Pseudomyxoma Peritonei – Signs, Causes, Diagnosis & Treatment

Overview

Pseudomyxoma Peritonei: It develops in your body as a slow-growing tumor in the appendix, also called as LAMN (Low-grade mucinous appendiceal neoplasm). Pseudomyxoma Peritonei (PMP) affects the other parts of your body such as

• Bowel
• Bladder or ovary

Mucin is a jelly-like substance produced by the tumor during the phase of this disease. Mucin can make your appendix to swell up like a balloon. The tumor cells will spread through the lining of your tummy as it will break through the wall of the appendix. You will experience symptoms and pressure on the bowel due to the excessive tumor cells and mucin build up in your tummy. This condition is very difficult to diagnose and medical professionals conduct lots of tests to identify this disease in the human body. It is rare cancer and spreads to your body through the lymphatic system or the bloodstream.

Primary malignant neoplasms of the peritoneum are rare as most malignant neoplasms are metastasis originating from carcinomas of the gastrointestinal tract especially the stomach colon and pancreas or the genital urinary tract particularly epithelial ovarian tumors. Pseudomyxoma peritonei or PMP is characterized by the accumulation of intraperitoneal mutinous societies commonly from a ruptured ovarian or appendiceal neoplasm. A mutinous lesion would grow and occlude the appendiceal lumen the appendix ruptures and the peritoneum is seeded with mucus producing cells that continue to proliferate.

Pseudomyxoma peritonei represents a spectrum of disease and can be thought of as two distinct groups. The first is diffused peritoneal adenomucosis (DPAM) characterized by intraperitoneal cellular mutant without tumor cells from a ruptured low grade appendiceal mucinous neoplasm formerly known as systematic adenoma were ruptured appendiceal mucocele. This carries a more indolent course and a better prognosis than peritoneal mucinous carcinomatosis characterized by parathion mutinous deposits with tumor cells from an underlying malignancy such as appendiceal adenocarcinoma with mucinous histology.

Patients are often asymptomatic until late in the course of their disease and will often describe a global deterioration in their health long before a diagnosis is made. Symptoms of abdominal pain and distension are common in the physical exam may reveal new hernia ascites and occasionally a palpable abdominal mass. Pseudomyxoma peritonei has a classic appearance on CT scan as intra-abdominal mucin causes scalping of the liver spleen edges and mesentery and have intermittent calcifications. In more aggressive cases segmental obstruction of the small bowel is also seen. The current treatment of patients with pseudomyxoma peritonei involves resection of as much of the tumor as possible with intensive cure or site of reduction and hyper-thermic intraperitoneal chemotherapy or HIPEC.

Symptoms of Pseudomyxoma Peritonei

The problem with this condition is the people who are suffering from this disease may not show any symptoms of the disease. This condition affects women more than men and It is in the ratio of 9:1. It is very difficult to diagnose this condition as people don’t show any symptoms.

Redness, swelling of the abdomen, irritation, and pain in the abdomen are the main symptoms of pseudomyxoma peritonei. The main symptom of pseudomyxoma peritonei is regarded as increased size of the abdomen. It happens to your body due to the following conditions. These include

• Neoplasm
• Renal abnormality or ascites

If the tumor keeps growing in your body, then it will result in an increased size of the abdomen or the regions that are specific to the abdomen. The tumor gets bigger due to the surface of the peritoneum as it can produce a liquid called peritoneal fluid by lining the abdominal walls with the help of the set of tissues. In order to put an end to the growth of your tumor, you need to reduce the amount of peritoneal fluid. This is not what you can do and that is why we would advise people to consult their healthcare professional immediately.

Ascites is a condition where the quantity of peritoneal fluid raises with respect to the growth of the tumor. If your body is absorbing more peritoneal fluid and it will ultimately result in the growth of the tumor. It can also lead to the formation of abdominal girth. An increased amount of peritoneal fluid may affect your internal organs in your body such as

• Reproductive organs
• Digestive organs
• Urinary system organs
• Biliary system or gastrointestinal system

People who are suffering from this condition may also experience severe abdominal pain. A person may encounter the following problems due to the damage of his internal organs in the body. These include

• Hernia
• Constipation
• Respiratory insufficiency
• Loss of weight
• Infertility
• Nausea and vomiting

What are the causes of pseudomyxoma peritonei?

The main cause of pseudomyxoma peritonei remains unknown. But doctors are taking lots of points into consideration. People always thought that hereditary and ecological factors contribute to this condition but there is no evidence to prove this point. The tumor grows due to none of the above factors. These factors are the main cause of most of the cancers and different types of tumors can contribute to the cause of this condition. However, an appendix is the origin of this condition as per the medical professional’s point of view.

