Biomaterial Database

Hypothalamic hamartoma and precocious puberty: report of a case. 1992

P H Hsiao, and W Y Tsai, and J S Lee, and H M Liu, and F J Hsieh

Department of Pediatrics, National Taiwan University Hospital, Taipei, R.O.C.

Hypothalamic hamartoma is reported to be associated with precocious puberty. Here, the authors present a seven-year-old girl whose onset of puberty occurred at the age of two. Under the impression of idiopathic precocious puberty, cyproterone acetate was initially tried. Since the effect of her medication was not satisfactory, it was discontinued at the age of five years and 11 months. However, rapid advance of bone age and vaginal spotting recurred after the withdrawal of treatment. She was re-evaluated at the age of six, and a magnetic resonance image (MRI) study of the head revealed a hypothalamic hamartoma. At that time, a long-acting analog of luteinizing hormone-releasing hormone (LHRHa), leuprolide acetate, was prescribed. Her secondary sex characteristics regressed and her hypothalamic-pituitary-gonad axis was suppressed after treatment. The clinical presentation, mechanism and treatment of precocious puberty caused by hypothalamic hamartomas are fully discussed in this report.

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
D008279	Magnetic Resonance Imaging	Non-invasive method of demonstrating internal anatomy based on the principle that atomic nuclei in a strong magnetic field absorb pulses of radiofrequency energy and emit them as radiowaves which can be reconstructed into computerized images. The concept includes proton spin tomographic techniques.	Chemical Shift Imaging,MR Tomography,MRI Scans,MRI, Functional,Magnetic Resonance Image,Magnetic Resonance Imaging, Functional,Magnetization Transfer Contrast Imaging,NMR Imaging,NMR Tomography,Tomography, NMR,Tomography, Proton Spin,fMRI,Functional Magnetic Resonance Imaging,Imaging, Chemical Shift,Proton Spin Tomography,Spin Echo Imaging,Steady-State Free Precession MRI,Tomography, MR,Zeugmatography,Chemical Shift Imagings,Echo Imaging, Spin,Echo Imagings, Spin,Functional MRI,Functional MRIs,Image, Magnetic Resonance,Imaging, Magnetic Resonance,Imaging, NMR,Imaging, Spin Echo,Imagings, Chemical Shift,Imagings, Spin Echo,MRI Scan,MRIs, Functional,Magnetic Resonance Images,Resonance Image, Magnetic,Scan, MRI,Scans, MRI,Shift Imaging, Chemical,Shift Imagings, Chemical,Spin Echo Imagings,Steady State Free Precession MRI
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
D002648	Child	A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL.	Children
D005260	Female		Females
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