| Manifestation | Surveillance and interventions |
| Colorectal polyposis | - Annual colonoscopy starting around age 10 to 15 years.
- Polypectomy when feasible. All polyps >5 mm should be resected.
- If colonoscopy examinations are negative by age 15 years, the interval can be lengthened to every two years. Further negative examinations may warrant longer intervals.
- Colonoscopic surveillance may be reasonable for attenuated FAP.
- Colectomy for individuals with classic FAP if polyp burden is not manageable or if there are significant symptoms (such as bleeding or obstruction) and/or suspected or documented colorectal cancer.
- Annual exam of rectum/rectal cuff after colectomy.
- Chemoprevention can be considered postcolectomy to reduce polyp burden in the rectum/rectal cuff.
|
| Gastric and duodenal polyps | - Endoscopy (forward and side viewing to visualize ampulla) starting at the onset of colonic polyposis and if colectomy is planned or around age 20 to 25 years, whichever comes first (earlier if there is a history of early onset gastric or duodenal cancer in the kindred). For high-risk individuals, screening at least annually is recommended. For low-risk individuals, the interval can be lengthened to two to five years.
- Polypectomy when feasible, particularly for polyps ≥1 cm.
- Referral to center of expertise for advanced duodenal polyposis.
|
| Desmoid tumors | - Abdominal CT or MRI if high risk for desmoid, or if symptoms or a palpable mass develops after colectomy.
|
| Thyroid cancer | - Palpation of thyroid and thyroid ultrasound every two to five years starting in the late teens.
- Treatment is similar to sporadic disease.
|
| Hepatoblastoma | - Screening for those with positive family history using alpha-fetoprotein, liver palpation, and abdominal ultrasound every three to six months from infancy to age five years.
|
| Other cancers (medulloblastoma, small bowel) | - Individualized screening based on family history.
|
| Retinal pigment epithelium hamartomas (RPEH-FAP) | - Fundoscopy through dilated pupil with indirect ophthalmoscope.
- These lesions are typically benign, age independent, and not precancerous.
|
| Cutaneous lesions | - Examination for skin findings annually.
- Management depends on symptoms and findings.
|
| Osteomas and dental abnormalities (supernumerary teeth, odontomas) | - Examination for osteomas and dental abnormalities annually.
- Management depends on symptoms and findings.
|
| Relatives | - Reproductive counseling.
- Inform first-degree relatives about the importance of counseling and possible testing.
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