- Hereditary Breast and Ovarian Cancer syndrome (HBOC)
- Lynch syndrome/Hereditary Non-Polyposis Colorectal Cancer (HNPCC)
- Familial Adenomatous Polyposis (FAP)/Attenuated Familial Adenomatous Polyposis (AFAP)
- MUTYH-associated Polyposis syndrome (MAP)
- MUTYH-associated Colon Cancer Risk
- Melanoma Cancer Syndrome (MCS)
- Li-Fraumeni Syndrome (LFS)
- PTEN Hamartoma Tumor syndrome (PHTS)
- Peutz-Jeghers Syndrome
- Hereditary Diffuse Gastric Cancer (HDGC) Syndrome
- Juvenile Polyposis Syndrome (JPS)
- Juvenile Polyposis Syndrome (JPS) and Hereditary Hemorrhagic Telangiectasia (HHT)
- PALB2-associated Cancer Risk
- CHEK2-associated Cancer Risk
- ATM-associated Cancer Risk
- NBN-associated Cancer Risk
- BARD1-associated Cancer Risk
- BRIP1-associated Cancer Risk
- RAD51C-associated Cancer Risk
- RAD51D-associated Cancer Risk
- Polymerase Proofreading-associated Syndrome (PPAS)
- Hereditary Mixed Polyposis Syndrome (HMPS)
Peutz-Jeghers Syndrome (PJS) STK11 ASSOCIATED CANCER RISKS
Peutz-Jeghers Syndrome (PJS)
STK11 ASSOCIATED CANCER RISKS
What does it mean to have a STK11 gene mutation, and a diagnosis of Peutz-Jeghers Syndrome (PJS)?
People with mutations in the STK11 gene have a condition called Peutz-Jeghers Syndrome, or PJS. People with PJS have a high risk for many different types of cancer, including breast, colon, pancreatic, stomach, small bowel, cervical, and endometrial. Women with PJS can also develop a type of ovarian tumor called a sex cord tumor, and men can develop tumors of the testes. These tumors do not usually become cancerous, but they do require medical attention. Cancers in people with PJS are often diagnosed at young ages.
One of the most common symptoms of PJS is an unusual type of polyp in the colon and other parts of the gastrointestinal system. These “Peutz-Jeghers polyps” can cause bleeding and/or blockages in the digestive system.
What can be done to protect people with PJS from cancer?
The National Comprehensive Cancer Network (NCCN) provides recommendations for lowering the risk of cancer in men and women with PJS. These recommendations include starting screening at younger ages than normal and having the screenings more often. For example, colonoscopies to check for colon cancer should begin in the late teenage years, and should be done every 2 to 3 years. Women with PJS should begin breast screening in their 20’s, and should have MRIs instead of, or in addition to, mammograms.
Because PJS is associated with many different cancer risks, it is recommended that people with mutations in the STK11 gene be cared for by healthcare professionals with experience in treating this condition.
Additional details about STK11 gene mutations and PJS, including information about the risks for different kinds of cancer, specific recommendations for medical care, and useful information for relatives of people who have a diagnosis of PJS, are available within our Support Organizations pags
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