Hadjilambris K, Fahlbusch R, Heinze E
Eur J Pediatr. 1986 Apr;145(1-2):148-50. doi: 10.1007/BF00441880.
A girl with precocious puberty due to a hypothalamic hamartoma is presented. At the age of 0.41 years vaginal bleeding was documented and signs of puberty were noted: PHIII, BII according to Tanner. The bone age was 1.3 years, and height velocity rose from the 50th to 90th percentile. Plasma concentrations of LH (5.85 mU/ml), FSH (3.29 mU/ml), growth hormone (30 ng/ml), and oestradiol (90 pg/ml) were elevated. The results of a neurological examination including an EEC recording as well as a skull roentgenogram, were unremarkable. The visual evoked potentials were grossly abnormal. A native and contrast CT scan visualized a tumour close to the suprasellar cisterna reaching the chiasma opticum. At the age of 1.2 years the tumours was removed. Histologically the tissue was identified as a hamartoma. Immediately after the operation vaginal bleeding ceased, pubertal development regressed, bone age did not advance any further, the visual evoked potentials normalized and the contrast CT did not show any tumour mass. The levels of LH, FSH, growth hormone and oestradiol 4 months post operation were decreased as follow: LH: 1.14 mU/ml, FSH: 0.70 mU/ml, GH: 15.1 ng/ml, oestradiol: 10 pg/ml. However, there was an increase of FSH (3 mU/ml) 1 year after the operation. No secondary sexual characters reappeared.
本文报告一名因下丘脑错构瘤导致性早熟的女孩。该女孩0.41岁时出现阴道出血，并伴有性早熟体征：根据坦纳分期为PHIII、BII期。骨龄为1.3岁，身高增长速度从第50百分位升至第90百分位。血浆促黄体生成素（LH，5.85 mU/ml）、促卵泡生成素（FSH，3.29 mU/ml）、生长激素（30 ng/ml）和雌二醇（90 pg/ml）浓度升高。包括脑电图记录及颅骨X线检查在内的神经系统检查结果均无异常。视觉诱发电位明显异常。头颅平扫及增强CT扫描显示，鞍上池附近有一肿瘤，累及视交叉。患儿1.2岁时行肿瘤切除术。组织学检查确诊为错构瘤。术后阴道出血立即停止，青春期发育消退，骨龄未再进展，视觉诱发电位恢复正常，增强CT未显示肿瘤残留。术后4个月，LH、FSH、生长激素和雌二醇水平下降如下：LH：1.14 mU/ml，FSH：0.70 mU/ml，GH：15.1 ng/ml，雌二醇：10 pg/ml。然而，术后1年FSH水平升高至3 mU/ml。未再出现第二性征。
