Table 6.

Recommended Surveillance for Individuals with FAP

System/ConcernEvaluationFrequency/Comment
Colorectal
adenomatous
polyps 		ColonoscopyEvery 1-2 yrs beginning: at age 10-15 yrs for classic FAP; in late adolescence for attenuated FAP
In persons who have undergone total colectomy w/IPAA: endoscopic surveillance of ileal pouchEvery 1-2 yrs
In persons who have undergone subtotal colectomy w/ileorectal anastomosis: surveillance of remaining rectum
  • Every 1-2 years or more frequently if high polyp burden
  • Note: Cancer may occur in remaining rectum, but risk is low. 1
In persons who have undergone total colectomy w/end ileostomy: ileoscopyEvery 1-2 yrs
Small-bowel
polyps & cancer 		EGD w/complete visualization of ampulla of Vater (using duodenoscope or clear cap)Every 6 mos-5 yrs depending on duodenal adenoma burden 2 beginning at age 20-25 yrs or prior to colectomy
Consider complete small bowel visualization by video capsule endoscopy or CT/MR enterography esp if duodenal polyposis is advanced (based on Spigelman scoring system).
Thyroid cancer
  • Palpation of thyroid
  • Thyroid ultrasound
  • Fine-needle aspiration if thyroid nodules are present
Every 2-5 years starting in late adolescence 3
CNS tumors Neurologic examAnnually beginning at diagnosis
Hepatoblastoma
  • Liver palpation
  • Abdominal ultrasound exam
  • Serum alpha-fetoprotein concentration
Every 3-6 mos during 1st 5 yrs of life 4
Gastric polyps
& cancer 		EGD
  • Every 6 mos-5 yrs (w/duodenal exam), beginning at age 20-25 yrs
  • If polyps are neoplastic or large consider annual exams.
Non-malignant
extraintestinal
manifestations 		Physical exam for extraintestinal manifestations (e.g., osteomas, dental abnormalities, cutaneous lesions)Annually
Desmoid tumors Abdominal palpationAnnually
MRI or CT scanNo routine screening recommended; however, in those who develop unexplained symptoms after colectomy, clinicians should have a low threshold to evaluate for desmoids that can cause compression of adjacent structures. 5
Adrenal tumors No evidence to warrant screening

CNS = central nervous system; EGD = esophagogastroduodenoscopy; FAP = familial adenomatous polyposis; IPAA = ileal pouch anal anastomosis

1.
2.

The frequency of EGD depends on the severity of duodenal adenomas; Spigelman staging criteria can help determine the frequency. The Spigelman staging criteria are summarized by Syngal et al [2015]; see also Table 5.

3.
4.
5.

Data to support screening of desmoid tumors are limited.

From: APC-Associated Polyposis Conditions

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