| FAP |
| Additional Types of Cancer in People With FAP |
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By
Miriam Komaromy,
MD
Reviewed
by Peggy Conrad,
MS, CGC and Jonathan
Terdiman, MD
Last updated August 15, 2000
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It's
a sobering fact that a diagnosis of familial adenomatous polyposis (FAP) — a hereditary
syndrome triggered by mutations in a specific gene —
virtually guarantees an eventual diagnosis of colon
cancer, unless the colon is removed. People with FAP
are also more likely to develop several additional types
of cancer than are members of the population at large:
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Cancer
of the Small Bowel
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| Up
to five percent of FAP patients will eventually
develop small bowel cancer. |
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Although extremely
rare in the general population, cancer of the small bowel
(or small intestine), the part of the digestive tract
located above the colon, is more than 300 times as likely
to occur in people with FAP. Most people with FAP syndrome
will eventually develop polyps, or adenomas, in the small
bowel. However, less than one percent of people with FAP
actually die from small bowel cancer.
Doctors recommend that people with FAP be screened for
small bowel cancer every four years. Endoscopy,
the procedure used for this, enables the doctor to make
a visual search for polyps using a miniature camera attached
to a thin, flexible tube that is guided through the patient's
mouth and down through the stomach and part of the small
bowel. |
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Gastric
Cancer
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| People
with FAP are also more likely to develop gastric (stomach)
polyps, although these do not appear to increase their
risk of developing gastric cancer. (The polyps that develop
in the stomach are a different type than those that develop
in the colon and small intestine, and are less likely
to become cancerous.) The exception to this occurs with
FAP patients of Japanese and Korean descent, whose risk
for stomach polyps becoming cancerous has been reported
to be three to four times greater than that of other FAP
patients. |
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Thyroid
Cancer
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| Although
women with FAP are at increased risk for thyroid cancer,
this disease is rare, both in the general population and
in the FAP group. Only one percent to two percent of women
with FAP develop thyroid cancer, and the disease is rarely
fatal. Therefore, experts do not recommend that people
with FAP undergo regular screening for thyroid cancer.
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 top
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Other
Cancers
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In
addition, people with FAP are more likely than members
of the general population to develop:
- Brain
cancer
- An
extremely rare form of childhood liver cancer
In
addition, scientists recently discovered that the combination
of colon cancer and brain cancer in a single family
— commonly referred to as Turcot's syndrome —
is actually a hallmark of both FAP and hereditary nonpolyposis colorectal cancer (HNPCC), another
hereditary syndrome associated with colon cancer.
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References
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Galle,
T. et al. (1999). Causes of death in familial adenomatous
polyposis. Scandinavian Journal of Gastroenterology
34(8): 808-812.
Johan, G. et al. (1992). The risk of upper gastrointestinal
cancer in familial adenomatous polyposis. Gastroenterology
102: 1980-1982
Cetta, F. et al. (1997). Thyroid carcinoma associated
with familial adenomatous polyposis. Histopathology
31(3): 231-6.
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