Development of rectal cancer in a young patient with a retained rectum after total colectomy for Familial Adenomatous Polyposis

Familial Adenomatous polyposis (FAP) is an inherited disorder and it represents the most common gastro intestinal Polyposis syndrome. The mutation of germ line in the APC gene is the main cause for FAP. The FAP is an inherited syndrome characterized by multiple (more than hundred) polyps in the colorectum with high risk of colorectal carcinoma and existence of extra colonic manifestations. Cancer prevention and maintaining a good quality of life are the main goals of management. The recommended surgical modalities are total proctocolectomy and ileoanal pouch or ileorectal anastomosis for AFAP.