Skip to Main Content


  • • Autosomal dominant syndrome

    • Multiple GI non-neoplastic hamartomas

    • Predisposition to cancers

    Characteristic mucocutaneous pigmentation: Dark, macular lesions on mouth, buccal mucosa, lips, anus

    • Polyps occur primarily in jejunum and ileum

    • Can act as lead point for intussusception due to obstruction




  • • Familial autosomal dominant

    • Mapped to chromosome 19p13.3, encodes serine/threonine kinase

    • Carriers of the gene predisposed to a number of early-onset cancers

    • Increased risk of GI and gonadal, breast, pancreas, and biliary cancers


Symptoms and Signs


  • • Mucocutaneous lesions present from birth

    • Cutaneous lesions may involve buccal mucosa and may extend beyond vermillion border of the lips


Laboratory Findings


  • Polyps: Hamartomas consist of supportive framework of smooth muscle tissue covered by hyperplastic epithelium


  • • Neoplasms of small bowel:

    • -Adenocarcinoma

      -Carcinoid tumor



When to Admit


  • • Complications from polyps:

    • -Intussusception






  • • Removal of polyps

    • -Endoscopic techniques should be used when possible




  • • Complete bowel obstruction

    • Intractable GI bleeding

    • Development of malignancy

    • Surgery may be required for intussusception caused by small-intestinal polyps


Treatment Monitoring


  • • Screening for gonadal tumors and breast cancer




  • • Intestinal obstruction

    • GI bleeding

    • Cancer of small bowel/colon (uncommon)



Bond JH. Polyp guideline: diagnosis, treatment, and surveillance for patients with colorectal polyps. Practice Parameters Committee of the American College of Gastroenterology. Am J Gastroenterol. 2000;95:3053.  [PubMed: 11095318]

Want remote access to your institution's subscription?

Sign in to your MyAccess profile while you are actively authenticated on this site via your institution (you will be able to verify this by looking at the top right corner of the screen - if you see your institution's name, you are authenticated). Once logged in to your MyAccess profile, you will be able to access your institution's subscription for 90 days from any location. You must be logged in while authenticated at least once every 90 days to maintain this remote access.


About MyAccess

If your institution subscribes to this resource, and you don't have a MyAccess profile, please contact your library's reference desk for information on how to gain access to this resource from off-campus.

Subscription Options

AccessSurgery Full Site: One-Year Subscription

Connect to the full suite of AccessSurgery content and resources including more than 160 instructional videos, 16,000+ high-quality images, interactive board review, 20+ textbooks, and more.

$995 USD
Buy Now

Pay Per View: Timed Access to all of AccessSurgery

24 Hour Subscription $34.95

Buy Now

48 Hour Subscription $54.95

Buy Now

Pop-up div Successfully Displayed

This div only appears when the trigger link is hovered over. Otherwise it is hidden from view.