title: Aldosterone-producing adrenocortical carcinoma with prominent hepatic metastasis diagnosed by liver biopsy: a case report.
abstract: BACKGROUND
  Aldosterone-producing adrenocortical carcinoma is a rare malignancy, which is usually diagnosed by histopathological examination of the excised tumor. In inoperable cases, aldosterone-producing ACC diagnosed by immunohistochemical staining of the metastatic tumor for Cytochrome P450 (CYP) 11β has not previously been reported and even in that case staining for adrenocortical-specific adrenal 4 binding protein/steroidogenic factor1 (Ad4BP/SF1) and steroidogenic enzymes has not been reported.
  
  
  METHODS
  We report the case of a 67-year-old Japanese woman with aldosterone-producing adrenocortical carcinoma. Laboratory findings showed severe hypopotassemia. Endocrinological examination revealed an increased plasma aldosterone concentration and suppressed plasma renin activity. Plasma dehydroepiandrosterone sulfate (DHEA-S) was elevated. Diurnal variation in serum cortisol was lost and administration of 1 mg and 8 mg dexamethasone did not suppress serum cortisol levels. From the 24-h urine collection sample, urine aldosterone and urine cortisol levels were greatly increased. Therefore, autonomous excess production was observed for the three adrenal cortex hormones. Abdominal computed tomography and magnetic resonance imaging showed a right adrenal tumor and a huge liver tumor. Adrenocortical carcinoma with metastatic liver cancer was strongly suggested, however surgery could not be considered due to stage IV disease: the liver tumor was too large and cardiac ultrasonography indicated that her cardiac function was poor. Therefore, a liver biopsy was taken to properly determine the diagnosis. Immunohistochemical stains for Ad4BP/SF1 and steroidogenic enzymes were positive. Ad4BP/SF-1 was originally identified as a steroidogenic, tissue-specific transcription factor implicated in the expression of the steroidogenic CYP gene encoding cytochrome P450s. Hence we could diagnose the patient as having adrenocortical carcinoma with metastatic liver cancer.
  
  
  CONCLUSIONS
  This rare case had severe hypopotassemia accompanied with not only increased cortisol and DHEA-S but also aldosterone. We reached the diagnosis of adrenocortical carcinoma with metastatic liver cancer based on positive immunohistochemical staining of Ad4BP/SF1 in the liver biopsy specimen. We have reported the first case of aldosterone-producing adrenocortical carcinoma diagnosed solely by immunohistochemical staining for adrenocortical-specific Ad4BP/SF1 and steroidogenic enzymes in a metastatic liver tumor.
authors.affiliation: Division of Diabetes, Metabolism and Endocrinology, Department of Internal Medicine, the Jikei University School of Medicine, 3-25-8 Nishishinbashi, Minato-ku, Tokyo, 105-8461, Japan. nm4koohs@yahoo.co.jp.
authors.forename: Kennosuke
authors.initials: K
authors.lastname: Ohashi
authors.affiliation: Division of Diabetes, Metabolism and Endocrinology, Department of Internal Medicine, the Jikei University School of Medicine, 3-25-8 Nishishinbashi, Minato-ku, Tokyo, 105-8461, Japan. takeshihayashi@jikei.ac.jp.
authors.forename: Takeshi
authors.initials: T
authors.lastname: Hayashi
authors.affiliation: Division of Diabetes, Metabolism and Endocrinology, Department of Internal Medicine, the Jikei University School of Medicine, 3-25-8 Nishishinbashi, Minato-ku, Tokyo, 105-8461, Japan. m-sakamoto@umin.ac.jp.
authors.forename: Masaya
authors.initials: M
authors.lastname: Sakamoto
authors.affiliation: Division of Diabetes, Metabolism and Endocrinology, Department of Internal Medicine, the Jikei University School of Medicine, 3-25-8 Nishishinbashi, Minato-ku, Tokyo, 105-8461, Japan. domaine.iuchi@gmail.com.
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Iuchi
authors.affiliation: Division of Diabetes, Metabolism and Endocrinology, Department of Internal Medicine, the Jikei University School of Medicine, 3-25-8 Nishishinbashi, Minato-ku, Tokyo, 105-8461, Japan. hiro1020@jikei.ac.jp.
authors.forename: Hirofumi
authors.initials: H
authors.lastname: Suzuki
authors.affiliation: Division of Diabetes, Metabolism and Endocrinology, Department of Internal Medicine, the Jikei University School of Medicine, 3-25-8 Nishishinbashi, Minato-ku, Tokyo, 105-8461, Japan. tebisawa@jikei.ac.jp.
authors.forename: Takanori
authors.initials: T
authors.lastname: Ebisawa
authors.affiliation: Division of Diabetes, Metabolism and Endocrinology, Department of Internal Medicine, the Jikei University School of Medicine, 3-25-8 Nishishinbashi, Minato-ku, Tokyo, 105-8461, Japan. tojo.k@jikei.ac.jp.
authors.forename: Katsuyoshi
authors.initials: K
authors.lastname: Tojo
authors.affiliation: Department of Pathology, Tohoku University School of Medicine, 2-1 Seiryo-machi, Aoba-ku, Sendai, Miyagi, 980-8575, Japan. hsasano@patholo2.med.tohoku.ac.jp.
authors.forename: Hironobu
authors.initials: H
authors.lastname: Sasano
authors.affiliation: Division of Diabetes, Metabolism and Endocrinology, Department of Internal Medicine, the Jikei University School of Medicine, 3-25-8 Nishishinbashi, Minato-ku, Tokyo, 105-8461, Japan. kazu-utsunomiya@jikei.ac.jp.
authors.forename: Kazunori
authors.initials: K
authors.lastname: Utsunomiya
mesh_terms: D000306:Adrenal Cortex Neoplasms / Q000378:metabolism / Q000473:pathology*; D018268:Adrenocortical Carcinoma / Q000378:metabolism / Q000473:pathology*; D000368:Aged; D000450:Aldosterone / Q000378:metabolism*; D001706:Biopsy; D005260:Female; D006801:Humans; D008099:Liver / Q000473:pathology*; D008113:Liver Neoplasms / Q000556:secondary*; D047368:Tumor Burden
chemical_list: D000450:Aldosterone