As the tumor cells entered into your body through the appendix, from there it will affect the other areas related to the abdominal cavity and pelvis. The tumor cells grow bigger and covers the areas within the abdominal cavity and pelvis and leads to the formation of peritoneal fluid. With the help of this liquid, it can produce mucinous ascites. It can increase the size of the latter due to the aggregation of mucin present in the abdomen. In order to find the profile of PMP, health professionals will increase the circumference of the abdomen and also checking the mucus in the abdominal cavity.

There are lots of theories to find out the causes of pseudomyxoma peritonei condition. Medical professionals check the patient’s medical records, experts and researchers concluded that this condition can originate from other organs except for appendix. A pseudomyxoma can originate from

• Urachal
• Colon
• Ovary
• Stomach and other digestive system organs

Diagnosis of Pseudomyxoma Peritonei

It is not an easy task for health professionals to diagnose this medical condition. Certain tests are conducted to detect this condition or to check the development of this condition in the human body. This condition is used to diagnose by the doctors while performing an operation or treating other conditions to the patients.

Doctors recommend tests to the patients based on the condition of the person. In some cases, a series of tests may need to detect this problem in the human body. The following are the various tests recommended by the doctors to their patients to diagnose the PMP condition.

CT Scan

The full form of CT is Computed tomography and it provides vital information to the doctors about the inner parts of the human body. This scan displays a 3D image and doctors can detect mucin and its developing stage. Doctors can know the severeness of the disease with the help of this test. This scan takes ten to thirty minutes to provide an image of the inner part of the body. Patients don’t need to panic while taking this test as it is a pain-free technique and also a little quantity of radiation is used for scanning.

People who are going to take this scan should not eat before four hours of the scan. In order to investigate more, the doctors may inject a dye into your veins that will help to highlight structures of interest. People who are suffering from conditions like asthma and iodine allergy may not require a dye injection as it may lead to dangerous interactions.

Ultrasound Scan

It is also known as sonography and it provides the necessary information to the doctors by taking pictures of the inner part of the abdomen like

• Small intestine
• Large intestine
• Kidneys
• Liver
• Spleen

There is no need to use radiation to perform this test but it uses sound waves. The first step in this test is to apply the ultrasound gel on the abdomen. With the help of a transducer, they introduce high-frequency sound waves into the abdomen through the gel and the transducer collects the sound waves that are reflected from the abdomen. These sound waves will be converted into pictorial representation by using a computer.

Biopsy

It is an important test and it gives a clear picture of the cell from which the tumor has progressed. After that, your physician may conduct a group of tests to identify the other tumor cells. Some patients may need various types of biopsy tests to get clarification on the tumor. Ultrasound-guided biopsy, surgical biopsy, core needle biopsy, cone biopsy, excision biopsy, and a fine needle aspiration biopsy are the various types of biopsy tests.

Blood tests

Your physician may recommend a few blood tests in order to know the levels of tumor markers that are present in the blood. Your body cells produce tumor markers at the benign or malignant stage.

How to treat this condition?

When compared to other types of cancer, these tumor cells grow very slowly and don’t show their symptoms. The main objective of the treatment is to eliminate the unusual tumors present in the abdomen. Based on the severeness of the patient’s condition and the number of tumor cells present inside of the abdomen, your doctor recommends treatment options to treat this condition.

Observant waiting

As the pseudomyxoma peritonei tumor cells grow slowly, your physician may not prefer surgery right after diagnosing the condition. Due to this reason, we would advise you to consult your physician regularly. They might recommend surgery if your body showing the symptoms of this condition. The main objective of your healthcare provider is to monitor your condition regularly and controlling it.

Surgery

Your physician may recommend surgery to your condition depending on the severeness of your disease. If the level of the tumor keeps growing inside the abdomen, then you need to make an appointment with your doctor for surgery. A pathologist will be with you during the surgery and he or she will remove the lining of the peritoneum and also removes the unusual tumor or tissues present inside the abdomen that is affected by the pseudomyxoma peritonei. Cytoreductive surgery with hyper-thermic intraperitoneal chemotherapy and debulking surgery are the two types of surgeries to treat tumors.

Chemotherapy

People should opt for chemotherapy if they have not had cytoreductive surgery. The main objective of this therapy is to ease the symptoms of the PMP and reduce the development of this condition. There are various in chemotherapy, it is given through the tablet capecitabine and in some people through an injection (mitomycin C) into the vein. Some of the side effects associated with chemotherapy, and due to this reason we would advise you to take regular tests and scans to your body and make appointments with your physician once in a month.

References

https://rarediseases.info.nih.gov/diseases/7488/pseudomyxoma-peritonei
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2999154/
https://pmj.bmj.com/content/78/917/170