Surgical Management of Familial Adenomatous Polyposis (FAP) Joji Utsunomiya 1 , Takehira Yamamura 1 , Nobuhisa Gondo 1 1The Second Department of Surgery, Hyogo College of Medicine Keyword: 予防的手術 , IRA , 機能温存的大腸全摘術(RPC) , IAA , IACA pp.585-592
Published Date 1997/3/25
DOI https://doi.org/10.11477/mf.1403105102
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 Hereditary tumors represented by familial adenomatous polyposis (FAP) has provided us with an opportunity to elucidate the etiopathegenesis of neoplastic disorders and to work out a new strategy towards cancer by presymptomatic molecular diagnosis, and medical and surgical prevention. Those patients and their families who are incidentally bear a heavy burden should receive an adequate medical care. At present a major part of the management of FAP is surgical prevention of colorectal cancer which could be fatal to the patient. An Indication for surgical prevention can be logically determined by the risk assessment which includes age, density of polyps and sex. A historical controversy on the preventive surgery for FAP, whether the rectum can be preserved or not, has been settled by the long-term follow-up study of restorative proctocolectomy. Currently, there are three surgical options for young patient with sparse polyposis without cancer, the IRA and treatment of polyposis in the remnant rectum when young followed by the delayed RPC in older age, the RPC with IAA or the RPC with IACA. All of them would be justified but only when performed by the specialists in the specific multidisciprinary centers for FAP or associated facilities where life long postoperative surveillance on the rectal and extracolonic lesions, extensive familial survey and authorized cooperative registration of those family members would be achieved. Surgery is, however, merely a prologue of a life long management of the FAP patients and their families under the specific cancer genetic strategy where the molecular diagnosis and computerized surveillance system would provide us more adequate treatment options including chemical prevention.

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