Is peutz jegher polyp premalignant?
The polyps are not premalignant, but a definite association exists between Peutz-Jeghers syndrome and gastrointestinal carcinoma. Evidence shows that the syndrome is associated with an increased risk of extraintestinal malignancy, especially carcinomas of the pancreas, breast, and reproductive organs.
What cancers are associated with Peutz-Jeghers syndrome?
Peutz-Jeghers syndrome (PJS) is an inherited condition that puts people at an increased risk for developing hamartomatous polyps in the digestive tract, as well as cancers of the breast, colon and rectum, pancreas, stomach, testicles, ovaries, lung, cervix, and other types listed below.
Is Peutz-Jeghers malignant?
People with Peutz-Jeghers syndrome have a high risk of developing cancer during their lifetimes. Cancers of the gastrointestinal tract, pancreas, cervix, ovary, and breast are among the most commonly reported tumors.
Why there is pigmentation in Peutz-Jeghers syndrome?
Peutz-Jeghers syndrome, usually inherited as an autosomal dominant trait, is the combination of skin hyperpigmentation and Peutz-Jeghers polyps in the gastrointestinal tract. The pigmentation consists of clusters of black-brown freckles about the lips, the buccal mucosa, and the perianal and genital area.
Who discovered Peutz-Jeghers syndrome?
Harold Jeghers in 1949 when he described 10 cases and was subsequently named Peutz Jeghers syndrome in 1954 by Dr. Andre Bruwer. The gene causing PJS (STK-11/LKB1) was identified in 1998 and allows early detection of the disease and screening of family members.
Who gets Peutz-Jeghers syndrome?
Peutz-Jeghers syndrome (PJS) is an inherited (passed down from a family member) condition. Approximately 50 percent of individuals with PJS have a parent with the syndrome. The other 50 percent of people diagnosed with PJS are the first in the family with the syndrome.
Is Peutz-Jeghers syndrome life threatening?
Peutz-Jeghers syndrome (PJS) is a rare, but life-threatening, familial inherited disease, characterized by mucocutaneous pigmentation and hamartomatous polyps. The estimated incidence of PJS ranges from 1/50,000 to 1/200,000.
Is Peutz-Jeghers syndrome rare?
Peutz-Jeghers syndrome (PJS) is a rare disorder in which growths called polyps form in the intestines. A person with PJS has a high risk of developing certain cancers.
Can Gardner syndrome be cured?
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.