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
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
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
4
Q
What medications may slow the growth of a desmoid tumor?
A
- Tamoxifen
- sulindac
- steroids
5
Q
What is the inheritance pattern?
A
Varying degree of penetrance from an autosomal-dominant gene
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
