Most people with<unk>  will have developed some form of neoplastic disease by age 60 Most patients will develop flat, brownish spots (melanotic macules) on the skin, especially on the lips and oral mucosa, during the first year of life, and a patient's first bowel obstruction due to intussusception usually occurs between the ages of six and 18 years. The cumulative lifetime cancer risk begins to rise in middle age. Cumulative risks by age 70 for all cancers, gastrointestinal (GI) cancers, and pancreatic cancer are 85%, 57%, and 11%, respectively. A 2011 Dutch study followed 133 patients for 14 years. The cumulative risk for cancer was 40% and 76% at ages 40 and 70, respectively. 42 (32%) of the patients died during the study, of which 28 (67%) were cancer related. They died at a median age of 45. Mortality was increased compared with the general population. A family with sinonasal polyposis were followed up for 28 years. Two cases of sinonasal type adenocarcinoma developed. This is a rare cancer. This report suggested that follow up of sinus polyps in this syndrome may be indicated. Monitoring Barium enema radiograph showing multiple polyps (mostly pedunculated) and at least one large mass at the hepatic flexure coated with contrast in a patient with<unk> . Some suggestions for surveillance for cancer include the following: * Small intestine with small bowel radiography every 2 years, * Esophagogastroduodenoscopy and colonoscopy every 2 years, * CT scan or MRI of the pancreas yearly, * Ultrasound of the pelvis and testes yearly * Mammography from age 25 annually * Papanicolaou smear (Pap smear) annually beginning at age 18-20 Follow-up care should be supervised by a physician familiar with<unk> . Genetic consultation and counseling as well as urological and gynecological consultations are often needed.
Peutz–Jeghers syndrome
Juvenile polyp
Familial adenomatous polyposis
Nijmegen breakage syndrome
MUTYH-associated polyposis
Juvenile polyposis syndrome
Serrated polyposis syndrome
Li–Fraumeni syndrome