AIM:To study the early diagnosis and management of familialadenomatous polyposis (FAP).METHODS:Eight pedigrees of FAP were collected and theirpedigree trees were protracted.Clinical characteristics andtreatment outcomes of FAP patients in these kindreds wereanalysed.RESULTS:A total of 157 members were investigated ineight kindreds and 25 patients with FAP were diagnosed.The ratio of male patients and female patients was 16:9and the average age at onset was 38 years.Among them,six patients died of cancer with a mortality rate of 28%,and 36% (9/25) FAP patients were diagnosed as synchronouscolorectal cancer on the basis of FAP.A proband wasdiagnosed as synchronous colorectal cancer with livermetastasis and died 11 mo later after partial colectomy andhepatic metastatic lesion biopsy.The other seven probandsreceived total abdominal colectomy and rectal mucosectomywith ileal pouch-anal anastomosis (IPAA),and one of themwas diagnosed as synchronous colon cancer on the basisof FAP and was still alive after 7.5 years follow-up.Amongthe other seven patients with synchronous colorectal canceron the basis of FAP underwent total abdominal colectomywith ileorectal anastomosis (IRA),one underwent totalremnant rectum resection and ileostomy for recurrentcarcinoma in the retained rectum 2.5 years later after theIRA and was still alive,while the others all died of recurrencewith a median survival time of 4.6 years.Through closefollow-up and termly endoscopic surveillance,three FAPpatients were detected before presenting symptoms at theage of 18,20 and 23 years,respectively.Prophylactic IPAAwas performed and results were satisfactory after thepatients were followed-up for 6,1,and 8 years,respectively.CONCLUSION:Pedigree investigation,close follow-up andtermly endoscopic surveillance are very important for earlydetection of FAP.Prophylactic IPAA can give satisfactoryresults to FAP patients. AIM: To study the early diagnosis and management of familialadenomatous polyposis (FAP). METHODS: Eight pedigrees of FAP were collected and their pedigree trees were protracted. Clinical features and treatment outcomes of FAP patients in these kindreds were analyzed .RESULTS: A total of 157 members were investigated ineight kindreds and 25 patients with FAP were diagnosed. The ratio of male patients and female patients was 16: 9 and the average age at onset was 38 years. Among them, six patients died of cancer with a mortality rate of 28%, and 36 % (9/25) FAP patients were diagnosed as synchronous colorectal cancer on the basis of FAP. A proband wasdiagnosed as synchronous colorectal cancer with liver metastasis and died 11 mo later after partial colectomy and hepatic metastatic lesion biopsy. The other seven probandsreceived total abdominal colectomy and rectal mucosectomy with ileal pouch-anal anastomosis (IPAA), and one of them was diagnosed as synchronous colon cancer on the basis of FAP and was sti The remaining seven patients with synchronous colorectal cancer on the basis of FAP underwent total abdominal colectomy with ileorectal anastomosis (IRA), one underwent total remnants rectum resection and ileostomy for recurrentcarcinoma in the retained rectum 2.5 years later after the IRA and was still alive, while the others all died of recurrence with a median survival time of 4.6 years. Through close follow-up and termly endoscopic surveillance, three FAPpatients were detected before presenting symptoms at the age of 18, 20 and 23 years, respectively. Prophylactic IPAA was performed and results were satisfactory after the patients were followed-up for 6,1, and 8 years, respectively. CONCLUSION: Pedigree investigation, close follow-up andtermly endoscopic surveillance are very important for early detection of FAP. Prophylactic IPAA can give satisfactory results to FAP patients.