A SUPER LONG-ACTING LH-RH ANALOG INDUCES REGRESSION OF HYPOTHALAMIC HAMARTOMA ASSOCIATED WITH PRECOCIOUS PUBERTY

We treated a 1-year-old female with a hypothalamic hamartoma and precocious puberty with leuprolide acetate depot, a super long-acting hormone-releasing hormone analogue (Tap-144-SR; [D-Leu(6)-[des-Gly(10)-NH2]-LH-RH ethylamide acetate). The infant's major symptoms were genital bleeding and gynaecomastia. The LH-RH analogue (30 mu g/kg) was injected subcutaneously once every 4 weeks. Clinical and laboratory manifestations of precocious puberty showed marked improvement. A follow-up after 16 months of treatment, the size of the tumour decreased significantly and remained unchanged for 2 years of further follow-up. To the best of our knowledge, this is the first hypothalamic hamartoma case in whom a decrease of tumour size under treatment with LH-RH analogue has been documented. But, because diagnosis of hamartoma is only based on neuroradiological and nor on histological examinations, the possibility of a gangliocytoma cannot be excluded with certainty.