Biomaterial Database

Precocious puberty and hypothalamic hamartoma. 1981

H I Hochman, and D M Judge, and S Reichlin

A 3-year-old boy with true precocious puberty and hamartoma of the hypothalamus is described. Preliminary diagnosis was established by the presence of unusually advanced puberty and skeletal age without other evidence of central nervous system dysfunction, elevated blood gonadotropin and testosterone concentrations, and positive computed tomography scans. Pneumoencephalography further delineated the tumor. Hypothalamic-pituitary-gonadal and adrenal functions were studied. Release of gonadotropins after the injection of synthetic luteinizing hormone-releasing hormone and the suppression of luteinizing hormone and of testosterone by oral fluoxymesterone were comparable to maximal responses of normal men. The diagnosis was confirmed by histologic and electron microscopic study of an excised portion of the tumor. Immunofluorescence studies indicated the presence of luteinizing hormone-releasing hormone in the hamartoma and radioimmunoassays detected thyroid-stimulating hormone-releasing hormone and somatostatin. The hamartoma resembles an autonomously functioning accessory hypothalamus. The ectopic neuroendocrine pathways, however, are responsive to hormonal stimulation and feedback. The clinical, endocrinologic, and pathologic features of published cases of precocious puberty and hypothalamic hamartoma are reviewed. Advanced or rapidly progressive true precocious puberty in the very young with elevated concentrations of blood gonadotropins and gonadal steroids and positive pneumoencephalography appear to be characteristic.

UI	MeSH Term	Description	Entries
D007028	Hypothalamic Hormones	Peptide hormones produced by NEURONS of various regions in the HYPOTHALAMUS. They are released into the pituitary portal circulation to stimulate or inhibit PITUITARY GLAND functions. VASOPRESSIN and OXYTOCIN, though produced in the hypothalamus, are not included here for they are transported down the AXONS to the POSTERIOR LOBE OF PITUITARY before being released into the portal circulation.	Hypothalamic Pituitary-Regulating Hormones,Hypothalamic Pituitary-Regulating Peptides,Hormones, Hypothalamic,Hormones, Hypothalamic Pituitary-Regulating,Hypothalamic Pituitary Regulating Hormones,Hypothalamic Pituitary Regulating Peptides,Peptides, Hypothalamic Pituitary-Regulating,Pituitary-Regulating Hormones, Hypothalamic,Pituitary-Regulating Peptides, Hypothalamic
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
D008297	Male		Males
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
D011863	Radioimmunoassay	Classic quantitative assay for detection of antigen-antibody reactions using a radioactively labeled substance (radioligand) either directly or indirectly to measure the binding of the unlabeled substance to a specific antibody or other receptor system. Non-immunogenic substances (e.g., haptens) can be measured if coupled to larger carrier proteins (e.g., bovine gamma-globulin or human serum albumin) capable of inducing antibody formation.	Radioimmunoassays
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
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D014057	Tomography, X-Ray Computed	Tomography using x-ray transmission and a computer algorithm to reconstruct the image.	CAT Scan, X-Ray,CT Scan, X-Ray,Cine-CT,Computerized Tomography, X-Ray,Electron Beam Computed Tomography,Tomodensitometry,Tomography, Transmission Computed,X-Ray Tomography, Computed,CAT Scan, X Ray,CT X Ray,Computed Tomography, X-Ray,Computed X Ray Tomography,Computerized Tomography, X Ray,Electron Beam Tomography,Tomography, X Ray Computed,Tomography, X-Ray Computer Assisted,Tomography, X-Ray Computerized,Tomography, X-Ray Computerized Axial,Tomography, Xray Computed,X Ray Computerized Tomography,X Ray Tomography, Computed,X-Ray Computer Assisted Tomography,X-Ray Computerized Axial Tomography,Beam Tomography, Electron,CAT Scans, X-Ray,CT Scan, X Ray,CT Scans, X-Ray,CT X Rays,Cine CT,Computed Tomography, Transmission,Computed Tomography, X Ray,Computed Tomography, Xray,Computed X-Ray Tomography,Scan, X-Ray CAT,Scan, X-Ray CT,Scans, X-Ray CAT,Scans, X-Ray CT,Tomographies, Computed X-Ray,Tomography, Computed X-Ray,Tomography, Electron Beam,Tomography, X Ray Computer Assisted,Tomography, X Ray Computerized,Tomography, X Ray Computerized Axial,Transmission Computed Tomography,X Ray Computer Assisted Tomography,X Ray Computerized Axial Tomography,X Ray, CT,X Rays, CT,X-Ray CAT Scan,X-Ray CAT Scans,X-Ray CT Scan,X-Ray CT Scans,X-Ray Computed Tomography,X-Ray Computerized Tomography,Xray Computed Tomography