title: Ectopic cortisol-producing adrenocortical adenoma in the renal hilum: histopathological features and steroidogenic enzyme profile.
abstract: Ectopic cortisol-producing adrenocortical adenomas (CPA) are extremely rare, and only four cases have previously been reported so far but the tumors were not ultrastructurally studied. Presented in this paper is the fifth case with ectopic CPA which was extensively examined to gain deeper insights in terms of the histopathological features and steroidogenic enzyme profile of the tumor. A 53-year-old woman complained of accidental discovery of left renal mass. She had a 5-year history of hypertension, weight gain, moon face, thin skin and systemic edema. These symptoms completely relieved after the tumor removal. Two years later, the above symptoms recurred, and a recurrent tumor was revealed in left renal hilum. The tumor was removed completely with relief of her symptoms of Cushing's syndrome. Histologically and ultrastructurally, the tumor was composed of compact cells and clear cells, and the former was prominent, suggesting an active secretory function of the tumor. The adenoma tissue showed a strong immunostaining for Melan-A, 3beta-hydroxysteroid dehydrogenase (HSD3B2) and 17alpha-hydroxylase1 (CYP17A1). Expression pattern for 11beta-hydroxylase 1 (CYP11B1), 11beta-hydroxylase 2 (CYP11B2), CYP17 and HSD3B2 mRNA in ectopic CPA was similar to that in the adrenal CPA. In conclusion, in terms of histopathological characteristic and steroidogenic enzyme profile, ectopic CPA is similar to adrenal CPA, suggesting that they are of identical cell origin.
authors.affiliation: Department of Endocrinology, Key Laboratory of Endocrinology, Ministry of Health, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences Beijing 100730, China.
authors.forename: Anli
authors.initials: A
authors.lastname: Tong
authors.affiliation: Department of Endocrinology, The First Hospital of Yulin Shanxi 719000, China.
authors.forename: Aihua
authors.initials: A
authors.lastname: Jia
authors.affiliation: Chinese Academy of Medical Sciences & Peking Union Medical College Beijing 100730, China.
authors.forename: Shujie
authors.initials: S
authors.lastname: Yan
authors.affiliation: Department of Radiology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences Beijing 100730, China.
authors.forename: Yan
authors.initials: Y
authors.lastname: Zhang
authors.affiliation: Department of Urology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences Beijing 100730, China.
authors.forename: Yi
authors.initials: Y
authors.lastname: Xie
authors.affiliation: Department of Urology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences Beijing 100730, China.
authors.forename: Guanghua
authors.initials: G
authors.lastname: Liu
mesh_terms: D018246:Adrenocortical Adenoma / Q000201:enzymology* / Q000378:metabolism / Q000473:pathology*; D014408:Biomarkers, Tumor / Q000032:analysis; D003480:Cushing Syndrome / Q000209:etiology; D005260:Female; D006801:Humans; D006854:Hydrocortisone / Q000378:metabolism*; D007150:Immunohistochemistry; D007680:Kidney Neoplasms / Q000201:enzymology* / Q000378:metabolism / Q000473:pathology*; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local / Q000473:pathology; D060888:Real-Time Polymerase Chain Reaction; D013254:Steroid 17-alpha-Hydroxylase / Q000378:metabolism
chemical_list: D014408:Biomarkers, Tumor; C501892:CYP17A1 protein, human; D013254:Steroid 17-alpha-Hydroxylase; D006854:Hydrocortisone
keywords: Cushing’s syndrome; Ectopic adrenocortical adenoma; steroidogenic enzyme; ultrastructure