Clinicopathologic characteristics and molecular landscape for pseudomyxoma peritonei of appendiceal origin

Pseudomyxoma peritonei (PMP) of appendiceal origin presents a unique challenge in oncology owing to its rarity, varied progression, and complex molecular characteristics. The current standard treatment using cytoreductive surgery combined with hyperthermic intraperitoneal chemotherapy has shown some success in extending survival rates; however, it is associated with significant physical burdens and high recurrence rates. Advances in understanding the molecular and genetic features, particularly mutations in genes such as KRAS, GNAS, and BRAF, have led to a promising shift toward more targeted and less invasive treatment options, potentially moving beyond the limitations of current therapies. Additionally, novel approaches targeting the tumor microenvironment in PMP have gained traction, given the role of factors such as mucin accumulation, hypoxia, and inflammatory cytokines in sustaining tumor persistence and resistance. Emerging personalized therapies and immunotherapies, particularly targeted treatments and peptide vaccine development for GNAS-mutated cancers, offer the potential to enhance treatment specificity and effectiveness. The challenges of multiomics approaches to capture the complex genomic and epigenomic landscape of PMP highlight the need for individualized optimal treatment in patients with PMP.