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一名患有分泌促卵泡生成素的垂体大腺瘤的男性,尽管睾酮水平处于正常高值,但仍存在性欲减退的情况。

Low libido despite high normal testosterone levels in a male with an FSH-secreting pituitary macroadenoma.

作者信息

Ghazal Asswad Randa, Khan Muhammad Ilyas, Elizabeth Gilkes Catherine, Daousi Christina, Thondam Sravan Kumar

机构信息

Department of Diabetes and Endocrinology, University Hospital Aintree, Liverpool University Hospitals NHS Foundation Trust, Liverpool, UK.

Department of Neurosurgery, The Walton Centre NHS Foundation Trust, Liverpool, UK.

出版信息

Endocrinol Diabetes Metab Case Rep. 2024 Mar 22;2024(1). doi: 10.1530/EDM-23-0143. Print 2024 Jan 1.

Abstract

SUMMARY

Functioning gonadotroph adenomas with clinical manifestations are extremely rare and the majority of these are FSH-secreting macroadenomas. Clinical symptoms are due to excess gonadotrophins and sex hormones, and these may be present for a long time before the diagnosis of pituitary adenoma is made. We present the case of a 37-year-old Caucasian male with clinical manifestations of an FSH-secreting pituitary macroadenoma. He had sexual dysfunction for a year followed by bilateral testicular pain and enlargement which was initially treated as suspected recurrent epididymitis, but his symptoms did not resolve. He presented a year later with headaches and bilateral superior temporal visual field defects. Brain imaging confirmed a pituitary macroadenoma with optic chiasm compression. Pituitary profile demonstrated an unusually high FSH with high normal LH and normal testosterone level. The patient successfully underwent transsphenoidal hypophysectomy and histology confirmed gonadotroph differentiation and immunoreactivity predominantly with FSH. Gonadotrophin levels and testosterone dropped significantly after surgery, and he was started on testosterone replacement. MR imaging, 2 years post surgery, showed no recurrence of pituitary adenoma. In conclusion, testicular enlargement and hypogonadal symptoms associated with low testosterone levels are recognised features in FSH-secreting pituitary adenomas. Our patient had hypogonadal symptoms but consistently high normal testosterone levels prior to surgery. The reason for low libido despite high testosterone is unclear. Our case highlights the need to suspect such rare underlying pituitary pathology when dealing with unusual combinations of hypogonadal symptoms, testicular enlargement with low or normal testosterone levels.

LEARNING POINTS

Functioning pituitary adenomas that secrete excess follicle-stimulating hormone (FSH) are very rare and often present with symptoms related to pituitary mass effect. Testicular enlargement alongside sexual dysfunction are commonly reported symptoms amongst male patients. Pituitary profile results demonstrate a raised FSH level with either a low, normal, or even high testosterone level which may not always correlate to clinical symptoms. Pituitary pathology should be considered in males presenting with unusual combinations of testicular enlargement and hypogonadal symptoms even with normal testosterone levels.

摘要

摘要

具有临床表现的功能性促性腺激素腺瘤极为罕见,其中大多数是分泌促卵泡生成素(FSH)的大腺瘤。临床症状是由于促性腺激素和性激素过多所致,在垂体腺瘤诊断之前这些症状可能已存在很长时间。我们报告一例37岁白种男性,具有分泌FSH的垂体大腺瘤的临床表现。他有一年的性功能障碍,随后出现双侧睾丸疼痛和肿大,最初被当作疑似复发性附睾炎治疗,但症状未缓解。一年后他出现头痛和双侧颞上象限视野缺损。脑部影像学检查证实为垂体大腺瘤并压迫视交叉。垂体激素检查显示FSH异常升高,LH正常高值,睾酮水平正常。患者成功接受经蝶窦垂体切除术,组织学检查证实为促性腺激素细胞分化,免疫反应主要针对FSH。术后促性腺激素水平和睾酮显著下降,遂开始给予睾酮替代治疗。术后2年的磁共振成像显示垂体腺瘤无复发。总之,睾丸肿大和与低睾酮水平相关的性腺功能减退症状是分泌FSH的垂体腺瘤的公认特征。我们的患者有性腺功能减退症状,但术前睾酮水平一直正常高值。尽管睾酮水平高但性欲低下的原因尚不清楚。我们的病例强调,当处理性腺功能减退症状、睾丸肿大与低或正常睾酮水平的不寻常组合时,需要怀疑这种罕见的潜在垂体病变。

学习要点

分泌过量促卵泡生成素(FSH)的功能性垂体腺瘤非常罕见,常表现出与垂体占位效应相关的症状。睾丸肿大伴性功能障碍是男性患者常见的报告症状。垂体激素检查结果显示FSH水平升高,睾酮水平可低、正常甚至高,这可能并不总是与临床症状相关。即使睾酮水平正常,对于出现睾丸肿大和性腺功能减退症状不寻常组合的男性,也应考虑垂体病变。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/59e4/10959031/f5c3098cc4ea/EDM23-0143fig1.jpg

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