Treatment of gonadotropin dependent precocious puberty due to hypothalamic hamartoma with gonadotropin releasing hormone agonist depot
a Developmental
Endocrinology Unit, São Paulo University Medical School, Brazil, b Radiology Department,
Hospital das Clinicas, São Paulo University Medical School,
Brazil
Correspondence to: Dr B B Mendonca, Hospital das Clinicas, Disciplina de Endocrinologia, Caixa Postal 3671, 01060-970, São Paulo-SP, Brazil.
Accepted 24
September 1998
The gonadotropin releasing hormone (GnRH) secreting
hypothalamic hamartoma (HH) is a congenital malformation consisting of a heterotopic mass of nervous tissue that contains GnRH neurosecretory neurons attached to the tuber cinereum or the floor of the third ventricle. HH is a well recognised cause of gonadotropin dependent precocious puberty (GDPP). Long term data are presented on eight children (five boys and three girls) with GDPP due to HH. Physical signs of puberty were observed before 2 years of age in all patients. At presentation with sexual precocity, the mean height standard deviation (SD) for chronological age was +1.60 (1.27) and the mean
height SD for bone 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 change in six patients who had magnetic
resonance imaging follow up. All patients were treated clinically with
GnRH agonists (GnRH-a). The duration of treatment varied from 2.66 to
8.41 years. Seven of the eight children had satisfactory responses to
treatment, shown by regression of pubertal signs, suppression of
hormonal levels, and improvement of height SD for bone age and
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.
© 1999 by Archives of Disease in Childhood
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