Question

Outline its management options.

Hint:

Cytoreductive surgery combined with HIPEC offers the best long-term outcomes for patients with pseudomyxoma peritonei. Early detection and aggressive treatment are key to improving survival.

Answer 1

The management of pseudomyxoma peritonei (PMP) involves a multidisciplinary approach, including surgery, chemotherapy, and sometimes supportive care. The primary goal is to remove as much of the disease as possible, with the aim of controlling symptoms and improving survival.
Step 1: Surgical Management:
- Cytoreductive Surgery (CRS): The cornerstone of treatment for PMP is cytoreductive surgery, which involves the removal of all visible disease from the peritoneal cavity. This may include resection of involved organs, such as the appendix, ovaries, or colon, depending on the extent of the disease. The objective is to achieve a "complete cytoreduction," which means removing all tumor implants, leaving no visible disease.
- Peritonectomy: In cases where CRS cannot be performed, a peritonectomy (removal of the affected peritoneal lining) may be considered to reduce the tumor burden and relieve symptoms.
- Omentectomy: The omentum is often heavily involved in PMP, and its removal is an essential part of the surgical management.
Step 2: Hyperthermic Intraperitoneal Chemotherapy (HIPEC):
HIPEC is often performed immediately after CRS to deliver heated chemotherapy directly into the peritoneal cavity. The heat increases the penetration of chemotherapy drugs into the tumor cells, enhancing their cytotoxic effect. HIPEC is particularly effective for PMP, as the mucinous nature of the disease can make it difficult for systemic chemotherapy to reach the tumor cells effectively.
Step 3: Systemic Chemotherapy:
Systemic chemotherapy may be used in combination with surgery or HIPEC to target any remaining microscopic disease or metastasis. The most commonly used chemotherapy agents include a combination of 5-fluorouracil (5-FU) and mitomycin C. However, systemic chemotherapy is generally less effective in PMP compared to HIPEC.
Step 4: Supportive Care:
PMP can cause significant symptoms due to ascites and bowel obstruction. Supportive care measures, such as draining ascites and managing bowel function, are important aspects of treatment. Pain management and nutritional support are also critical for improving the patient’s quality of life.
Step 5: Prognosis and Follow-up:
The prognosis of PMP depends on the extent of the disease and the success of surgical resection. Patients who achieve complete cytoreduction and undergo HIPEC have a better long-term prognosis. Follow-up is essential for monitoring recurrence, which may occur months or even years after initial treatment. Regular imaging studies and clinical evaluations are important in detecting recurrence early.