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鞍上-松果体区生殖细胞瘤掩盖中枢性尿崩症和低促性腺激素性性腺功能减退症

Masking of central diabetes insipidus and hypogonadotrophic hypogonadism by germ cell tumour in suprasellar--pineal region.

作者信息

Isa S H Md, Wong M, Khalid B A K

机构信息

School of Medicine and Health Sciences, Monash University Malaysia, No. 20 & 22, Jalan PJS 11/5, Bandar Sunway, 46150 Petaling Jaya, Selangor.

出版信息

Med J Malaysia. 2006 Dec;61(5):630-2.

Abstract

A patient with beta hCG-secreting germ cell carcinoma of the pineal and suprasellar regions presented with hydrocephalus, Parinaud's syndrome, hypopituitarism and polyuria. Central diabetes insipidus was strongly suspected although the water deprivation test was not diagnostic. The polyuria however, responded to ADH analogue when the hypothyroidism and hypocortisolism were treated. Pubertal development was evident and serum testosterone was normal despite the low FSH/LH, suggesting hCG stimulation of Leydig cells. This case illustrates that a beta hCG-germ cell tumour of the suprasellar region causing hypopituitarism can mask the presence of central diabetes insipidus and hypogonadotrophic hypogonadism.

摘要

一名患有松果体区和鞍上区β人绒毛膜促性腺激素(β-hCG)分泌性生殖细胞癌的患者,出现了脑积水、帕里诺德综合征、垂体功能减退和多尿症状。尽管禁水试验未确诊,但强烈怀疑存在中枢性尿崩症。然而,在治疗甲状腺功能减退和皮质醇减少症后,多尿症状对抗利尿激素(ADH)类似物有反应。青春期发育明显,尽管促卵泡生成素(FSH)/促黄体生成素(LH)水平较低,但血清睾酮正常,提示β-hCG刺激了睾丸间质细胞。该病例表明,鞍上区的β-hCG生殖细胞肿瘤导致垂体功能减退时,可掩盖中枢性尿崩症和低促性腺激素性性腺功能减退的存在。

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