Pseudomyxoma peritonei natural history, complications and prognosis

Pseudomyxoma peritonei is a rare disease characterized by the accumulation of mucinous ascites and mucinous tumor disseminated in the peritoneal cavity, mainly the abdomen and pelvis. The disease mainly originates from primary appendiceal tumors and less commonly from the ovary and colon. In 2010, WHO published a classification which divides pseudomyxoma peritonei (PMP) into low and high grades. Cytoreductive surgery with hyperthermic intraperitoneal chemoperfusion (CRS/HIPEC) is the mainstay of treatment appendiceal pseudomyxoma peritonei (PMP).

• Pseudomyxoma peritonei (PMP) is an uncommon disease characterized by mucinous ascites in intraperitoneal cavities, originating from a low-grade mucinous neoplasm of the appendix. If left untreated, patients with pseudomyxoma peritonei may progress to develop abdominal or pelvic pain, bloating, abdominal distension, weight change, pelvic mass and infertility due to compression impact of tumor on visceral organs.^[1]
• The tumor produces abundant mucins which accumulate because of gravity effect in the mesentery, the greater and lesser omentum, under the diaphragm, around the liver and other visceral organ surfaces.^[2]
• The optimal treatment is cytoreductive surgery (CRS) combined with hyperthermic intraperitoneal chemoperfusion (HIPEC).^[3]

• Prognosis of pseudomyxoma peritonei is generally good because of change in treatment modalities, traditional debulking surgery has been replaced by cytoreductive surgery combined with hyperthermic intraperitoneal chemoperfusion (HIPEC).^[4]
• According to WHO classification, pseudomyxoma peritonei (PMP) is divided into disseminated peritoneal adenomucinosis (DPAM) and peritoneal mucinous carcinomatosis (PMCA), with DPAM type having better prognosis with higher survival rate based on their histological morphology. In contrast, tumors arising from colorectal neoplasm are usually high grade with a bad prognosis.^[5][6][7]

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