POLIPOSE ADENOMATOSA FAMILIAR: UMA ABORDAGEM ABRANGENTE SOBRE A MANIFESTAÇÃO CLÍNICA, RASTREAMENTO, ABORDAGEM CIRÚRGICA E QUIMIOPREVENÇÃO

Familial Adenomatous Polyposis (FAP) is an autosomal dominant hereditary disease caused by pathogenic variants in the Adenomatous Polyposis Coli (APC) gene. The clinical picture manifests with 100 or more adenomatous colorectal polyps and is 100% associated with the risk of developing colorectal cancer (CRC). Patients with this pathology also have an increased risk of extra-colonic manifestations, such as desmoid tumors, osteomas, duodenal adenomas, including gastric cancer, and thyroid cancer. The treatment to prevent RCC is prophylactic colectomy, which has many complications and interferes with the patient's quality of life. This disease can take two forms: the classic form and the attenuated form. The classic form is represented by the presence of hundreds to thousands of adenomatous polyps in the colon and rectum, which appear in adolescence at small sizes and progressively increase in size. On the other hand, the attenuated form of familial adenomatous polyposis (FAP) manifests itself with few adenomas in the colon, starting later in life and presenting an 80% risk of malignant transformation, as well as an increased risk of extra-colonic manifestations. For this reason, the aim of this work is to discuss FAP so that it is possible to disseminate its knowledge and conduct according to the latest literature. For this reason, the aim of this work is to discuss FAP so that it is possible to disseminate its knowledge and conduct in accordance with the latest literature. An integrative literature review was therefore carried out, using a quantitative, qualitative and descriptive approach and searching the PubMed, Scielo and BVSdatabases with the descriptors: “familial adenomatous polyposis”, “Chemoprevention”, “treatment”. As a result, she was able to understand the basic concept of the disease and how it should be treated and managed on a daily basis.