Hypothalamic hamartomas and precocious puberty

Abstract

Ten children, five boys and five girls with true precocious puberty at an early age were found to have hypothalamic hamartomas on brain imaging. Very early onset of puberty, varying from a few weeks to three years of age, and rapid progression were characteristic. Accelerated growth velocity and markedly advanced bone age were evident in all. Gonadotropin and gonadal hormone levels were elevated above the prepubertal range. Six children had associated developmental delay or hyperactivity.

Gonadotrophin and prolactin secretory dynamics in girls with normal puberty, idiopathic precocious puberty and precocious puberty due to hypothalamic hamartoma

“…The increased LH response to LHRH in our patients with hypothalamic hamartoma is consistent with several earlier reports ( Takeuchi et al ., 1979 ; Markin et al ., 1987 ; Colaco et al ., 1993 ; Albright & Lee, 1993), but not with one study of 9 children with hypothalamic hamartoma ( Mahachoklertwattana et al ., 1993 ). Possible explanations for the inconsistency among studies include heterogeneity in the underlying hypothalamic hamartomas or heterogeneity in the distribution of sex and pubertal stage between the comparison groups.…”

Management and outcome of central precocious puberty

“…Highly suggestive signs for the diagnosis of a hypothalamic hamartoma are: (i) precocious onset of pubertal development at a very young age [usually < 4 years; often < 2 years of age (Cassio et al , 2000;Comite et al , 1984;Colaco et al , 1993;Mahachoklertwattana et al , 1993;Kornreich et al , 1995;de Brito et al , 1999;Jung et al , 1999)]; (ii) hormonal findings compatible with central activation of the GnRH pulse generator (i.e. pulsatile gonadotropin secretion and increased LH response in the GnRH stimulation test); (iii) the demonstration of an isointense tumour in typical location showing no gadolinium enhancement on magnetic resonance imaging (MRI) ( Fig.…”