Hypothalamic hamartomas (HHs) are benign
tumors that are often associated with
central precocious puberty. Resection of HHs has been recommended as a treatment option for selected cases of pedunculated lesions, especially in young children. The role of surgery has to be evaluated in the light of the availability of effective medical treatment with
gonadotropin-releasing hormone analogs (GnRHas). The authors report the long-term results of total resection of HHs in two children with
central precocious puberty and compare it with medical management in four children. The two surgically treated patients underwent total resection of pedunculated HHs at 1.75 (Case 1) and 3.25 years (Case 2) and have received
follow-up care for 9 and 8 years, respectively. There were no postoperative complications and computerized tomography scanning confirmed complete
tumor removal in both patients. Both patients subsequently experienced some regression of secondary sexual characteristics. The response of
luteinizing hormone to
GnRH became prepubertal in one patient and was diminished in the other. However, the growth velocity remained elevated (> 7 cm/year), bone age remained advanced (> +2 standard deviations) 5 years after surgery, decreasing adult height prediction. In one child, GnRHa
therapy was initiated 7 years postsurgery. Four children were treated solely with
GnRH agonists and have received
follow-up care for 2.3, 6, 9, and 9 years, respectively. These patients have had a complete regression of endocrinological abnormalities, including a normalization of growth velocity and reduction in the rate of skeletal maturation. No side effects were noted with
decapeptyl treatment, and one child developed sterile
abscesses while receiving
Lupron-Depot. The proven efficacy of GnRHa in suppressing puberty and reducing bone age advancement leads the authors to advise against surgery as the initial management of
central precocious puberty caused by HHs.