaken diagnoses of some ovarian cancers such as PMP.”
News medical highlights that the disease is more likely to occur in those aged 59 and above. The health site writes: “A pre-existing neoplasm, either benign or malignant, in the appendix that is still alive and continues to develop in the abdomen, can cause PMP.”
How is PMP diagnosed?
It can be difficult to diagnose PMP and doctors sometimes find it by accident during treatment for other conditions, or during laparotomy. Dr Mesilhy explains that when diagnosing the disease, a doctor will do the examination and symptoms survey. Testing will take place before treatment for PMP, these include an ultrasound scan, CT scan and an MRI scan.
In some cases, patients will have a biopsy, which is when a doctor removes a small piece of tissue or cells. This is then examined by a pathologist who examines the tissue or cells under a microscope to look for signs of cancer. The main line of treatment includes surgery and chemotherapy.