Treatment of gonadotropin dependent precocious puberty due to hypothalamichamartoma with gonadotropin releasing hormone agonist depot

The gonadotropin releasing hormone (GnRH) secreting hypothalamic hamartoma (HH) is a congenital malformation consisting of a heterotopic mass of nervo us tissue that contains GnRH neurosecretory neurons attached to the tuber c inereum or the floor of the third ventricle. HH is a well recognised cause of gonadotropin dependent precocious puberty (GDPP). Long term data are pre sented on eight children (five boys and three girls) with GDPP due to HH. P hysical signs of puberty were observed before 2 years of age in all patient s. At presentation with sexual precocity, the mean height standard deviatio n (SD) for chronological age was +1.60 (1.27) and the mean height SD for bo ne age was -0.92 (1.77). Neurological symptoms were absent at presentation and follow up. The hamartoma diameter ranged from 5 to 18 mm and did not ch ange in six patients who had magnetic resonance imaging follow up. All pati ents were treated clinically with GnRH agonists (GnRH-a). The duration of t reatment varied from 2.66 to 8.41 years. Seven of the eight children had sa tisfactory responses to treatment, shown by regression of pubertal signs, s uppression of hormonal levels, and improvement of height SD for bone age an d predicted height. One patient had a severe local reaction to GnRH-a with failure of hormonal suppression and progression of pubertal signs. It seems that HH is benign and that GnRH-a treatment provides satisfactory and safe control for most children with GDPP due to HH.