Pseudomyxoma Peritonei

Pseudomyxoma peritonei (PMP) is an uncommon form of cancer that impacts the peritoneal cavity, encompassing the abdomen and pelvis. Its name, literally translating to “false mucinous tumor of the peritoneum,” originates from its unique characteristic of not forming solid tumors. Instead, it proliferates by continuously generating mucin, a gelatinous substance found in mucus. Over time, this mucin accumulates in the peritoneal cavity, leading to the colloquial term “jelly belly” for this condition.

This cancer typically originates as a polyp, often developing on the inner lining of the appendix. While some research suggests that it may arise in other organs, such occurrences are rare. The cancer cells secrete mucin, causing the appendix to enlarge and eventually rupture, allowing mucin to disseminate throughout the peritoneal cavity.

Unlike conventional cancers, PMP doesn’t spread through the bloodstream or lymphatic system. However, it can extend to the surface of various abdominal and pelvic organs, complicating treatment. However, complete removal of all affected tissue including organs, is necessary for curing PMP.

Causes

Pseudomyxoma peritonei originates from mucinous tumors, most commonly from the appendix. These tumors can rupture, releasing mucinous material into the peritoneal cavity. Other origins include ovarian tumors and, rarely, tumors in the gastrointestinal tract.

Types of Pseudomyxoma Peritonei

PMP is categorized into two main types:

1. Disseminated Peritoneal Adenomucinosis (DPAM): This benign variant, although non-cancerous, can still pose serious or even fatal consequences if left untreated.
2. Peritoneal Mucinous Carcinomatosis (PMCA): In this type, tumor cells demonstrate malignant characteristics, indicating cancerous growth.

Differentiating between these types is crucial for determining appropriate treatment strategies and prognoses.

Prevalence

PMP is considered extremely rare, with an estimated incidence of less than one case per million individuals annually. However, due to its slow-growing nature and often asymptomatic presentation in the early stages, it may be underdiagnosed.

Symptoms of Pseudomyxoma peritonei

Initially, individuals with pseudomyxoma peritonei (PMP) may not exhibit any symptoms. However, as the condition progresses, the following signs may manifest:

• Abdominal pain
• Alterations in bowel habits
• Enlargement of the ovary in females
• Presence of a hernia, characterized by a bulge near the groin
• Decreased appetite
• Abdominal distension or swelling
• Weight gain or an increase in waist size

Risk Factors

Risk factors for developing pseudomyxoma peritonei include a history of appendiceal or ovarian tumors, as well as certain genetic syndromes such as familial adenomatous polyposis (FAP) and Muir-Torre syndrome.

Diagnosing Pseudomyxoma peritonei

Diagnosing PMP can be challenging due to its similarity to other diseases. In some cases, it may be incidentally discovered while investigating unrelated medical concerns.

To confirm the presence of PMP, your doctor will conduct a thorough examination and inquire about your symptoms. Subsequently, imaging studies will be performed to visualize the abdominal region and other relevant areas of the body.

Common diagnostic techniques for PMP include:

• Computed tomography (CT) scan: This imaging procedure utilizes X-rays from various angles to generate detailed cross-sectional images of the abdomen. Contrast material may be administered orally or intravenously to enhance image clarity.
• Magnetic resonance imaging (MRI) scan: By employing powerful magnets and radio waves, MRI scans produce high-resolution images of bodily structures without using radiation.
• Ultrasound scan: Utilizing sound waves, ultrasound scans create images of internal organs, aiding in the detection of abnormalities.
• Laparoscopy: During this minimally invasive procedure, a thin, flexible instrument with a camera is inserted through a small incision in the abdominal wall, allowing direct visualization of the abdominal cavity.
• Biopsy: Involves obtaining a small tissue sample for microscopic examination to confirm the presence of abnormal cells characteristic of PMP.

These diagnostic modalities enable healthcare providers to accurately identify PMP and devise appropriate treatment plans tailored to individual needs.

