Multiple hepatic adenomas caused by long-term administration of androgenic steroids for aplastic anemia in association with familial adenomatous polyposis

Nakao, Atsunori; Sakagami, K; Nakata, Yusei; Komazawa, Katsutaka; Amimoto, Tatsuya; Nakashima, Koichiro; Isozaki, Hiroshi; Takakura, Norihisa; Tanaka, Noriaki

doi:10.1007/s005350070081

Cited by 85 publications

(50 citation statements)

References 23 publications

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“…Almost all hepatic adenomas arise in one of three clinical settings: (1) reproductive age women, usually with a history of oral contraceptive pill (OCP) therapy (1), (2) excess androgen exposure such as in patients on androgen steroid therapy for Fanconi's anemia (2,3), and (3) glycogen storage disease Types I and III (4). Rare cases of hepatic adenomas have also been reported in a number of other clinical settings.…”

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confidence: 99%

Hepatic Adenomas: Analysis of Sex Steroid Receptor Status and the Wnt Signaling Pathway

et al. 2002

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Hepatic adenomas are strongly linked to excess hormonal exposure, but little else is known about their pathogenesis. The Wnt signaling pathway, which is activated in both hepatocellular carcinomas and hepatoblastomas, has not been studied in hepatic adenomas. Fifteen hepatic adenomas were studied by immunohistochemistry for estrogen, progesterone, and androgen receptors (ER, PR, AR, respectively) and correlated with the results of immunostaining for ␤-catenin. Direct sequencing was performed to look for mutations in key genes involved in the Wnt signaling pathway: Exon 3 of ␤-catenin encompassing the glycogen synthase kinase 3␤ (GSK-3␤) phosphorylation region and the mutational cluster region of the adenomatosis polyposis coli protein (APC). Analysis for loss of heterozygosity (LOH) at chromosome 5q was also performed. Immunostaining for both ER and PR was present in 11/15 (73%) adenomas, and staining with one hormone receptor was positively associated with staining for the other receptor. AR positivity was present in 3/15 cases. Nuclear accumulation of ␤-catenin was present in 7/15 (46%) of adenomas, indicating activation of the Wnt signaling pathway. However, no ␤-catenin mutations, no APC mutations in the mutational cluster region, and no 5q LOH were detected. Two APC polymorphisms of unknown significance were seen. No clear association between ␤-catenin nuclear accumulation and hormone receptor positivity was discerned. Activation of the Wnt signaling pathway appears to be important in a subset of hepatic adenomas but does not result from common ␤-catenin or APC mutations and does not appear to be directly linked to hormonal receptor status.

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confidence: 99%

Hepatic Adenomas: Analysis of Sex Steroid Receptor Status and the Wnt Signaling Pathway

et al. 2002

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“…According to our literature review, only seven cases, including our case, have been reported (Table 1) [5,[10][11][12][13][14] .…”

Section: Discussionmentioning

confidence: 96%

“…Gastroendoscopy also found thick polyps without carcinoma. On the basis of clinical features [15] and previous literature [10][11][12][13] , we performed a total colectomy and ileoanal anastomosis and hepatic posterior sectoriectomy on December 2008. Macroscopically, numerous polyps of various sizes, including one lateral spreading tumor in the ascending colon, were found in the mucosal surface of the resected colon specimen.…”

Section: Case Reportmentioning

confidence: 99%

Hepatocellular adenoma associated with familial adenomatous polyposis coli

Sakaguchi¹,

Kurachi²,

Mori³

et al. 2012

WJH

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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.

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“…Although most adenomas are solitary, in 20-30% of cases multiple adenomas can be found [2]. It is associated with the use of exogenous sex steroids including oral contraceptives and anabolic androgens [3,4]. One of the complications of hepatic adenoma is intraperitoneal hemorrhage from rupture.…”

Section: Discussionmentioning

confidence: 99%

Orthotopic Liver Transplantation for Hepatic Adenoma in a Patient with Portal Vein Agenesis

Sibulesky¹,

Taner²,

Willingham³

et al. 2009

TOTRANSJ

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Congenital portal vein agenesis, known as the Abernathy malformation, was first described in 1793. It is associated with focal nodular hyperplasia and adenomas due to abnormal hepatic regeneration secondary to abnormal or absent flow to the liver. We report a case of a 25-year-old Caucasian female with a complicated medical history including idiopathic membranoproliferative glomerulonephritis, hypertension, hyperlipidemia, Sweet's syndrome, acute febrile neutrophilic dermatosis, who incidentally was diagnosed with multiple liver nodules, the largest 14 cm in diameter, arising at the junction of the left and right hepatic lobes. She was also noted to have a congenital absence of the portal vein. There was no evidence of cirrhosis or portal hypertension. Liver function tests and -fetoprotein were within normal limits. A biopsy of the largest mass revealed hepatocellular adenoma. Because of the size, location, and multiplicity of the lesions, we elected to proceed with orthotopic liver transplantation. She subsequently underwent deceased donor liver transplantation using the piggyback technique. Despite dividing the portal vein shunt after completion of the caval anastomosis and minimizing the portal vein clamp time, her spleen spontaneously ruptured, for which splenectomy was performed. The patient did well postoperatively with good liver function.

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Multiple hepatic adenomas caused by long-term administration of androgenic steroids for aplastic anemia in association with familial adenomatous polyposis

Cited by 85 publications

References 23 publications

Hepatic Adenomas: Analysis of Sex Steroid Receptor Status and the Wnt Signaling Pathway

Hepatic Adenomas: Analysis of Sex Steroid Receptor Status and the Wnt Signaling Pathway

Hepatocellular adenoma associated with familial adenomatous polyposis coli

Orthotopic Liver Transplantation for Hepatic Adenoma in a Patient with Portal Vein Agenesis

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