Pseudomyxoma peritonei – a revisit: report of 2 cases and literature review

BACKGROUND Pseudomyxoma peritonei (PMP) is a rare, chronic, relapsing, diagnostically challenging and poorly understood disease characterized by disseminated mucinous ascites and peritoneal implants. CASE PRESENTATION We report two cases of PMP that represent the two biological variants of disseminated peritoneal adenomucinosis (DPAM)--the benign variant and the peritoneal mucinous carcinomatosis (PMCA)--the malignant variant, both of which were characterized by multiple relapses and progression of the disease despite aggressive management. CONCLUSION Even with a better understanding and recent advances in the management of these cases, PMP remains an enigmatic disease with a protracted clinical course characterized by multiple recurrences despite surgery and/or chemotherapy. Recognition of PMP as a delayed consequence years later should alert all surgeons to be extremely vigilant when treating mucinous neoplasms of the appendix, with special care being directed towards adequate excision and thorough debridement at the initial diagnosis.