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青春期由分泌人绒毛膜促性腺激素(BhCG)和甲胎蛋白的下丘脑混合性生殖细胞肿瘤所致。

Puberty due to a mixed germ cell tumour of the hypothalamus secreting BhCG and alpha-fetoprotein.

作者信息

Howlett T A, el-Senoussi M, Fox J L, Woodhouse N J

出版信息

Horm Res. 1987;25(1):13-7. doi: 10.1159/000180627.

DOI:10.1159/000180627
PMID:2434402
Abstract

A 14 year-old boy presented with visual impairment due to a large suprasellar tumour secreting beta-human chorionic gonadotrophin (BhCG) and alpha-fetoprotein (alpha FP). He was sexually mature with an advanced bone age of 17 years, and suffering from partial hypopituitarism. Treatment with external radiotherapy resulted in a reduction of tumour size and fall of the serum testosterone BhCG and alpha FP levels. We conclude that pubertal development had been initiated and maintained by ectopic hCG production from his intracerebral mixed germ cell tumour. Patients with tumours in the pineal and suprasellar regions should be screened for elevated levels of BhCG and alpha FP. We suspect that many of these tumours cause precocious puberty by secreting BhCG.

摘要

一名14岁男孩因鞍上巨大肿瘤分泌β-人绒毛膜促性腺激素(BhCG)和甲胎蛋白(αFP)而出现视力障碍。他性成熟,骨龄提前至17岁,且患有部分垂体功能减退。外照射放疗导致肿瘤体积缩小,血清睾酮、BhCG和αFP水平下降。我们得出结论,青春期发育是由其脑内混合性生殖细胞瘤异位产生的hCG启动并维持的。松果体区和鞍上区肿瘤患者应筛查BhCG和αFP水平升高情况。我们怀疑这些肿瘤中有许多通过分泌BhCG导致性早熟。

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引用本文的文献

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Diabetes insipidus and increased serum levels of leptin and lactate-dehydrogenase (LDH) in an adolescent boy with a primary intracranial germinoma. Case report and an endocrinological revaluation of literature.一名患有原发性颅内生殖细胞瘤的青少年男孩出现尿崩症以及血清瘦素和乳酸脱氢酶(LDH)水平升高。病例报告及文献的内分泌学再评估
J Endocrinol Invest. 1999 Jul-Aug;22(7):558-61. doi: 10.1007/BF03343609.
2
Primary intrasellar mixed germ-cell tumor with precocious puberty and diabetes insipidus.伴有性早熟和尿崩症的原发性鞍内混合性生殖细胞肿瘤。
Childs Nerv Syst. 1997 Jan;13(1):42-6. doi: 10.1007/s003810050038.
3
Pineal ganglioglioma with premature thelarche. Report of a case and review of the literature.
Childs Nerv Syst. 1996 Feb;12(2):103-6. doi: 10.1007/BF00819507.