Snyder P J
Department of Medicine, University of Pennsylvania School of Medicine, Philadelphia.
Endocrinol Metab Clin North Am. 1987 Sep;16(3):755-64.
The frequency of gonadotroph cell adenomas among all unselected pituitary adenomas is likely much higher than previously suspected. The prevalence in one series of 139 men with pituitary macroadenomas was 17 per cent (24 per cent if adenomas secreting only alpha subunit are included). The clinical characteristics of patients with gonadotroph cell adenomas are similar. Most are middle-aged men who have a history of normal pubertal development and a normal fertility history and by examination are normally virilized and have testes of normal size. They are brought to medical attention because of visual impairment, which is the result of the enormous size of the adenoma. The most common hormonal characteristics of gonadotroph cell adenomas in vivo is hypersecretion of FSH, which is often accompanied by hypersecretion of FSH-beta and alpha subunits and less often by hypersecretion of LH-beta or intact LH. Another common characteristic is secretion of FSH and/or LH-beta in response to TRH. A few patients with gonadotroph cell adenomas hypersecrete intact LH and therefore have supranormal serum testosterone concentrations. A larger number have secondary hypogonadism, because the adenomas are not secreting intact LH but are compressing the normal gonadotroph cells and impairing LH secretion. These patients have concentrations of intact LH that are not elevated, despite subnormal testosterone concentrations. The testosterone increases markedly in response to human chorionic gonadotropin. Both the clinical and hormonal characteristics of gonadotroph cell adenomas usually make them readily distinguishable from pituitary enlargement due to long-standing primary hypogonadism. Most gonadotroph cell adenomas are now managed first by transsphenoidal surgery to attempt to restore vision as quickly as possible, and then by supervoltage radiation to prevent regrowth of the remaining adenomatous tissue. Surgery usually does improve vision, as well as the pretreatment hormonal abnormalities, and radiation reduces FSH hypersecretion further. Dopamine agonist therapy is experimental but warrants further trial. The hormonal abnormalities detected prior to treatment, such as supranormal basal concentrations of FSH, alpha, and FSH-beta and the FSH and LH-beta responses to TRH, can be used to monitor the response to therapy.
在所有未经筛选的垂体腺瘤中,促性腺激素细胞腺瘤的发生率可能比之前怀疑的要高得多。在一组139例垂体大腺瘤男性患者中,其患病率为17%(若包括仅分泌α亚基的腺瘤,则为24%)。促性腺激素细胞腺瘤患者的临床特征相似。大多数是中年男性,有青春期发育正常和生育史正常的病史,经检查通常有正常的男性化表现且睾丸大小正常。他们因视力损害而就医,这是腺瘤巨大所致。促性腺激素细胞腺瘤在体内最常见的激素特征是促卵泡生成素(FSH)分泌过多,常伴有FSH-β和α亚基分泌过多,较少伴有促黄体生成素-β(LH-β)或完整促黄体生成素(LH)分泌过多。另一个常见特征是对促甲状腺激素释放激素(TRH)有反应时分泌FSH和/或LH-β。少数促性腺激素细胞腺瘤患者分泌完整的LH,因此血清睾酮浓度超常。更多患者有继发性性腺功能减退,因为腺瘤不分泌完整的LH,而是压迫正常的促性腺激素细胞并损害LH分泌。尽管睾酮浓度低于正常,但这些患者的完整LH浓度并未升高。给予人绒毛膜促性腺激素后,睾酮会显著升高。促性腺激素细胞腺瘤的临床和激素特征通常使其很容易与因长期原发性性腺功能减退导致的垂体增大区分开来。现在大多数促性腺激素细胞腺瘤首先通过经蝶窦手术进行治疗,以尽快恢复视力,然后进行超高压放疗以防止剩余腺瘤组织再生。手术通常确实能改善视力以及治疗前的激素异常,放疗可进一步降低FSH分泌过多。多巴胺激动剂治疗尚处于试验阶段,但值得进一步试验。治疗前检测到的激素异常,如FSH、α和FSH-β的基础浓度超常以及FSH和LH-β对TRH的反应,可用于监测治疗反应。