Hereditary and familial colorectal cancer Treatment
Treatment
- Lynch syndrome
Surveillance decreases the incidence of CRC and related deaths.
- Colon: 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.
- Endometrium/Ovary: 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].
- Upper urinary tract: annual urinalysis begining age 30 - 35 years.
- Upper GI tract: EGD every 1 - 2 years, beginning at age 30 - 35 years.
- Other: annual physical examination should include a review of symptoms suggestive of cancer and skin examination looking for sebaceous carcinoma.
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
- Colon: 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:
- Colon: 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 tract: EGD every 1 - 3 years beginning at age 20 - 25 yars. Followed by endoscopic ultrasound for suspicious lesions at the ampulla. Starting age of 25 to 30 years [4].
- Other: Annual physical examination and possibly thyroid ultrasound due to the increased risk for thyroid cancer [5].
- MUTYH-associated polyposis
- Colon: Individuals often develop proximally located CRCs. Individuals should receive colonoscopic survelliance every 2 - 3 years, starting in their mid-20s or mid-30s [6]. Subtotal colectomy is advised for individuals who develop colon cancer. Subtotal colectomy is considered for individuals with problematic colonoscopic management or when polyps become large or exhibit high-grade dysplasia.
- Duodenum: Screening for upper-GI tract tumors is also recommended due to the risk for duodenal cancer. EGD every 1 - 3 years, beginning at age 20 - 25 years.
- Hamartomatous polyposis conditions:
- Peutz-Jeghers syndrome:
- Colon: COlonoscopy every 2 - 3 years, starting with symptoms or in late teens.
- Breast: annual mammogram and breast MRI starting at age 25 years. Biannual clinical breast exam beginning at age 25 years.
- Pancreas: MRCP and/or endoscopic ultrasound of the pancreas every 1 - 2 years starting at age 30 years.
- Stomach/small bowel: EGD and abdominal CT with oral contrast every 2 - 3 years , starting at age 10 years.
- Cervix/Uterus/Ovary: annual pelvic examination, Pap smear and transvaginal ultrasound starting at age 18 years.
- Testes: annual testicular exam starting at age 10.
- Juvenile polyposis syndrome:
- Colon: colonoscopy every 2 - 3 years, starting with symptoms or in late teens.
- Stomach: EGD every 1 - 3 years.
- Hyperplastic polyposis (HPP)
- Colon: colonoscopy every 1 - 2 years in compromised individuals. Removal of all polyps is recommended. Prophylactic colectomy when polyps are unmanageable.
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) - ↑ Lubbe SJ, Di Bernardo MC, Chandler IP, Houlston RS (2009). "Clinical implications of the colorectal cancer risk associated with MUTYH mutation". J. Clin. Oncol. 27 (24): 3975–80. doi:10.1200/JCO.2008.21.6853. PMID 19620482. Unknown parameter
|month=ignored (help)