Wide L, Lundberg P O
J Clin Endocrinol Metab. 1981 Nov;53(5):923-30. doi: 10.1210/jcem-53-5-923.
Pituitary tumors producing FSH have hitherto been reported only in males, all of whom have had normal or raised LH levels in serum. This report describes a female with a pituitary adenoma associated with supranormal serum levels of FSH. The FSH was also qualitatively abnormal when compared with FSH in the serum of other postmenopausal women, had a lower apparent molecular weight on gel chromatography, and was less negatively charged, as shown by electrophoresis. The results of LRH tests and suppression tests with ethinyl estradiol indicated autonomy of the FSH-producing adenoma. The FSH level increased concomitant with tumor enlargement and decreased after surgical removal of the pituitary adenoma or pituitary irradiation. The serum level of the glycoprotein alpha-subunit was raised about 100-fold. Any free FSH beta-subunits were not detectable in serum. The abnormal FSH had antigenic sites in common with both the alpha- and beta-subunits of FSH. The LH level was extremely low, and there was no response to LRH tests or ethinyl estradiol treatment. After gel chromatography, a small amount of LH, corresponding to 1/50th of the average for the patient's age, was detected at the position for normal LH. There was no GH response to insulin-induced hypoglycemia, while the cortisol increase was normal. Thyroid and adrenal functions were normal. The PRL level was within the normal range and increased slightly after estrogen treatment.
迄今,仅在男性中报道过产生促卵泡激素(FSH)的垂体肿瘤,所有这些男性血清中的促黄体生成素(LH)水平均正常或升高。本报告描述了一名患有垂体腺瘤且血清FSH水平超常的女性。与其他绝经后女性血清中的FSH相比,该患者的FSH在质量上也存在异常,在凝胶色谱上的表观分子量较低,并且如电泳所示,带负电荷较少。促性腺激素释放激素(LRH)试验和炔雌醇抑制试验的结果表明,产生FSH的腺瘤具有自主性。FSH水平随肿瘤增大而升高,在手术切除垂体腺瘤或垂体放疗后降低。糖蛋白α亚基的血清水平升高了约100倍。血清中未检测到任何游离的FSHβ亚基。异常的FSH与FSH的α亚基和β亚基都有共同的抗原位点。LH水平极低,对LRH试验或炔雌醇治疗无反应。凝胶色谱后,在正常LH的位置检测到少量LH,相当于患者年龄平均值的1/50。对胰岛素诱导的低血糖无生长激素(GH)反应,而皮质醇升高正常。甲状腺和肾上腺功能正常。催乳素(PRL)水平在正常范围内,雌激素治疗后略有升高。