What is Jagermeister 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.

How do you know if you have Peutz-Jeghers syndrome?

Children with Peutz-Jeghers syndrome often develop small, dark-colored spots on the lips , around and inside the mouth , near the eyes and nostrils, and around the anus. These spots may also occur on the hands and feet. They appear during childhood and often fade as the person gets older.

How do you test for Peutz-Jeghers syndrome?

Children are tested for Peutz-Jeghers syndrome (PJS) by performing an upper gastroenterology (GI) endoscopy and a colonoscopy. These procedures allow the doctor to look inside the esophagus, stomach, duodenum and large intestine using separate long, flexible tubes with cameras on the end.

What are hamartomatous polyps?

Hamartomatous polyps are composed of the normal cellular elements of the gastrointestinal tract, but have a markedly distorted architecture. The hamartomatous polyposis syndromes are a heterogeneous group of disorders that are inherited in an autosomal-dominant manner.

Is there a cure for Peutz Jeghers syndrome?

Currently, there is no cure for Peutz-Jeghers syndrome (PJS). Patients undergo lifelong surveillance of organs to monitor for cancer and prevent secondary problems from the polyps.

Is Lynch syndrome autosomal dominant?

Lynch syndrome runs in families in an autosomal dominant inheritance pattern. This means that if one parent carries a gene mutation for Lynch syndrome, there’s a 50 percent chance that mutation will be passed on to each child.

How many people in the world have Peutz Jeghers syndrome?

Peutz Jeghers syndrome (PJS) is an autosomal dominant genetic condition affecting around 1/50,000 and 1/200,000 individuals.

Is STK11 a tumor suppressor?

The STK11 gene (also called LKB1) provides instructions for making an enzyme called serine/threonine kinase 11. This enzyme is a tumor suppressor, which means that it helps keep cells from growing and dividing too fast or in an uncontrolled way.

Are hamartomatous polyps cancerous?

Though most of the gastric hamartomatous polyps are benign, certain types are associated with increased malignant potential. These include certain polyps associated with specific genetic familial polyposis syndromes and gastric inverted hamartomatous polyps.

What does hamartomatous mean?

(HA-mar-TOH-muh) A benign (not cancer) growth made up of an abnormal mixture of cells and tissues normally found in the area of the body where the growth occurs.