title: Hepatocellular adenoma associated with familial adenomatous polyposis coli.
abstract: Hepatocellular adenoma (HCA) is a benign liver tumor that most frequently occurs in young women using oral contraceptives. We report a rare case of HCA in a 29 years old female with familial adenomatous polyposis (FAP). The first proband was her sister, who underwent a total colectomy and was genetically diagnosed as FAP. A tumor, 3.0 cm in diameter, was detected in the right lobe of the liver during a screening study for FAP. A colonoscopy and gastroendoscopy revealed numerous adenomatous polyps without carcinoma. The patient underwent a total colectomy and ileo-anal anastomosis and hepatic posterior sectoriectomy. The pathological findings of the liver tumor were compatible with HCA. The resected specimen of the colon revealed multiple colonic adenomatous polyps. Examination of genetic alteration revealed a germ-line mutation of the adenomatous polyposis coli (APC) gene. Inactivation of the second APC allele was not found. Other genetic alterations in the hepatocyte nuclear factor 1 alpha and β-catenin gene, which are reported to be associated with HCA, were not detected. Although FAP is reported to be complicated with various neoplasias in extracolic organs, only six cases of HCA associated with FAP, including the present case, have been reported. Additional reports will establish the precise mechanisms of HCA development in FAP patients.
authors.affiliation: Keisuke Inaba, Department of Surgery, Iwata City Hospital, Iwata 438-8500, Japan.
authors.forename: Keisuke
authors.initials: K
authors.lastname: Inaba
authors.forename: Takanori
authors.initials: T
authors.lastname: Sakaguchi
authors.forename: Kiyotaka
authors.initials: K
authors.lastname: Kurachi
authors.forename: Hiroki
authors.initials: H
authors.lastname: Mori
authors.forename: Hong
authors.initials: H
authors.lastname: Tao
authors.forename: Toshio
authors.initials: T
authors.lastname: Nakamura
authors.forename: Yasuo
authors.initials: Y
authors.lastname: Takehara
authors.forename: Satoshi
authors.initials: S
authors.lastname: Baba
authors.forename: Masato
authors.initials: M
authors.lastname: Maekawa
authors.forename: Haruhiko
authors.initials: H
authors.lastname: Sugimura
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Konno
keywords: Adenomatous polyposis coli gene; Extrahepatic manifestation; Familial adenomatous polyposis coli; Hepatic adenoma; Hepatocyte nuclear factor 1 alpha