Testosterone-secreting virilizing adrenal adenoma with human chorionic gonadotrophin receptors and 21-hydroxylase deficiency.
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.
Leinonen
P
P
Third Department of Medicine, University of Helsinki, Finland.
Ranta
T
T
Siegberg
R
R
Pelkonen
R
R
Heikkilä
P
P
Kahri
A
A
D000236:Adenoma / Q000097:blood / Q000378:metabolism*; D000310:Adrenal Gland Neoplasms / Q000097:blood / Q000378:metabolism*; D000312:Adrenal Hyperplasia, Congenital; D005260:Female; D006801:Humans; D008875:Middle Aged; D011974:Receptors, LH / Q000378:metabolism*; D013739:Testosterone / Q000097:blood / Q000378:metabolism*; D014407:Tumor Cells, Cultured
D011974:Receptors, LH; D013739:Testosterone