Familial adenomatous polyposis syndromes

Spigelman criteria[36]Spigelman AD, Williams CB, Talbot IC, et al. Upper gastrointestinal cancer in patients with familial adenomatous polyposis. Lancet. 1989 Sep 30;2(8666):783-5. http://www.ncbi.nlm.nih.gov/pubmed/2571019?tool=bestpractice.com

The Spigelman criteria are used to assess the severity of duodenal/periampullary polyps in patients with familial adenomatous polyposis (FAP)/attenuated FAP. There are 4 categories, and each factor in a category is assigned a point value. The 4 categories are:

• Duodenal/periampullary polyp number (1-4 = 1 point; 5-20 = 2 points; >20 = 3 points)

• Size (1-4 mm = 1 point; 5-10 mm = 2 points; >10 mm = 3 points)

• Histology (tubular = 1 point; tubulovillous = 2 points; villous = 3 points)

• Degree of dysplasia (low grade = 1 point; high grade = 3 points).

Scoring:

• 0 points is classified as stage 0 disease

• 1 to 4 points is classified as stage 1 disease

• 5 to 6 points is classified as stage 2 disease

• 7 to 8 points is classified as stage 3 disease

• 9 to 12 points is classified as stage 4 disease.

Recommended treatment and surveillance vary for each Spigelman stage.