Chapter 47: Colon and Rectum- Polyposis Syndromes (Gardner) Flashcards

1
Q

What are the characteristics?

A
  • Neoplastic polyps of the small bowel and colon
  • cancer by age 40 in 100% of undiagnosed patients, as in FAP
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2
Q

What are the other associated findings?

A
  • Desmoid tumors (in abdominal wall or cavity)
  • osteomas of skull (seen on x-ray)
  • sebaceous cysts
  • adrenal and thyroid tumors
  • retroperitoneal fibrosis
  • duodenal and periampullary tumors
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3
Q

What is a desmoid tumor?

A
  • Tumor of the musculoaponeurotic sheath
  • usually of the abdominal wall
  • benign but grows locally
  • treated by wide resection
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4
Q

What medications may slow the growth of a desmoid tumor?

A
  1. Tamoxifen
  2. sulindac
  3. steroids
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5
Q

What is the inheritance pattern?

A

Varying degree of penetrance from an autosomal-dominant gene

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6
Q

What is the treatment of colon polyps in patients with Gardner’s syndrome?

A

Total proctocolectomy and ileostomy

Total colectomy and rectal mucosal removal (mucosal proctectomy) and ileoanal anastomosis

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