Biomaterial Database

[Hypothalamic hamartoma with precocious puberty--a case report]. 1985

I Date, and Y Yagyu, and S Mino, and T Ohhashi

A case of hypothalamic hamartoma with precocious puberty is presented and the literature of reported cases is reviewed. An 8-year-old boy was admitted to our hospital because of precocious puberty and mental retardation. His genital development was Tanner's stage 4 and pubic hair was Tanner's stage 3. Bone age was 11 years. Plain CT showed an isodense mass in the suprasellar cistern which was not enhanced following contrast administration. Metrizamide CT cisternography showed a filling defect in the suprasellar cistern. Endocrinological evaluation revealed high levels of serum luteinizing hormone (LH) and testosterone with a marked response of LH to LH-RH injection. A left frontotemporal craniotomy was performed and the tumor was partially removed. The tumor was gray, firm and well-circumscribed with poor vascularity. Postoperatively, a right oculomotor palsy and transient diabetes insipidus developed. He was discharged ambulatory one month later. Serum LH and testosterone returned to normal and the response of LH to LH-RH injection became normal. Hamartoma was diagnosed on histological examination. Electron micrographic study showed numerous dense granules with approximately 0.1 mu in diameter, in which Judge proved LH-RH by immunofluorescent study in 1977. Our case supports the hypothesis that hypothalamic hamartoma may cause precocious puberty by autonomous secretion of LH-RH and we consider that neurosurgical treatment is recommended.

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
D007034	Hypothalamus, Posterior	The part of the hypothalamus posterior to the middle region consisting of several nuclei including the medial maxillary nucleus, lateral mammillary nucleus, and posterior hypothalamic nucleus (posterior hypothalamic area). The posterior hypothalamic area is concerned with control of sympathetic responses and is sensitive to conditions of decreasing temperature and controls the mechanisms for the conservation and increased production of heat.	Hypothalamic Region, Posterior,Posterior Hypothalamic Region,Area Hypothalamica Posterior,Hypothalamus Posterior,Mammillary Region,Posterior Hypothalamus,Posterior Periventricular Nucleus,Premammillary Nucleus,Supramammillary Commissure,Supramammillary Nucleus,Area Hypothalamica Posteriors,Commissure, Supramammillary,Commissures, Supramammillary,Hypothalamic Regions, Posterior,Hypothalamica Posterior, Area,Hypothalamica Posteriors, Area,Hypothalamus Posteriors,Mammillary Regions,Nucleus, Posterior Periventricular,Nucleus, Premammillary,Nucleus, Supramammillary,Periventricular Nucleus, Posterior,Posterior Hypothalamic Regions,Posterior, Area Hypothalamica,Posterior, Hypothalamus,Posteriors, Area Hypothalamica,Posteriors, Hypothalamus,Region, Mammillary,Region, Posterior Hypothalamic,Regions, Mammillary,Regions, Posterior Hypothalamic,Supramammillary Commissures
D007118	Immunoassay	A technique using antibodies for identifying or quantifying a substance. Usually the substance being studied serves as antigen both in antibody production and in measurement of antibody by the test substance.	Immunochromatographic Assay,Assay, Immunochromatographic,Assays, Immunochromatographic,Immunoassays,Immunochromatographic Assays
D007986	Luteinizing Hormone	A major gonadotropin secreted by the adenohypophysis (PITUITARY GLAND, ANTERIOR). Luteinizing hormone regulates steroid production by the interstitial cells of the TESTIS and the OVARY. The preovulatory LUTEINIZING HORMONE surge in females induces OVULATION, and subsequent LUTEINIZATION of the follicle. LUTEINIZING HORMONE consists of two noncovalently linked subunits, alpha and beta. Within a species, the alpha subunit is common in the three pituitary glycoprotein hormones (TSH, LH and FSH), but the beta subunit is unique and confers its biological specificity.	ICSH (Interstitial Cell Stimulating Hormone),Interstitial Cell-Stimulating Hormone,LH (Luteinizing Hormone),Lutropin,Luteoziman,Luteozyman,Hormone, Interstitial Cell-Stimulating,Hormone, Luteinizing,Interstitial Cell Stimulating Hormone
D007987	Gonadotropin-Releasing Hormone	A decapeptide that stimulates the synthesis and secretion of both pituitary gonadotropins, LUTEINIZING HORMONE and FOLLICLE STIMULATING HORMONE. GnRH is produced by neurons in the septum PREOPTIC AREA of the HYPOTHALAMUS and released into the pituitary portal blood, leading to stimulation of GONADOTROPHS in the ANTERIOR PITUITARY GLAND.	FSH-Releasing Hormone,GnRH,Gonadoliberin,Gonadorelin,LH-FSH Releasing Hormone,LHRH,Luliberin,Luteinizing Hormone-Releasing Hormone,Cystorelin,Dirigestran,Factrel,Gn-RH,Gonadorelin Acetate,Gonadorelin Hydrochloride,Kryptocur,LFRH,LH-RH,LH-Releasing Hormone,LHFSH Releasing Hormone,LHFSHRH,FSH Releasing Hormone,Gonadotropin Releasing Hormone,LH FSH Releasing Hormone,LH Releasing Hormone,Luteinizing Hormone Releasing Hormone,Releasing Hormone, LHFSH
D008297	Male		Males
D011184	Postoperative Period	The period following a surgical operation.	Period, Postoperative,Periods, Postoperative,Postoperative Periods
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
D011859	Radiography	Examination of any part of the body for diagnostic purposes by means of X-RAYS or GAMMA RAYS, recording the image on a sensitized surface (such as photographic film).	Radiology, Diagnostic X-Ray,Roentgenography,X-Ray, Diagnostic,Diagnostic X-Ray,Diagnostic X-Ray Radiology,X-Ray Radiology, Diagnostic,Diagnostic X Ray,Diagnostic X Ray Radiology,Diagnostic X-Rays,Radiology, Diagnostic X Ray,X Ray Radiology, Diagnostic,X Ray, Diagnostic,X-Rays, Diagnostic
D002648	Child	A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL.	Children