Abstract
Pseudomyxoma peritonei (PMP) is a rather uncommon syndrome in oncology with a unique biological behavior and an estimated incidence of one to two cases per million per year. Clinically, it usually presents with a variety of unspecific signs and symptoms including abdominal pain and distention, ascites, or even bowel obstruction. Despite its intimidating clinical manifestation, PMP is characterized by satisfactory survival rates when treated with cytoreduction and hyperthermic intraperitoneal chemotherapy (HIPEC). We present two interesting cases of PMP deriving from the appendix with a rather atypical presentation, which was successfully treated with cytoreduction and HIPEC. In addition, we intend to raise clinical suspicion on the diagnosis of PMP and comment on several challenging issues concerning the origin and classification of PMP.