Identification of gonadotropin-releasing hormone in neurons of a hypothalamic hamartoma in a boy with precocious puberty. 1985

F L Culler, and H E James, and M L Simon, and K L Jones

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.

UI MeSH Term Description Entries
D007029 Hypothalamic Neoplasms Benign and malignant tumors of the HYPOTHALAMUS. Pilocytic astrocytomas and hamartomas are relatively frequent histologic types. Neoplasms of the hypothalamus frequently originate from adjacent structures, including the OPTIC CHIASM, optic nerve (see OPTIC NERVE NEOPLASMS), and pituitary gland (see PITUITARY NEOPLASMS). Relatively frequent clinical manifestations include visual loss, developmental delay, macrocephaly, and precocious puberty. (From Devita et al., Cancer: Principles and Practice of Oncology, 5th ed, p2051) Hypothalamic Tumors,Hypothalamic-Chiasmatic Neoplasms,Hypothalamic-Pituitary Neoplasms,Benign Hypothalamic Neoplasms,Hypothalamic Cancer,Hypothalamic Neoplasms, Benign,Hypothalamic Neoplasms, Malignant,Hypothalamic Teratomas,Hypothalamo-Neurohypophysial Region Neoplasms,Hypothalamus Neoplasms,Malignant Hypothalamic Neoplasms,Neoplasms, Hypothalamic,Neoplasms, Hypothalamic, Benign,Neoplasms, Hypothalamic, Malignant,Neoplasms, Hypothalamic-Chiasmatic,Neoplasms, Hypothalamic-Pituitary,Neoplasms, Hypothalamo-Neurohypophysial Region,Neoplasms, Hypothalamus,Tumors, Hypothalamus,Benign Hypothalamic Neoplasm,Cancer, Hypothalamic,Cancers, Hypothalamic,Hypothalamic Cancers,Hypothalamic Chiasmatic Neoplasms,Hypothalamic Neoplasm,Hypothalamic Neoplasm, Malignant,Hypothalamic Pituitary Neoplasms,Hypothalamic Teratoma,Hypothalamic Tumor,Hypothalamic-Chiasmatic Neoplasm,Hypothalamic-Pituitary Neoplasm,Hypothalamo Neurohypophysial Region Neoplasms,Hypothalamo-Neurohypophysial Region Neoplasm,Hypothalamus Neoplasm,Hypothalamus Tumor,Hypothalamus Tumors,Malignant Hypothalamic Neoplasm,Neoplasm, Benign Hypothalamic,Neoplasm, Hypothalamic,Neoplasm, Hypothalamic-Chiasmatic,Neoplasm, Hypothalamic-Pituitary,Neoplasm, Hypothalamo-Neurohypophysial Region,Neoplasm, Hypothalamus,Neoplasm, Malignant Hypothalamic,Neoplasms, Hypothalamic Chiasmatic,Neoplasms, Hypothalamic Pituitary,Neoplasms, Hypothalamo Neurohypophysial Region,Neoplasms, Malignant Hypothalamic,Teratoma, Hypothalamic,Teratomas, Hypothalamic,Tumor, Hypothalamic,Tumor, Hypothalamus,Tumors, Hypothalamic
D007031 Hypothalamus Ventral part of the DIENCEPHALON extending from the region of the OPTIC CHIASM to the caudal border of the MAMMILLARY BODIES and forming the inferior and lateral walls of the THIRD VENTRICLE. Lamina Terminalis,Preoptico-Hypothalamic Area,Area, Preoptico-Hypothalamic,Areas, Preoptico-Hypothalamic,Preoptico Hypothalamic Area,Preoptico-Hypothalamic Areas
D007124 Immunoenzyme Techniques Immunologic techniques based on the use of: (1) enzyme-antibody conjugates; (2) enzyme-antigen conjugates; (3) antienzyme antibody followed by its homologous enzyme; or (4) enzyme-antienzyme complexes. These are used histologically for visualizing or labeling tissue specimens. Antibody Enzyme Technique, Unlabeled,Enzyme Immunoassay,Enzyme-Labeled Antibody Technique,Immunoassay, Enzyme,Immunoperoxidase Techniques,Peroxidase-Antiperoxidase Complex Technique,Peroxidase-Labeled Antibody Technique,Antibody Enzyme Technic, Unlabeled,Enzyme-Labeled Antibody Technic,Immunoenzyme Technics,Immunoperoxidase Technics,Peroxidase-Antiperoxidase Complex Technic,Peroxidase-Labeled Antibody Technic,Antibody Technic, Enzyme-Labeled,Antibody Technic, Peroxidase-Labeled,Antibody Technics, Enzyme-Labeled,Antibody Technics, Peroxidase-Labeled,Antibody Technique, Enzyme-Labeled,Antibody Technique, Peroxidase-Labeled,Antibody Techniques, Enzyme-Labeled,Antibody Techniques, Peroxidase-Labeled,Enzyme Immunoassays,Enzyme Labeled Antibody Technic,Enzyme Labeled Antibody Technique,Enzyme-Labeled Antibody Technics,Enzyme-Labeled Antibody Techniques,Immunoassays, Enzyme,Immunoenzyme Technic,Immunoenzyme Technique,Immunoperoxidase Technic,Immunoperoxidase Technique,Peroxidase Antiperoxidase Complex Technic,Peroxidase Antiperoxidase Complex Technique,Peroxidase Labeled Antibody Technic,Peroxidase Labeled Antibody Technique,Peroxidase-Antiperoxidase Complex Technics,Peroxidase-Antiperoxidase Complex Techniques,Peroxidase-Labeled Antibody Technics,Peroxidase-Labeled Antibody Techniques,Technic, Enzyme-Labeled Antibody,Technic, Immunoenzyme,Technic, Immunoperoxidase,Technic, Peroxidase-Antiperoxidase Complex,Technic, Peroxidase-Labeled Antibody,Technics, Enzyme-Labeled Antibody,Technics, Immunoenzyme,Technics, Immunoperoxidase,Technics, Peroxidase-Antiperoxidase Complex,Technics, Peroxidase-Labeled Antibody,Technique, Enzyme-Labeled Antibody,Technique, Immunoenzyme,Technique, Immunoperoxidase,Technique, Peroxidase-Antiperoxidase Complex,Technique, Peroxidase-Labeled Antibody,Techniques, Enzyme-Labeled Antibody,Techniques, Immunoenzyme,Techniques, Immunoperoxidase,Techniques, Peroxidase-Antiperoxidase Complex,Techniques, Peroxidase-Labeled Antibody
