Abstract
Hypothalamic hamartomas, are benigns tumors of central nervous system, related to development of the true sexual precocious puberty, have been more frecuently diagnosed due to improvement of the radiologic technology (computed tomography and MRI scanning).
We studied two children with precocious sexual development (Case 1: boy, onset the development of public hair and growth of the penis at 6 months of age; Case 2: girl with breast development and menarche at 6 months.
In both patients hypothalamic hamartomas were demonstrated by MRI (Case 2) and CT and MRI (case 1).
At the first evaluation we observed enlargenment of the penis (8,0 cm) and a testicular diameter of 2,5 cm in case 1; and b reast stage Tanner II in case 2. Both had a pubertal response to the GnRH stimulation test (100 mg IV), LH 51,4 and 28.3 mUI/ml, respectively and normal levels of DHEAS for age.
The children were treated with GnRH analog depot (D-Trp6-GnRH) in dosage of 1.5 mg IM every month for 36 and 18 months respectively. Gonadotropin secretion was inhibited with use of GnRH analog. Physical signs of puberty did not progress futher, without menstrual periods and with height velocity remained between normal range. Laboratory data showed normalization of testosterone level in case 1 (144,5 to 15 ng/dl).
We conclude that GnRH analog therapy is efficient in patients with true precocious puberty secondary to hyphotalamic hamartoma.
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Domenice, S., Latronico, A., Mendonca, B. et al. 10 GnRH AGONIST THERAPY IN TRUE PRECOCIUS PUBERTY SECONDARY TO HYPOTHALAMIC HAMARTOMA (IHH). Pediatr Res 36, 673 (1994). https://doi.org/10.1203/00006450-199411000-00034
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DOI: https://doi.org/10.1203/00006450-199411000-00034