What Is Pseudomyxoma Peritonei? Symptoms, Causes, Diagnosis & Treatment

Written by Medicover Team and Medically Reviewed by Dr Kovvuru Bhaskar Reddy , Oncologists

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1. Causes 2. Find a Doctor 3. Diagnosis 4. Treatment 5. FAQs

Pseudomyxoma peritonei is a clinical condition wherein mucin-producing tumour cells proliferate within the abdominal cavity, leading to the accumulation of mucinous ascites. This condition predominantly originates from appendiceal neoplasms but can also arise from ovarian, colorectal, and pancreatic origins.

Types of Pseudomyxoma Peritonei

Pseudomyxoma peritonei can be classified into different types based on tumour grade and biological behaviour.

Low-Grade PMP:

Low-grade PMP, also known as disseminated peritoneal adenomucinosis (DPAM), is characterized by a relatively indolent course and a better prognosis compared to high-grade PMP.

High-Grade PMP:

High-grade PMP, also known as peritoneal mucinous carcinomatosis (PMCA), exhibits more aggressive behaviour and a higher propensity for invasion and metastasis.

What Are the Symptoms of Pseudomyxoma Peritonei?

The symptoms of Pseudomyxoma Peritonei can vary depending on the extent of mucin accumulation and tumour spread within the abdominal cavity.

Abdominal Distension:

One of the hallmark symptoms of PMP is progressive abdominal distension caused by the accumulation of mucinous fluid within the peritoneal cavity.

Abdominal Pain:

Patients may experience varying degrees of abdominal pain, often exacerbated by the increasing pressure of the mucinous deposits on abdominal structures.

Changes in Bowel Habits:

Altered bowel movements, including constipation and diarrhoea, can occur due to the mechanical obstruction caused by the mucinous deposits.

Weight Changes:

Unintentional weight gain or loss can be observed in patients with PMP, often correlating with the extent of the mucinous accumulation.

Hernias:

The increased intra-abdominal pressure may lead to the development of hernias, particularly umbilical and inguinal hernias.

What Causes Pseudomyxoma Peritonei?

The causes of Pseudomyxoma Peritonei are associated with mucin-producing tumours that spread cells throughout the peritoneal cavity.

Appendiceal Neoplasms:

The most common cause of PMP is a mucinous tumour of the appendix, particularly a low-grade appendiceal mucinous neoplasm (LAMN). These tumours rupture and disseminate mucin-producing cells throughout the peritoneal cavity.

Ovarian Tumors:

Ovarian mucinous tumours are another potential source of PMP. These tumours may be primary or metastatic, often complicating the diagnostic process.

Other Origins:

In rare cases, PMP may originate from mucinous tumours of the pancreas or colon, further complicating its aetiology.

When Should You See a Doctor for Pseudomyxoma Peritonei?

If you experience persistent or worsening symptoms of Pseudomyxoma Peritonei, consult an oncologist. Early evaluation can help identify complications, guide treatment decisions, and improve outcomes. Prompt medical attention is important when symptoms affect daily activities, nutrition, or overall health.

You should see a doctor if you have:

Persistent abdominal swelling or abdominal pain.
Changes in bowel habits or unexplained weight changes.
Development of hernias or worsening digestive symptoms.

Get medical help immediately if:

Severe abdominal pain or bowel obstruction symptoms.
Difficulty breathing due to abdominal pressure.
Rapid worsening of symptoms requiring hospitalization.

These could be signs of a serious complication like Pseudomyxoma Peritonei, which needs urgent care.

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How is Pseudomyxoma Peritonei Diagnosed?

Diagnosing Pseudomyxoma Peritonei involves imaging studies, laboratory investigations, and tissue sampling to confirm the condition.

Radiology and Imaging:

CT Scans:

Computed tomography (CT) scans are instrumental in diagnosing PMP. They provide detailed images of the mucinous deposits and their distribution within the peritoneal cavity.

MRI:

Magnetic resonance imaging (MRI) can be utilized to characterize the mucinous tumours further and assess their relationship with adjacent structures.

Biopsy:

A percutaneous or surgical biopsy of the mucinous deposits is often necessary to confirm the diagnosis and determine the tumour's histological subtype.

