A spontaneous and severe hyperstimulation of the ovaries revealing a gonadotroph adenoma

We report an unusual case of a gonadotroph adenoma in a 34-yr-old woman, revealed by a dramatic rise in the plasma estradiol (E-2) concentration (26,800 pmol/L; normal, <370), with nonsuppressed FSH and LH levels (4.9 and 2.4 mIU/mL, respectively). The PRL level was,503 ng/mL. The testosterone and progesterone levels were 7 and 17 nmol/L, respectively. The levels of inhibin alpha, inhibin A, and inhibin B were increased compared to normal values in both the follicular (fp) and luteal (Ip) phases of the menstrual cycle [inhibin alpha 1986 IU/L (fp normal, <700; Ip normal, <1650); inhibin A, 254 pg/mL (fp normal, <20; Ip normal, <120); inhibin B, 246 pg/mL (fp normal, <150; Ip normal, <30 Ip)]. Pituitary magnetic resonance imaging revealed a huge pituitary adenoma. After transphenoidal surgery, the patient presented with pituitary insufficiency and diabetes: insipidus. RT-PCR of the tumor tissue was positive for LH beta, FSH beta, alpha-subunit, and PRL. This case is of particular interest because 1) although the E-2 level was extremely high, the patient did not present with ascitis, suggesting that chronic elevated E-2 does not play a crucial role in the hyperstimulation symptoms; 2) the extreme rise in E-2 was related to the cosecretion of FSH and LH, confirming the two-cell two-gonadotrophin theory; and 3) tthee rise in inhibin B is associated with FSH secretion, whereas the rise in inhibin A is probably due to luteinization.