What is pseudomyxoma peritonei (PMP)?
It is a rare but serious disease that develops when a mucous (mucinous) tumor spreads within the abdominal cavity, creating thick clumps of mucus that can put pressure on surrounding organs. The most common source is a tumor that originates in the appendix, but it can also originate in the ovaries, bladder, or other parts of the digestive tract.
Symptoms
PMP often develops slowly, and symptoms are mostly nonspecific:
Increase in abdominal circumference
Bloating, discomfort or abdominal pain
Constipation or changes in digestion
Loss of appetite
Unexplained weight loss
In women – often discovered accidentally during gynecological surgeries
Making a diagnosis
The diagnosis is established by a combination of imaging and surgical methods:
Ultrasound and CT/MRI of the abdomen – reveal mucus accumulation and enlargement of the abdominal cavity
Tumor markers: CEA, CA 19-9, CA-125 may be elevated. More about tumor markers.
Laparoscopy or laparotomy – to take a sample and confirm the diagnosis
Treatment
Surgical treatment
Cytoreductive surgery aims to remove all visible accumulations of mucus and tumor tissue. Surgeons often remove affected parts of the peritoneum, intestines, ovaries, spleen, or gallbladder, depending on the extent of the disease. You can read more about surgical treatment in a separate article.
HIPEC (hyperthermic intraperitoneal chemotherapy)
Immediately after surgery, heated chemotherapy (usually mitomycin C, cisplatin, or oxaliplatin) is injected into the abdominal cavity. The high temperature (around 42°C) allows the chemotherapy to penetrate the tissues more effectively and destroy any remaining tumor cells. HIPEC is administered locally, so side effects are fewer than with systemic chemotherapy.
Systemic chemotherapy
In some cases, especially in more aggressive (so-called high-grade) forms of PMP and if the disease is so widespread that surgery is not possible, systemic chemotherapy via infusion is also used. The most common combinations of drugs used are:
FOLFOX (5-FU, Leucovorin, Oxaliplatin)
FOLFIRI (5-FU, Leucovorin, Irinotecan)
Sometimes targeted therapy (e.g. bevacizumab)
The decision on chemotherapy is made by a multidisciplinary team based on the histological findings, the patient's general condition, and the extent of the disease. You can read more about chemotherapy in a separate article.
Follow-up after treatment
Regular check-ups (clinical, laboratory, and imaging) every 3–6 months for the first few years
Monitoring tumor markers
Possibility of reoperation if the disease returns
Conclusion
Pseudomyxoma peritonei is a disease that requires a specialized and individualized approach. Although it is rare, effective treatment methods exist today that significantly prolong life and improve its quality. Treatment in centers with experience in treating this disease, especially with the use of surgery with HIPEC, is crucial.
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