Serum Markers:

Elevated levels of tumour markers such as carcinoembryonic antigen (CEA) and cancer antigen 19-9 (CA 19-9) may support the diagnosis but are not definitive.

What Are the Treatment Options for Pseudomyxoma Peritonei?

The treatment of Pseudomyxoma Peritonei focuses on removing tumour deposits, controlling disease progression, and improving quality of life.

Cytoreductive Surgery (CRS):

Cytoreductive surgery aims to remove as much of the visible tumour burden as possible. This often involves extensive surgical procedures, including peritonectomy and organ resection.

Hyperthermic Intraperitoneal Chemotherapy (HIPEC):

Following cytoreductive surgery, HIPEC is employed to deliver heated chemotherapy directly into the peritoneal cavity. This technique enhances the penetration of chemotherapeutic agents into the residual tumour cells, improving treatment efficacy.

Systemic Chemotherapy:

Systemic chemotherapy may be used as an adjunct to surgical interventions, particularly in cases of high-grade PMP or when complete cytoreduction is not achievable.

Palliative Care:

In advanced cases where curative treatment is not feasible, palliative care focuses on symptom management and improving quality of life. This may involve drainage of ascites, pain management, and nutritional support.

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What is the Prognosis for Pseudomyxoma Peritonei?

The prognosis of Pseudomyxoma Peritonei depends on tumour grade, disease extent, and response to treatment.

With Surgery:

The prognosis of PMP significantly improves with timely and appropriate surgical intervention. Patients undergoing complete cytoreduction and HIPEC have a better overall survival rate.

Without Surgery:

Without surgical intervention, the prognosis of PMP is generally poor. The progressive accumulation of mucinous ascites leads to severe complications, including bowel obstruction, respiratory difficulties, and, ultimately, a significant reduction in life expectancy.

How Can Pseudomyxoma Peritonei Be Prevented?

Early detection and monitoring of associated tumours may help identify Pseudomyxoma Peritonei at an earlier stage.

Regular Monitoring:

Individuals with a known history of appendiceal or ovarian mucinous tumours should undergo regular monitoring and imaging to detect any early signs of PMP.

Genetic Counseling:

For those with a family history of mucinous tumours, genetic counseling and testing may help identify potential risks and facilitate early intervention.

Symptom Awareness:

Raising awareness about the symptoms of PMP among at-risk populations can lead to earlier diagnosis and improved treatment outcomes.

Frequently Asked Questions

1. What are the symptoms of pseudomyxoma peritonei?

Symptoms may include abdominal swelling, abdominal pain, bloating, nausea, changes in bowel habits, loss of appetite, and increasing abdominal girth due to mucin accumulation.

2. What causes pseudomyxoma peritonei?

Pseudomyxoma peritonei is most commonly caused by a ruptured mucin-producing tumor of the appendix that spreads mucus-producing cells throughout the abdominal cavity.

3. How is pseudomyxoma peritonei treated?

Treatment usually involves cytoreductive surgery to remove visible tumor deposits, followed by Hyperthermic Intraperitoneal Chemotherapy (HIPEC) to target remaining cancer cells.

4. What is the life expectancy without surgery?

Without treatment, pseudomyxoma peritonei can progressively worsen and may become life-threatening due to organ compression, bowel obstruction, and other complications.

5. How is pseudomyxoma peritonei diagnosed?

Diagnosis typically involves CT scans, MRI scans, biopsy, pathological examination, and sometimes analysis of abdominal fluid to identify mucin-producing tumor cells.

6. What is the survival rate of pseudomyxoma peritonei cancer?

Survival rates vary depending on the tumor grade, extent of disease, and treatment received. Many patients achieve long-term survival after successful cytoreductive surgery and HIPEC.

7. What are the first signs of pseudomyxoma peritonei?

Early signs often include abdominal bloating, increasing abdominal size, mild abdominal discomfort, unexplained weight changes, and digestive symptoms.

8. How do you treat pseudomyxoma peritonei?

The standard treatment is cytoreductive surgery combined with HIPEC. Additional treatments may be considered depending on disease severity and individual patient factors.

9. Is pseudomyxoma peritonei fatal?

It can be fatal if left untreated. However, advances in surgery and HIPEC have significantly improved outcomes and long-term survival for many patients.