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Gardner syndrome

Gardner syndrome is a form of familial adenomatous polyposis (FAP) that is characterized by multiple colorectal polyps and various types of tumors

Prevalence

N/A

N/A

US Estimated

N/A

Europe Estimated

Age of Onset

ICD-10

D12.6

Inheritance

This condition does not appear to have a clear pattern of inheritance.

5 Facts you should know

FACT

1

Gardner syndrome is an autosomal dominant form of polyposis characterized by the presence of multiple polyps in the colon together with tumors outside the colon

 

FACT

2

The number of polyps increases with age, and hundreds to thousands of polyps can develop in the colon

FACT

3

Cancers related to Gardner syndrome commonly appear in the thyroid, liver, and kidneys

 

FACT

4

The countless polyps in the colon predispose to the development of colon cancer; if the colon is not removed, the chance of colon cancer is considered to be very significant

FACT

5

Polyps may also grow in the stomach, duodenum, spleen, kidneys, liver, mesentery, and small bowel

Gardner syndrome is also known as...

Gardner syndrome is also known as:

  • Gardner's syndrome
  • Polyposis coli and multiple hard and soft tissue tumors
  • Intestinal polyposis
  • osteomas, sebaceous cysts

What’s your Rare IQ?

What is the average age that patients with Gardner syndrome are diagnosed?

Common signs & symptoms

Dental abnormalities

Fundic gland or adenomatous polyps of the stomach

Adenomatous polyps of the small intestines

Osteomas

(benign bone growths)

Congenital hypertrophy of the retinal pigment epithelium

(a flat, pigmented spot within the outer layer of the retina)

Benign skin abnormalities

such as epidermoid cysts, fibromas

Current treatments

Although there is no cure for Gardner syndrome, treatment and management options are available to reduce the risk of cancer. For example, affected people typically undergo regular screening for the various polyps and tumors associated with Gardner syndrome to permit early diagnosis and treatment. This screening regimen may include:

Sigmoidoscopy or colonoscopy

every one to two years, beginning at age ten to 12 years. Once polyps are detected, colonoscopy is recommended annually until colectomy (removal of colon).

EGD (esophagogastroduodenoscopy)

beginning by age 25 and repeated every one to three years.

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