title: PURE ANDROGEN-PRODUCING ADRENAL TUMOR: CLINICAL FEATURES AND PATHOGENESIS.
abstract: OBJECTIVE
  Pure androgen-secreting adrenal tumors (PASATs) are extremely rare, most reports involving only a single case. This study examined 9 cases of PASAT, with an attempt to characterize its clinical features and to explore the pathogenesis.
  
  
  METHODS
  Clinical data of 9 patients with PASAT were retrospectively reviewed. Immunostaining was conducted, and the aryl hydrocarbon receptor-interacting protein gene (AIP) was amplified and directly sequenced.
  
  
  RESULTS
  The onset age of the patients ranged from 3.5 to 64 years. All 8 female patients had virilization, whereas the 7-year-old male patient presented with sexual precocity. Serum testosterone levels were elevated (4.1 to 52.3 nmol/L). Adrenal masses were detected and removed in all patients and histologically diagnosed as adrenocortical adenoma or carcinoma. Two patients had both PASATs and growth hormone (GH)-secreting pituitary adenomas (GH pituitary adenoma). Immunohistochemistry revealed nuclear immunoreactivity for p53 in 3 of 7 patients and nuclear immunoreactivity for cyclin D1 in 2 of 7 patients. Immunostaining of β-catenin showed nuclear, cytoplasmic, and membrane immunoreactivity (2 of 7 patients) or merely cytoplasmic immunoreactivity (1 of 7 patients). The adrenocortical carcinoma showed positive staining for both p53 and cyclin D1 and a high Ki-67 index of 60%. Mutations p.Lys177Argfs*19 and p.Asp287Val in the AIP gene were identified in PASATs of the 2 patients with concomitant presence of GH pituitary adenoma.
  
  
  CONCLUSIONS
  Clinical features of PASATs vary with gender and age of the patients. Abnormal p53 and β-catenin expression might be involved in the tumorigenesis of these tumors. AIP mutations might be responsible for the concomitant presence of PASATs and GH pituitary adenoma.
  
  
  BACKGROUND
  ACA = adrenocortical adenoma ACC = adrenocortical carcinoma AIP = aryl hydrocarbon receptor-interacting protein DHEAS = dehydroepiandrosterone sulfate; GH growth hormone PASAT = pure androgen-secreting adrenal tumor.
authors.forename: Anli
authors.initials: A
authors.lastname: Tong
authors.forename: Jun
authors.initials: J
authors.lastname: Jiang
authors.forename: Fen
authors.initials: F
authors.lastname: Wang
authors.forename: Chunyan
authors.initials: C
authors.lastname: Li
authors.forename: Yushi
authors.initials: Y
authors.lastname: Zhang
authors.forename: Xueyan
authors.initials: X
authors.lastname: Wu
mesh_terms: D000293:Adolescent; D000306:Adrenal Cortex Neoplasms / Q000235:genetics / Q000378:metabolism* / Q000473:pathology; D018246:Adrenocortical Adenoma / Q000235:genetics / Q000378:metabolism* / Q000473:pathology; D000328:Adult; D000728:Androgens / Q000378:metabolism*; D002648:Child; D002675:Child, Preschool; D004252:DNA Mutational Analysis; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011629:Puberty, Precocious / Q000235:genetics / Q000473:pathology; D012189:Retrospective Studies; D014770:Virilism / Q000235:genetics / Q000473:pathology; D055815:Young Adult
chemical_list: D000728:Androgens