New developments and future directions
in pseudomyxoma peritonei pathology
Novel treatments for cancer are being introduced all the time. For example, some cancers, including high grade pseudomyxoma peritonei (PMP) and appendiceal adenocarcinomas, may be treated with drugs such as cetuximab that are directed against the epidermal growth factor receptor on cancer cells.
However, a common gene that is mutated in cancer cells is RAS, and these particular drugs are not effective in cancers with RAS mutations. Therefore, testing for RAS status is now part of the histopathological assessment of tissue from patients with these tumours. Furthermore, research into drugs that might actively target mutated RAS genes is ongoing.
Our abilities to detect genetic abnormalities in cancer cells are constantly improving. Research into the abnormal genes of PMP is in its infancy, but one day could lead to new treatments.
Download The pathology of pseudomyxoma peritonei and appendix tumours
We hope you have found our pathology pages useful. Many thanks to Norman Carr for his gound-breaking work in this field and for working on this guide on our behalf. If you want to download the guide as an e-document (PDF).
Cetuximab
Cetuximab is a drug used to treat several types of cancer, including appendix cancers, colorectal cancer, and pancreatic cancer. It works by blocking a protein on the surface of cancer cells that helps them grow and spread.
This protein is called the epidermal growth factor receptor or EGFR. Cancer cells often have too much activity in the EGFR which leads them to grow out of control. Cetuximab is an antibody that attaches to the EGFR and prevents it from functioning properly. This effectively puts the brakes on cancer cell growth.
Cetuximab only works against cancer cells that have an active EGFR. For this reason, doctors test the EGFR status of your tumor before prescribing cetuximab. If your EGFR is not active or overexpressed, cetuximab likely will not help fight your cancer.
References
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.