Identification of gonadotropin-releasing hormone in neurons of a hypothalamic hamartoma in a boy with precocious puberty.
We have studied a 3 1/12-year-old boy who presented with a hypothalamic mass and precocious puberty. His history suggested a course of isosexual precocity progressing from birth. Gelastic seizures also began at an early age. Endocrine evaluation revealed normal thyroid-stimulating hormone and growth hormone secretion, elevated basal and stimulated prolactin concentrations, and luteinizing hormone responses to sequential intravenous injections of gonadotropin-releasing hormone (GnRH) that were pubertal in pattern and magnitude. A needle biopsy of the mass recovered tissue that contained neurons histologically similar to those found in the normal hypothalamus, and the mass was characterized as a hypothalamic hamartoma. Immunohistochemical staining of this tissue with anti-GnRH antiserum demonstrated positive staining for GnRH immunoreactivity in neurons. This suggests a neurosecretory pathogenesis for the precocious puberty found in patients with hamartomas in the hypothalamic region.
D001707:Biopsy, Needle; D002675:Child, Preschool; D006222:Hamartoma / Q000378:metabolism* / Q000473:pathology; D006801:Humans; D007029:Hypothalamic Neoplasms / Q000378:metabolism* / Q000473:pathology; D007031:Hypothalamus / Q000473:pathology; D007124:Immunoenzyme Techniques; D008297:Male; D009474:Neurons / Q000378:metabolism / Q000648:ultrastructure; D010901:Pituitary Function Tests; D010906:Pituitary Hormone-Releasing Hormones / Q000378:metabolism*; D011629:Puberty, Precocious / Q000378:metabolism* / Q000473:pathology; D014057:Tomography, X-Ray Computed
D010906:Pituitary Hormone-Releasing Hormones