D008297 Male Males
D009474 Neurons The basic cellular units of nervous tissue. Each neuron consists of a body, an axon, and dendrites. Their purpose is to receive, conduct, and transmit impulses in the NERVOUS SYSTEM. Nerve Cells,Cell, Nerve,Cells, Nerve,Nerve Cell,Neuron
D010901 Pituitary Function Tests Examinations that evaluate functions of the pituitary gland. Pituitary Gland Function Tests,Function Test, Pituitary,Function Tests, Pituitary,Pituitary Function Test,Test, Pituitary Function,Tests, Pituitary Function
D010906 Pituitary Hormone-Releasing Hormones Peptides, natural or synthetic, that stimulate the release of PITUITARY HORMONES. They were first isolated from the extracts of the HYPOTHALAMUS; MEDIAN EMINENCE; PITUITARY STALK; and NEUROHYPOPHYSIS. In addition, some hypophysiotropic hormones control pituitary cell differentiation, cell proliferation, and hormone synthesis. Some can act on more than one pituitary hormone. Hormones, Pituitary Hormone Releasing,Hypophysiotropic Hormones,Hypothalamic Hypophysiotropic Hormone,Hypothalamic Releasing Factor,Hypothalamic Releasing Hormone,Hypothalamic Releasing Hormones,Hormone, Hypothalamic Hypophysiotropic,Hormones, Hypophysiotropic,Hypophysiotropic Hormone, Hypothalamic,Pituitary Hormone Releasing Hormones,Releasing Hormone, Hypothalamic
D011629 Puberty, Precocious Development of SEXUAL MATURATION in boys and girls at a chronological age that is 2.5 standard deviations below the mean age at onset of PUBERTY in the population. This early maturation of the hypothalamic-pituitary-gonadal axis results in sexual precocity, elevated serum levels of GONADOTROPINS and GONADAL STEROID HORMONES such as ESTRADIOL and TESTOSTERONE. Familial Precocious Puberty,Idiopathic Sexual Precocity,Precocious Puberty,Precocious Puberty, Central,Precocious Puberty, Male Limited,Precocious Puberty, Male-Limited,Pubertas Praecox,Sexual Precocity,Testotoxicosis,Central Precocious Puberties,Central Precocious Puberty,Familial Precocious Puberties,Idiopathic Sexual Precocities,Male-Limited Precocious Puberties,Male-Limited Precocious Puberty,Praecox, Pubertas,Precocious Puberties,Precocious Puberties, Central,Precocious Puberties, Familial,Precocious Puberties, Male-Limited,Precocious Puberty, Familial,Precocities, Idiopathic Sexual,Precocities, Sexual,Precocity, Idiopathic Sexual,Precocity, Sexual,Puberties, Central Precocious,Puberties, Familial Precocious,Puberties, Male-Limited Precocious,Puberties, Precocious,Puberty, Central Precocious,Puberty, Familial Precocious,Puberty, Male-Limited Precocious,Sexual Precocities,Sexual Precocities, Idiopathic,Sexual Precocity, Idiopathic
D002675 Child, Preschool A child between the ages of 2 and 5. Children, Preschool,Preschool Child,Preschool Children
D006222 Hamartoma A focal malformation resembling a neoplasm, composed of an overgrowth of mature cells and tissues that normally occur in the affected area. Hamartomas

Related Publications

F L Culler, and H E James, and M L Simon, and K L Jones
March 1999, Archives of disease in childhood,
F L Culler, and H E James, and M L Simon, and K L Jones
July 2016, The Journal of pediatrics,
F L Culler, and H E James, and M L Simon, and K L Jones
January 1977, The New England journal of medicine,
F L Culler, and H E James, and M L Simon, and K L Jones
November 2000, The Journal of clinical endocrinology and metabolism,
F L Culler, and H E James, and M L Simon, and K L Jones
November 1984, The Journal of clinical endocrinology and metabolism,
F L Culler, and H E James, and M L Simon, and K L Jones
January 2004, Pediatric neurosurgery,
F L Culler, and H E James, and M L Simon, and K L Jones
January 1960, Acta psychiatrica Scandinavica,
F L Culler, and H E James, and M L Simon, and K L Jones
February 1981, Pediatrics,
F L Culler, and H E James, and M L Simon, and K L Jones
September 2011, Indian journal of endocrinology and metabolism,
F L Culler, and H E James, and M L Simon, and K L Jones
October 2011, The Korean journal of laboratory medicine,
Copied contents to your clipboard!