Find Information About:

Drugs & Supplements

Get information and reviews on prescription drugs, over-the-counter medications, vitamins, and supplements. Search by name or medical condition.

Pill Identifier
WebMD

Pill Identifier

Having trouble identifying your pills?

Enter the shape, color, or imprint of your prescription or OTC drug. Our pill identification tool will display pictures that you can compare to your pill.

Get Started
My Medicine
WebMD

My Medicine

Save your medicine, check interactions, sign up for FDA alerts, create family profiles and more.

Get Started

WebMD Health Experts and Community

Talk to health experts and other people like you in WebMD's Communities. It's a safe forum where you can create or participate in support groups and discussions about health topics that interest you.

  • Second Opinion
    WebMD

    Second Opinion

    Read expert perspectives on popular health topics.

  • Community
    WebMD

    Community

    Connect with people like you, and get expert guidance on living a healthy life.

Got a health question? Get answers provided by leading organizations, doctors, and experts.

Get Answers

Sign up to receive WebMD's award-winning content delivered to your inbox.

Sign Up

Cancer Health Center

Font Size
A
A
A

Unusual Cancers of Childhood Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Abdominal Cancers

continued...

Survival is consistent with the advanced stage of disease observed in most children with colorectal cancer, with an overall mortality rate of approximately 70%. For patients with a complete surgical resection or for those with low-stage/localized disease, survival is significantly prolonged, with curative potential.[80]

Genetic syndromes associated with colorectal cancer

About 20% to 30% of adult patients with colorectal cancer have a significant history of familial cancer; of these, about 5% have a well-defined genetic syndrome.[99] The incidence of these syndromes in children has not been well defined. In one review, 16% of patients younger than 40 years had a predisposing factor for the development of colorectal cancer.[100] A later study documented immunohistochemical evidence of mismatch repair deficiency in 31% of colorectal carcinoma samples in patients aged 30 years or younger.[101] The most common genetic syndromes associated with the development of colorectal cancer are shown in Tables 3 and 4.

Table 3. Common Genetic Syndromes Associated With Adenomatous Polyposis

Syndrome Gene Gene Function Hereditary Pattern
Attenuated familial adenomatous polyposis APC(5' mutations),AXIN2 Tumor suppressor Dominant
Familial adenomatous polyposis (Gardner syndrome) APC Tumor suppressor Dominant
Lynch syndrome (hereditary nonpolyposis colorectal cancer) MSH2, MLH1, MSH6, PMS2, EPCAM Repair/stability Dominant
Li-Fraumeni syndrome TP53(p53) Tumor suppressor Dominant
MYH-associated polyposis MYH(MUTYH) Repair/stability Recessive
Turcot syndrome APC Tumor suppressor Dominant
MLH1 Repair/stability Dominant

Table 4. Common Genetic Syndromes Associated With Hamartomatous Polyps

Syndrome Gene Gene Function Hereditary Pattern
Cowden syndrome PTEN Tumor suppressor Dominant
Juvenile polyposis syndrome BMPR1A, SMAD4, ENG Tumor suppressor Dominant
Peutz-Jeghers syndrome STK11 Tumor suppressor Dominant

Familial polyposis is inherited as a dominant trait, which confers a high degree of risk. Early diagnosis and surgical removal of the colon eliminates the risk of developing carcinomas of the large bowel.[102] Some colorectal carcinomas in young people, however, may be associated with a mutation of the adenomatous polyposis coli (APC) gene, which also is associated with an increased risk of brain tumors and hepatoblastoma.[103] The familial APC syndrome is caused by mutation of a gene on chromosome 5q, which normally suppresses proliferation of cells lining the intestine and later development of polyps.[104] A double-blind, placebo-controlled, randomized phase I trial in children aged 10 to 14 years with familial adenomatous polyposis (FAP) reported that celecoxib at a dose of 16 mg/kg/day is safe for administration for up to 3 months. At this dose, there was a significant decrease in the number of polyps detected on colonoscopy.[105][Level of evidence: 1iiDiv] The role of celecoxib in the management of FAP is not known.

1 | 2 | 3 | 4 | 5 | 6 | 7 | 8 | 9 | 10 | 11
1 | 2 | 3 | 4 | 5 | 6 | 7 | 8 | 9 | 10 | 11
Next Article:

Today on WebMD

Colorectal cancer cells
New! I AM Not Cancer Facebook Group
Lung cancer xray
See it in pictures, plus read the facts.
 
sauteed cherry tomatoes
Fight cancer one plate at a time.
Ovarian cancer illustration
Real Cancer Perspectives
 
Jennifer Goodman Linn self-portrait
Blog
what is your cancer risk
HEALTH CHECK
 
colorectal cancer treatment advances
Video
breast cancer overview slideshow
SLIDESHOW
 
prostate cancer overview
SLIDESHOW
lung cancer overview slideshow
SLIDESHOW
 
ovarian cancer overview slideshow
SLIDESHOW
Actor Michael Douglas
Article