How common is Peutz-Jeghers syndrome?

How common is Peutz-Jeghers syndrome?

Peutz Jeghers syndrome is a rare disorder that affects males and females in equal numbers and can occur in any racial or ethnic group. The birth prevalence of PJS is estimated to be between 1/50,000 and 1/200,000.

Can Peutz-Jeghers syndrome cause cancer?

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 syndrome benign?

People with PJS can develop polyps — called Peutz-Jeghers polyps — in the small bowel, colon, stomach, lungs, nose, bladder and rectum. These polyps are considered hamartomatous polyps. Hamartomatous polyps are benign (not cancerous) overgrowths of tissues.

Is Peutz-Jeghers syndrome 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.

Why there is pigmentation in Peutz Jeghers syndrome?

Peutz–Jeghers syndrome (PJS) is an autosomal dominant polyposis disorder due to germline LKB1 mutations, characterized by intestinal polyposis and mucocutaneous skin pigmentation (Figure 11).

Are hamartomatous polyp cancerous?

Hamartomatous polyps, in and of themselves, are benign entities; however, these hamartomatous polyposis syndromes have a malignant potential for the development of colorectal cancer as well as extracolonic cancers. Early detection and proper surveillance are vital to minimizing the risk of carcinoma.

Why there is pigmentation in Peutz-Jeghers syndrome?

Why is there pigmentation in Peutz-Jeghers syndrome?