Adenomatous cytologic features infrequent, focal at most
Most polyps are adenomatous, either pure or mixed
Most polyps are sessile
Most are polypoid, regardless of histology
Glands are vertically arranged and not complex
Frequently shows features of juvenile polyps: prominent stroma, cystic dilation
Patients with syndrome are considered at risk for development of colorectal carcinoma
Level of risk is unclear because of rarity of syndrome
25-50% incidence of cancer has been reported
Referral bias may affect these numbers
Increased screening of patients and first degree relatives is indicated
Removal of lesions >1 cm is recommended
Total or partial colectomy may be indicated if numerous
Rare familial cases have been reported
Pattern of heredity is not consistent
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