Testosterone-secreting virilizing adrenal adenoma with human chorionic gonadotrophin receptors and 21-hydroxylase deficiency

Clin Endocrinol (Oxf). 1991 Jan;34(1):31-5. doi: 10.1111/j.1365-2265.1991.tb01732.x.

Abstract

A 60-year-old woman was evaluated for persistently elevated serum testosterone concentrations after bilateral ovariectomy. Her serum cortisol, androstenedione, dehydroepiandrosterone sulphate and 17-hydroxyprogesterone levels were normal, and decreased after dexamethasone administration. Those of testosterone (17.8-18.4 nmol/l) were remarkably high (normal range 0.7-2.8 nmol/l), were not suppressed by dexamethasone, but clearly increased after hCG administration (up to 128 nmol/l). Computed tomography revealed an adenoma in the right adrenal gland and adrenal scintigraphy under dexamethasone suppression visualized this adenoma. A right adrenalectomy was performed. (1) The tumour was histologically and ultrastructurally adrenocortical adenoma of zona reticularis cell type. (2) The adenoma tissue contained hCG receptors (198 fmol/g). (3) During tissue culture both ACTH and hCG were capable of maintaining its testosterone production, which was attenuated with time without stimulation. (4) The adenoma tissue did not elaborate 21-hydroxylated steroids in contrast to normal adrenal tissue. Thus the aberrant endocrine behaviour of this gonadotrophin-responsive testosterone-secreting adenoma of adrenal zona reticularis cell origin can be explained by ectopic functional hCG receptors and the lack of 21-hydroxylase activity.

Publication types

  • Case Reports

MeSH terms

  • Adenoma / blood
  • Adenoma / metabolism*
  • Adrenal Gland Neoplasms / blood
  • Adrenal Gland Neoplasms / metabolism*
  • Adrenal Hyperplasia, Congenital*
  • Female
  • Humans
  • Middle Aged
  • Receptors, LH / metabolism*
  • Testosterone / blood
  • Testosterone / metabolism*
  • Tumor Cells, Cultured

Substances

  • Receptors, LH
  • Testosterone