A paradoxical inhibition of androgenic hyperproduction by a Sertoli-Leydig cell tumour ovary.

A 17 year old woman was evaluated for amenorrhoea and severe hirsutism (Ferriman-Gallway index = 31). Pelvic ultrasound demonstrated a right unilateral ovarian mass (6 x 5 cm), whereas the computed tomography for the adrenal gland was normal. Endocrinological findings revealed normal concentrations of oestradiol, progesterone, dihydroepiandrosterone sulphate, cortisol, prolactin, follicle-stimulating hormone, luteinizing hormone and adrenocorticotrophic hormone (ACTH). Total testosterone, free testosterone, androstenedione and 17-hydroxy-progesterone concentrations, already elevated at basal conditions, did not increase after an ACTH test, whereas they decreased significantly after dexamethasone administration and increased after a human chorionic gonadotrophin test. Of all the tumour markers investigated, tissue polypeptide antigen and alpha 1-fetoprotein showed an increase in concentration. Selective venous ovarian catheterization indicated the presence of an androgen-producing tumour in the right ovary. The histopathological diagnosis was consistent with a Sertoli-Leydig cell tumour ranking between an intermediate and a poor grade of differentiation, with heterologous elements characterized by mucinous epithelium of the gastro-intestinal type. An endocrine evaluation performed postoperatively showed a normalization of all serum pathological hormones and tumour markers studied. Some particular aspects were focused on and discussed.