Pseudomyxoma peritonei natural history, complications and prognosis
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Parminder Dhingra, M.D. [2]
Overview
Pseudomyxoma peritonei is a rare disease characterized by the accumulation of mucinous ascites and mucinous tumor disseminated in the peritoneal cavity, mainly abdomen and pelvis; the disease almost always 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).
Natural History
- Pseudomyxoma peritonei (PMP) is an uncommon disease characterized by mucinous ascites in intraperitoneal cavities, it is 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, and infertility due to compression impact of tumor on visceral organs.
- Psuedomyxoma peritonei tumour produces abundant mucins which accumulate because of to gravity effect in the mesentery, the greater and lesser omentum, and the under the diaphragm, around the liver and other visceral organs surfaces.
- The optimal treatment is cytoreductive surgery (CRS) combined with hyperthermic Intraperitoneal chemotherapy (HIPEC).[1]
Prognosis
- Prognosis of pseudomyxoma peritonei is generally good because of change in treatment modalities, mainly surgery techniques which developed by Sugarbaker. Traditional debulking surgery replaced by cytoreductive surgery combined with hyperthermic intraperitoneal chemotherapy(HIPEC).[2]
- Although pseudomyxoma peritonei is a neoplastic disease, it runs in a chronic, an indolent course with late invasion and only rare metastasis outside the peritoneum.
- Biological features of the tumor and access to the current standard of care at specialized oncology centers with a peritoneal surface malignancy program comprise the most important prognostic determinants of pseudomyxoma peritonei.
References
- ↑ Mittal R, Chandramohan A, Moran B (August 2017). "Pseudomyxoma peritonei: natural history and treatment". Int J Hyperthermia. 33 (5): 511–519. doi:10.1080/02656736.2017.1310938. PMID 28540829.
- ↑ Sugarbaker PH (April 2001). "Cytoreductive surgery and peri-operative intraperitoneal chemotherapy as a curative approach to pseudomyxoma peritonei syndrome". Eur J Surg Oncol. 27 (3): 239–43. doi:10.1053/ejso.2000.1038. PMID 11373099.