From the Research
The recommended next step for this 47-year-old woman with progressive malignant pseudomyxoma peritonei (PMP) from appendiceal mucinous adenocarcinoma is to proceed with cytoreductive surgery (CRS) combined with hyperthermic intraperitoneal chemotherapy (HIPEC). Despite showing disease progression after both FOLFOX and FOLFIRI with bevacizumab regimens, CRS-HIPEC remains the definitive treatment option for PMP with peritoneal metastasis. The surgery would involve complete removal of all visible tumor deposits throughout the peritoneal cavity, followed by HIPEC using mitomycin C or oxaliplatin to target microscopic disease. This approach is particularly appropriate since imaging confirms the disease remains confined to the peritoneum without distant metastases to liver or lungs. The patient should be counseled about the extensive nature of the surgery, potential for multi-organ resection, and the approximately 30-60% five-year survival rates for this condition when treated with CRS-HIPEC. Following surgery, additional systemic chemotherapy might be considered based on surgical findings and pathology results. Given the poor response to standard chemotherapy regimens, molecular profiling of the tumor tissue could also be considered to identify potential targeted therapy options if CRS-HIPEC is unsuccessful or not feasible. Some studies have explored alternative chemotherapy regimens, such as the combination of oxaliplatin, irinotecan, and capecitabine with bevacizumab 1, but these are not directly applicable to the current scenario where CRS-HIPEC is the primary treatment consideration. Other studies have investigated the safety and efficacy of various combination therapies, including S-IROX 2 and CAPOXIRI plus bevacizumab 3, but these do not alter the recommendation for CRS-HIPEC in this case. The key consideration is that CRS-HIPEC offers a potential cure for PMP, and its benefits in terms of morbidity, mortality, and quality of life outweigh those of continued systemic chemotherapy alone.