The development of<unk>  <unk>  is the result of multiple genetic mutations. Genes involved in the pathogenesis of<unk>  include APC and MUTYH genes. Many of patients have a positive family history of colorectal cancer or polyps. However, some of them have no previous family history.<unk>  is associated with different malignancies including stomach cancer, periampullary cancer, pancreatic cancer, hepatoblastoma, bile duct cancer, papillary thyroid cancer, and medulloblastoma. Duodenal adenoma, adrenal masses, desmoid tumor, osteomas, congenital hypertrophy of the retinal pigment epithelium, epidermoid cysts, and fibromas are also associated with<unk> .<unk>  may be classified according to severity into three subtypes which include profuse, intermediate, and attenuated.<unk>  has less severe variants, including Gardner's syndrome and Turcot syndrome.<unk>  must be differentiated from other diseases that lead to the formation of multiple gastrointestinal polyps, such as Peutz–Jeghers syndrome, juvenile<unk>  syndrome, Cowden syndrome, Carney syndrome, and hereditary non–polyposis colon cancer (Lynch syndrome).<unk>  is a rare disease that affects individuals worldwide. The incidence and prevalence of<unk>  is approximately 3-20 and 12 per 100,000 individuals, respectively. Screening for<unk>  by genetic testing and/or colonoscopy is recommended among patients with history of multiple colonic adenomas and family history of<unk> .<unk>  is a rare disease that usually starts from childhood. Patients develop hundreds to thousands colon polyps till early twenties. If left untreated, almost all of them develop colorectal cancer around 40 years of age. The majority of patients with<unk>  are asymptomatic till the development ofcolorectal cancer. Common symptoms of<unk>  are gastrointestinal bleeding, pain, and altered bowel habits. patients may feel fatigued after occult bleeding. Colonoscopy is considered as a gold standard for evaluating the intestine, as well as for diagnostic and therapeutic approaches. Tissue biopsy and polypectomy may be done during colonoscopy. Surgery is the mainstay of treatment for<unk> . The preferred technique of surgery is laparoscopic total proctocolectomy with ileal pouch anal anastomosis(IPAA) and mucosectomy. Effective measures for the primary prevention of<unk>  include genetic counseling. The most common method to detect a mutation is direct sequencing of the APC gene. All patients with<unk>  will develop colorectal cancer. For secondary prevention of colorectal cancer total colectomy must be done. Effective tests for the secondary prevention of other complications and associated conditions include annual pouchoscopy following surgery and annual physical examination and ultrasound.
Familial adenomatous polyposis
Hereditary nonpolyposis colorectal cancer
Peutz-Jeghers syndrome
Fabry's disease
Cretinism
Ataxia telangiectasia
Neurofibromatosis type 1
Pickering syndrome