Hereditary and familial colorectal cancer Treatment
Treatment
- Lynch syndrome
Surveillance decreases the incidence of CRC and related deaths.
Screening colonoscopy should be initiated by 20–25 years of age and repeated every 1–2 years.
Subtotal colectomy with ileorectal anastomosis is advised with the appearance of colon cancer. Annual rectal surveillance is indicated thereafter. Segmental resection can be considered if annual colonoscopy can be assured thereafter.
Prophylactic hysterectomy and bilateral salpingo-oophorectomy reduce the incidence of endometrial and ovarian cancer, should be discussed with women after completion of childbearing [1] [2]. Screening for these malignancies is also used but is less effective [2].
Long term aspirin use has been shown to decrease CRC incidence in Lynch syndrome and may be recommended in the future.
- Familial adenomatous polyposis (FAP): FAP and attenuated FAP
- FAP
Colonoscopy every 1–2 years beginning at age 10–12 years old.
Once adenomatous polyps emerge, and annual follow-up colonoscopy is recommended until colectomy. Colectomy should be considered when: - More than 20 adenomas - Adenomas > 1cm - Advanced histology
Total procto-colectomy with ileal pouch anal anastamosis (IPAA) is recommended when large numbers of adenomas are found in the rectum. If the rectum is preserved with ileo-rectal anastamosis (few or no rectal adenomas are present). Annual or more frequent endoscopic follow-up is required. Up to 33% of patients with a preserved rectum need completion proctectomy later, because of diffuse polyposis.
There is a questionable role of NSAIDs.
- Attenuated FAP:
Screening every 1–2 year, beginning in the late teenage years.
Up to 33% of patients can be managed with colonoscopy and polypectomy [3].
Up to 66% will eventually require colectomy, although colectomy with ileorectal anastamosis is virtually always done to spare the rectum.
Annual post-operative surveillance is required for polyp ablation, but subsequent proctectomy is rarely needed.
- FAP and attenuated FAP:
Upper GI endoscopy should be performed every 1–3 years followed by endoscopic ultrasound for suspicious lesions at the ampulla. Starting age of 25 to 30 years [4].
Annual physical examination and possibly thyroid ultrasound due to the increased risk for thyroid cancer [5].
- MUTYH-associated polyposis
- Hamartomatous polyposis conditions:
- Hyperplastic polyposis (HPP)
References
- ↑ Schmeler KM, Lynch HT, Chen LM; et al. (2006). "Prophylactic surgery to reduce the risk of gynecologic cancers in the Lynch syndrome". N. Engl. J. Med. 354 (3): 261–9. doi:10.1056/NEJMoa052627. PMID 16421367. Unknown parameter
|month=ignored (help) - ↑ 2.0 2.1 Kwon JS, Sun CC, Peterson SK; et al. (2008). "Cost-effectiveness analysis of prevention strategies for gynecologic cancers in Lynch syndrome". Cancer. 113 (2): 326–35. doi:10.1002/cncr.23554. PMID 18506736. Unknown parameter
|month=ignored (help) - ↑ Burt RW, Leppert MF, Slattery ML; et al. (2004). "Genetic testing and phenotype in a large kindred with attenuated familial adenomatous polyposis". Gastroenterology. 127 (2): 444–51. PMID 15300576. Unknown parameter
|month=ignored (help) - ↑ Gallagher MC, Phillips RK, Bulow S (2006). "Surveillance and management of upper gastrointestinal disease in Familial Adenomatous Polyposis". Fam. Cancer. 5 (3): 263–73. doi:10.1007/s10689-005-5668-0. PMID 16998672.
- ↑ Herraiz M, Barbesino G, Faquin W; et al. (2007). "Prevalence of thyroid cancer in familial adenomatous polyposis syndrome and the role of screening ultrasound examinations". Clin. Gastroenterol. Hepatol. 5 (3): 367–73. doi:10.1016/j.cgh.2006.10.019. PMID 17258512. Unknown parameter
|month=ignored (help)