Treatments

The treatment approach for pseudomyxoma peritonei (PMP) is determined by factors such as tumor type (cancerous or noncancerous), tumor size, and overall health status. Treatment options may include:

1. Observation: For small, slow-growing tumors, a “watch and wait” approach may be recommended. Regular monitoring allows healthcare providers to assess tumor progression.
2. Debulking Surgery: This surgical procedure aims to remove as much of the tumor mass as feasible. Although debulking surgery does not provide a cure for PMP, it can alleviate symptoms and improve overall well-being.
3. The Sugarbaker technique: This gold standard treatment for PMP is potentially curative but is highly intensive and carries significant risks. The procedure involves the extensive removal of the peritoneal lining and affected tissues (cytoreductive surgery or CRS), followed by the administration of heated chemotherapy drugs directly into the abdominal cavity (hyperthermic intraperitoneal chemotherapy or HIPEC) to eliminate any remaining cancer cells. Subsequently, additional chemotherapy, including fluorouracil, may be delivered to eliminate residual tumor cells. While CRS with HIPEC is a complex and lengthy procedure, it offers the potential for a cure in select cases of PMP.
   • Surgery may include various procedures such as peritonectomy, omentectomy, bowel resection, splenectomy, cholecystectomy, liver capsulectomy, and hysterectomy with bilateral salpingo-oophorectomy. The operation typically lasts 10 to 14 hours, with HIPEC administered to ensure thorough treatment.
4. Chemotherapy: In cases where cytoreductive surgery is not feasible, chemotherapy may be utilized as a primary treatment modality. Chemotherapeutic agents are administered intravenously to target cancer cells and inhibit tumor progression.

The choice of treatment modality depends on individualized factors such as tumor characteristics, disease stage, and overall health status. Collaborative decision-making between patients and healthcare providers ensures the selection of the most appropriate treatment strategy tailored to individual needs.

Home Care Tips

Patients need to follow their healthcare provider’s instructions for postoperative care after surgery and chemotherapy. This may include:

• Dietary modifications
• Activity restrictions
• watch out for signs of infection or complications.
• Maintaining a healthy lifestyle
• Regular exercise
• Balanced diet

These tips can support overall recovery and well-being.

Side Effects of Treatment for Pseudomyxoma Peritonei

Cytoreductive Surgery:

1. Paralytic Ileus: Bowel movement may be sluggish or absent following surgery.
2. Peripheral Neuropathy: Nerve damage may cause tingling or numbness in affected areas.
3. Blood Clots: Prolonged surgery duration increases the risk of blood clots.
4. Anastomotic Leak: Leakage at the site where two parts of a channel are sewn together.
5. Septicemia and Sepsis: Infection of peritoneal fluid may spread to the bloodstream, leading to systemic infection.
6. Pleural Effusion: Accumulation of fluid in the tissue surrounding the lungs.

Chemotherapy:

1. Neutropenia: Reduced levels of immune cells increase susceptibility to infections.
2. Mucositis: Inflammation of the mucous lining of the gastrointestinal tract.
3. Nausea and Vomiting: Temporary side effects that may lead to poor appetite and undernutrition.
4. Fatigue: Recovery from treatment may take time, resulting in persistent fatigue.

Prevention

While there are no specific measures to prevent pseudomyxoma peritonei, early detection and treatment of appendiceal or ovarian tumors may reduce the risk of tumor rupture and dissemination of mucinous material within the peritoneal cavity. Regular medical check-ups and screening tests may aid in early detection and intervention.

When to See a Doctor

Individuals experiencing persistent abdominal symptoms such as distension, pain, or changes in bowel habits should seek medical evaluation, especially if there is a history of appendiceal or ovarian tumors. Prompt diagnosis and treatment can improve outcomes and quality of life for patients with pseudomyxoma peritonei.

Outlook/Prognosis

The prognosis for pseudomyxoma peritonei varies depending on factors such as the extent of tumor spread, response to treatment, and overall health of the individual. With advances in surgical techniques and multimodal treatment approaches, many patients experience long-term survival and improved quality of life following treatment.

In conclusion, pseudomyxoma peritonei is a rare abdominal condition that requires prompt diagnosis and comprehensive treatment. By understanding its causes, symptoms, risk factors, diagnosis, treatments, home care tips, prevention strategies, and when to seek medical attention, individuals can take proactive steps to manage this condition effectively.

Sources

• National Organization for Rare Disorders. Pseudomyxoma Peritonei.
• Pseudomyxoma Survivor. What is Pseudomyxoma Peritonei?
• PMP Awareness Organization
• Cancer Council. Pseudomyxoma Peritonei (PMP) 
• Cancer Research UK. About pseudomyxoma peritonei
• MacMillan Cancer Support. Pseudomyxoma Peritonei (PMP). 
• NICE. Complete cytoreduction for pseudomyxoma peritonei (Sugarbaker technique).
• NORD. Pseudomyxoma Peritone