What is pseudomyxoma peritonei?
Pseudomyxoma peritonei (PMP) is an unusual condition in which a mucinous cancer spreads within the abdominal cavity causing mucin (mucus) to accumulate. The cancer usually arises in the appendix, although other sites of origin are encountered occasionally. We’ll get to the primary appendix tumours that can produce PMP in a further page.
The abdominal cavity is also called the peritoneal cavity, because it is lined by a thin membrane called the peritoneum. This membrane covers the abdominal organs and the inside of the abdomen. The word peritonei reflects the fact that the peritoneum is involved in PMP.
PMP is different from other cancers that involve the abdomen in two main ways:
- It distributes itself widely through the abdominal cavity with the production of copious amounts of mucin
- It tends not spread to distant organs such as the lungs or bones (although there are exceptions)
The pathology of pseudomyxoma peritonei and appendix tumours
Pseudomyxoma Survivor and N Carr, MB BS FRCPath FRCPA FAcadMEd, Pseudomyxoma Survivor website, 2023
A Consensus for Classification and Pathologic Reporting of Pseudomyxoma Peritonei and Associated Appendiceal Neoplasia
Carr, Norman J. FRCPath; Cecil, Thomas D. MD; Mohamed, Faheez; Sobin, Leslie H.; Sugarbaker, Paul H.; González-Moreno, Santiago MD PhD; Taflampas, Panos MD; Chapman, Sara PhD; Moran, Brendan J. MD, A Consensus for Classification and Pathologic Reporting of Pseudomyxoma Peritonei and Associated Appendiceal Neoplasia, The American Journal of Surgical Pathology: January 2016 – Volume 40 – Issue 1 – p 14-26 doi: 10.1097/PAS.0000000000000535
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Complete cytoreduction for pseudomyxoma peritonei (Sugarbaker technique)
National Institute for Health and Care Excellence (NICE) Complete cytoreduction for pseudomyxoma peritonei (Sugarbaker technique), April 2004 [Online]. Available https://www.nice.org.uk/Guidance/ipg56 [Accessed February 2018]. Under review February 2018.
Cancer Research UK, Pseudomyxoma peritonei, 2016 [Online]. Available http://www.cancerresearchuk.org/about-cancer/pseudomyxoma-peritonei [Accessed February 2018].
Pseudomyxoma peritonei (PMP)
Macmillan, Pseudomyxoma peritonei (PMP), 2016 [Online]. Available https://www.macmillan.org.uk/information-and-support/pseudomyxoma-peritonei-pmp [Accessed February 2018].
Gastrointestinal stromal tumors in the appendix: A clinicopathologic and immunohistochemical study of four case
Miettinen M, Sobin LH (2001). Gastrointestinal stromal tumors in the appendix: A clinicopathologic and immunohistochemical study of four cases. The American journal of surgical pathology. Retrieved April 6, 2023, from doi:10.1097/00000478-200111000-00013. Accessed April 6, 2023.
Primary signet ring cell carcinoma of the appendix: a rare case report and our 18-year experience
Ko YH, Jung CK, Oh SN, et al (2008). Primary signet ring cell carcinoma of the appendix: a rare case report and our 18-year experience. World J Gastroenterol. 2008;14(37):5763-5768. doi:10.3748/wjg.14.5763