Synonyms
Definition
Pseudomyxoma peritonei (PMP) is a rare epithelial neoplasm, arising in the majority of cases from the appendix, and characterized by disseminated peritoneal mucinous tumor deposition and progressive accumulation of mucinous ascites. PMP is a registered rare disease (no. 843) by the National Organization of Rare Disorders.
Characteristics
Epidemiology
In Western populations, the estimated incidence is 1–2 per million, accounting for approximately 1,500 new cases per year in the USA and 50–70 new cases per annum in the UK. Most series show a female predominance, typically a 70:30 ratio. The mean age at presentation is in the mid-sixth decade of life, 10 years younger than the mean age for colorectal cancer. Although of “bowel origin,” it is not known whether PMP shares PMP etiological risk factors such as family history, Westernized diet, obesity, and physical inactivity, as there is a paucity of large-scale epidemiological studies in this area.
Histor...
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References
5th International Workshop on Peritoneal Surface Malignancy (2006) Peritoneal Surface Oncology Group International (PSOGI). Societa Italiana di Terapie Integrate Locoregionali in Oncologia (SITILO), Milan (http://www.peritonealworkshop2006.com)
Bibi R, Pranesh N, Saunders MP et al (2006) A specific cadherin phenotype may characterise the disseminating yet non-metastatic behaviour of pseudomyxoma peritonei. Br J Cancer 95:1258–1264
Bradley RF, Stewart JH, Russell GB et al (2006) Pseudomyxoma peritonei of appendiceal origin: a clinicopathologic analysis of 101 patients uniformly treated at a single institution, with literature review. Am J Surg Pathol 30:551–559
Carr NJ, Cecil TD, Mohamed F, Sobin LH, Sugarbaker PH, Gonzalez-Moreno S, et al. (2016) A Consensus for Classification and Pathologic Reporting of Pseudomyxoma Peritonei and Associated Appendiceal Neoplasia: The Results of the Peritoneal Surface Oncology Group International (PSOGI) Modified Delphi Process. Am J Surg Pathol 40(1):14–26
Chua TC, Moran BJ, Sugarbaker PH, Levine EA, Glehen O, Gilly FN, et al (2012) Early- and long-term outcome data of patients with pseudomyxoma peritonei from appendiceal origin treated by a strategy of cytoreductive surgery and hyperthermic intraperitoneal chemotherapy. Journal of clinical oncology: official journal of the American Society of Clinical Oncology 30(20):2449–56
Roberts DL, O’Dwyer ST, Stern PL, Renehan AG (2015) Global gene expression in pseudomyxoma peritonei, with parallel development of two immortalized cell lines. Oncotarget 6(13):10786-800
Ronnett BM, Shmookler BM, Sugarbaker PH et al (1997) Pseudomyxoma peritonei: new concepts in diagnosis, origin, nomenclature, and relationship to mucinous borderline (low malignant potential) tumors of the ovary. Anat Pathol 2:197–226
Sugarbaker PH (2006) New standard of care for appendiceal epithelial neoplasms and pseudomyxoma peritonei syndrome? Lancet Oncol 7:69–76
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© 2014 Springer-Verlag Berlin Heidelberg
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Renehan, A.G., O’Dwyer, S.T., Stern, P.L. (2014). Pseudomyxoma Peritonei. In: Schwab, M. (eds) Encyclopedia of Cancer. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-27841-9_4843-2
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DOI: https://doi.org/10.1007/978-3-642-27841-9_4843-2
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Publisher Name: Springer, Berlin, Heidelberg
Online ISBN: 978-3-642-27841-9
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