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hamartomatous polyposis syndrome; familial hamartomatous polyposis

Classification: - juvenile polyposis syndrome - Peutz-Jeghers syndrome - PTEN hamartoma tumor syndrome - Cowden's disease - Bannayan-Zonana syndrome Pathology: - hamartomatous polyps of the gastrointestinal tract Genetics: - autosomal-dominant pattern of inheritance Complications: - increased risk for multiple cancers, including colon cancer [1] Management: - surveillance & genetic counseling

Related

hamartoma

Specific

Cowden's disease; multiple hamartoma syndrome including Lhermitte-Duclos disease (cerebelloparenchymal disorder) juvenile polyposis coli Peutz-Jeghers syndrome (hamartomatous intestinal polyposis) PTEN hamartoma tumor syndrome

General

intestinal polyposis syndrome

References

  1. Medical Knowledge Self Assessment Program (MKSAP) 15, 17, 18. American College of Physicians, Philadelphia 2009, 2015, 2018
  2. Zbuk KM, Eng C. Hamartomatous polyposis syndromes. Nat Clin Pract Gastroenterol Hepatol. 2007 Sep;4(9):492-502. Review. PMID: 17768394