ROLE OF SURGICAL RESECTION IN THE TREATMENT OF HYPOTHALAMIC HAMARTOMAS CAUSING PRECOCIOUS PUBERTY - REPORT OF 6 CASES

Hypothalamic hamartomas (HHs) are benign tumors that are often associa ted with central precocious puberty. Resection of HHs has been recomme nded 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 th e long-term results of total resection of HHs in two children with cen tral precocious puberty and compare it with medical management in four children. The two surgically treated patients underwent total resecti on of pedunculated HHs at 1.75 (Case 1) and 3.25 years (Case 2) and ha ve received follow-up care for 9 and 8 years, respectively There were no postoperative complications and computerized tomography scanning co nfirmed complete tumor removal in both patients. Both patients subsequ ently 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 stan dard deviations) 5 years after surgery, decreasing adult height predic tion. In one child, GnRHa therapy was initiated 7 years postsurgery. F our 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, inc luding a normalization of growth velocity and reduction in the rate of skeletal maturation. No side effects were noted with decapeptyl treat ment, and one child developed sterile abscesses while receiving Lupron -Depot. The proven efficacy of GnRHa in suppressing puberty and reduci ng bone age advancement lends the authors to advise against surgery as the initial management of central precocious puberty caused by HHs.