What is pseudomyxoma peritonei?
Pseudomyxoma peritonei is a rare condition in which the abdomen fills with a gelatinous substance called mucin. This mucin is produced by tumor cells that usually originate in the appendix.
It is also known as "jelly belly" due to the characteristic appearance of the gelatinous substance that accumulates in the abdomen.
Origin and types
Origin
In most cases, pseudomyxoma originates from:
- Appendix: Most common origin (mucinous tumors of the appendix)
- Ovary: Less frequent
- Other locations: Rarely, colon or other organs
Types according to grade
- Low-grade: Slow-growing cells, better prognosis
- High-grade: More aggressive cells, requires more intensive treatment
The tumor grade is fundamental for prognosis and treatment planning.
Symptoms
Symptoms develop gradually as mucin accumulates:
- Progressive abdominal distension
- Feeling of fullness
- Abdominal discomfort or pain
- Digestive changes
- Weight loss
- In women, sometimes discovered during gynecological surgery
Many patients are diagnosed incidentally during surgery for another reason.
Diagnosis
Diagnosis is usually based on:
- CT scan: Shows characteristic appearance of mucin in the abdomen
- MRI: Can provide additional details
- Tumor markers: CEA and CA 19-9 may be elevated
- Surgical confirmation: Often final diagnosis is made during surgery
Treatment
Standard treatment is the combination of:
Cytoreductive surgery (CRS)
The goal is to remove all visible disease:
- Removal of mucin and affected peritoneum
- Appendectomy (if not already done)
- Removal of affected organs or parts of organs
- Complete cytoreduction is crucial for the best outcomes
HIPEC
Heated intraperitoneal chemotherapy:
- Applied during surgery after tumor removal
- Treats microscopic residual disease
- Standard treatment in most centers
What does surgery involve?
Surgery for pseudomyxoma can be extensive and may include:
- Peritonectomy (removal of peritoneum)
- Removal of the omentum
- Splenectomy
- Resection of intestinal segments
- In women, removal of uterus and ovaries
- Resection of liver capsule if affected
Duration and hospitalization
- Surgery duration: 6-12 hours depending on extent
- Hospitalization: 10-20 days
- Full recovery: 2-3 months
Prognosis
Pseudomyxoma peritonei, especially low-grade, has a favorable prognosis when treated properly:
Low-grade pseudomyxoma
- 10-year survival rates above 70-80%
- Many patients achieve long-term cure
- Slow progression if recurrence occurs
High-grade pseudomyxoma
- More reserved prognosis
- May require additional treatments
- More intensive follow-up
Follow-up
After treatment, regular follow-up includes:
- Periodic CT scans
- Tumor marker monitoring (CEA, CA 19-9)
- Clinical evaluation
- Long-term surveillance (disease can recur years later)
If recurrence occurs
In cases of recurrence:
- Repeat surgery may be possible
- Low-grade tumors progress slowly
- Good quality of life can be maintained
- Systemic chemotherapy in selected cases
A message of hope
Pseudomyxoma peritonei, despite its rare nature, is one of the peritoneal diseases with the best prognosis when treated properly. The combination of complete cytoreductive surgery and HIPEC offers excellent long-term results, especially in low-grade tumors. It is essential to be treated at a specialized center with experience in this disease.
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