True precocious puberty of a girl with hamartoma of the CNS successfully treated by operation

Abstract

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.