Synonyms
Hyperplastic polyposis syndrome; Serrated adenomatous polyposis syndrome
Definition
Serrated polyposis syndrome is a clinically defined syndrome characterized by the occurrence of multiple serrated polyps in the large intestine. The definition of serrated polyposis syndrome (SPS) includes (1) at least 5 serrated polyps proximal to the sigmoid with two or more being larger than 10 mm, (2) any number of serrated polyps proximal to sigmoid in an individual who has a first-degree relative with serrated polyposis, and (3) more than 20 serrated polyps of any size distributed throughout the colon. SPS is usually asymptomatic with no associated extracolonic manifestations. Two clinical forms have been described, namely, type 1 characterized by larger and proximally located serrated polyps commonly showing BRAF mutations and/or MMR gene methylation and MSI and a high risk of cancer, whereas type 2 is characterized by numerous small hyperplastic polyps which show KRAS mutations and...
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References and Further Reading
Crowder, C. D., Sweet, K., Lehman, A., & Frankel, W. L. (2012). Serrated polyposis is an underdiagnosed and unclear syndrome: The surgical pathologist has a role in improving detection. The American Journal of Surgical Pathology, 36(8), 1178–1185.
Guarinos, C., Sánchez-Fortún, C., RodrÃguez-Soler, M., Alenda, C., Payá, A., & Jover, R. (2012). Serrated polyposis syndrome: Molecular, pathological and clinical aspects. World Journal of Gastroenterology, 18(20), 2452–2461.
Kalady, M. F., Jarrar, A., Leach, B., Laguardia, L., O’malley, M., Eng, C., & Church, J. M. (2011). Defining phenotypes and cancer risk in hyperplastic polyposis syndrome. Diseases of the Colon and Rectum, 54(2), 164–170.
Snover, D. C. (2011). Update on the serrated pathway to colorectal carcinoma. Human Pathology, 42(1), 1–10, Epub 2010 Sep 24.
Snover, D. C., Ahnen, D. J., Burt, R. W., & Odze, R. D. (2010). Serrated polyps of the colon and rectum and serrated polyposis. In F. T. Bosman, F. Carneiro, R. H. Hruban, & N. D. Theise (Eds.), WHO classification of tumours of the digestive system (4th ed.). Lyon: IARC.
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Ensari, A. (2017). Serrated Polyposis Syndrome. In: Carneiro, F., Chaves, P., Ensari, A. (eds) Pathology of the Gastrointestinal Tract. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-40560-5_1487
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DOI: https://doi.org/10.1007/978-3-319-40560-5_1487
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