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Turcot Syndrome

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What is Turcot syndrome?

Turcot syndrome is a condition in which cells become abnormal and form masses called polyps. A polyp is benign (noncancerous) but can eventually turn malignant (cancerous, meaning it can spread to other parts of the body). Turcot syndrome is rare and is considered to be an alternative form of two more common syndromes associated with polyp formations: Lynch syndrome and familial adenomatous polyposis (FAP).

People with Turcot syndrome have multiple adenomatous colon polyps (polyps in the colon made up of cells that form mucous), an increased risk of colorectal cancer, and an increased risk of brain cancer. The type of brain cancer generally depends on whether the Turcot syndrome is more similar to Lynch syndrome or FAP. The two most common types of brain tumors in Turcot syndrome are:

Glioblastoma. This type of brain tumor is a very aggressive form of astrocytoma that is commonly found in families who have features of Lynch syndrome.

Medulloblastoma.This type of brain tumor begins in granular cells in the cerebellum (back of the brain). Medulloblastoma most often occurs in children and is commonly found in families who have features of FAP.

What causes Turcot syndrome?

Turcot syndrome is a genetic condition. This means that the risk of Turcot syndrome can be passed from generation to generation in a family. In families with glioblastoma and other features of Lynch syndrome, mutations (alterations) have been found in two genes: MLH1and PMS2. In families with medulloblastoma and other features of FAP, mutations have been found in the APC gene.

How is Turcot syndrome inherited?

Normally, every cell has two copies of each gene: one inherited from the mother and one inherited from the father. Turcot syndrome follows an autosomal dominant inheritance pattern, in which a mutation needs to happen in only one copy of the gene for the person to have an increased risk of getting that disease. This means that a parent with a gene mutation may pass along a copy of their normal gene or a copy of the gene with the mutation. Therefore, a child who has a parent with a mutation has a 50% chance of inheriting that mutation. A brother, sister, or parent of a person who has a mutation also has a 50% chance of having the same mutation.

How common is Turcot syndrome?

Turcot syndrome is considered to be rare.

How is Turcot syndrome diagnosed?

A person with multiple adenomatous colon polyps and/or colorectal cancer, along with either glioblastoma or medulloblastoma, may have Turcot syndrome. Blood tests are available to look for the three genes linked to Turcot syndrome. People who may have Turcot syndrome can have a blood test to look for a mutation in the APC gene associated with FAP or the MLH1 gene associated with Lynch syndrome. They may also have testing for the mutation in PMS2 if Turcot syndrome is suspected. If a specific gene mutation is found, other family members may also be diagnosed with Turcot syndrome if they are tested and have the same gene mutation. However, some families that appear to have Turcot syndrome may not have a detectable gene mutation.

What are the estimated cancer risks associated with Turcot syndrome?

The risks of cancer and other features depend on whether the Turcot syndrome appears to be more similar to Lynch syndrome or FAP; visit the sections for those conditions for a summary of cancer risks and other features.

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