Adolescent ; Adult ; Child ; Child, Preschool ; Female ; Humans ; Hypothalamic Neoplasms ; pathology ; radiotherapy ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Pituitary Neoplasms ; pathology ; radiotherapy

Brain Neoplasms ; complications ; Humans ; Hypothalamic Diseases ; etiology ; Syndrome

BACKGROUND: Gs alpha gene mutation, that constitutively increases intracellular cAMP, is found in some acromegalic patients. It was demonstrated that increased intracellular cAMP levels suppress the expression of rat TRH receptor (TRH-R) mRNA. We previously demonstrated that transient expression of a mutant Gs alpha gene suppress the rat TRH-R gene expression in the cultured rat growth hormone-secreting tumor cell line (GH3), whereas TRH-R gene expression in adenomas with Gs alpha gene mutation (gsp oncogene) did not differ from that in tumors without the mutation. The discrepancy suggests the possibilities that the effect of permanent expression of mutant Gs alpha gene on TRH-R gene expression is different from that of transient expression of the mutant gene and hypothalamic hormones including TRH regulate the gene expression. METHODS: We investigated whether permanent expression of the mutant-type Gs alpha does not suppress the TRH receptor gene expression in GH3 cells, and whether TRH suppresses the gene expression by using reverse transcription-polymerase chain reaction (RT-PCR) and in vitro transcription. RESULTS: Permanent expression of a mutant-type Gs alpha increased basal cAMP levels up to 1.7-fold relative to the controls, whereas the wild-type cell line did not show increased cAMP levels. Permanent expression of a mutant-type Gs alpha increased TRH receptor mRNA level up to 2.8 fold compared with the controls. Treatment of the permanently transfected GH3 cells with TRH suppressed TRH-R gene expression more prominently compared to the wild type GH3 cells. CONCLUSION: These results suggest that permanent expression of mutant Gs alpha enhances the expression of TRH-R in GH-secreting pituitary tumors with gsp oncogene, but the gene expression may also be regulated by other factors including TRH.
Acromegaly ; Adenoma ; Animals ; Cell Line ; Cell Line, Tumor ; Gene Expression* ; GTP-Binding Proteins ; Humans ; Hypothalamic Hormones ; Oncogenes ; Pituitary Neoplasms ; Rats ; Receptors, Thyrotropin-Releasing Hormone ; RNA, Messenger

Extra ventricular neurocytoma (EVN) is a rare brain tumor with histologic features similar with a central neurocytoma, but located outside of the ventricular system. In this study, we present an unusual case of hypothalamic EVN in a 14-year-old patient. The patient underwent subtotal removal and had tumor relapse. The patient was then treated using intensity modulated radiation therapy, and the tumor remained stable for 24 months. This case report may be important in that this is the first pediatric case of EVN located in the hypothalamic region. EVN has similar radiologic features with pilocytic astrocytomas and therefore a hypothalamic EVN may be misdiagnosed as a hypothalamic glioma. Also, the pathologic-radiologic-clinical correlation of EVN located in the hypothalamic area may be different from that of EVNs originating from other usual sites.
Adolescent ; Astrocytoma ; Brain Neoplasms ; Glioma ; Humans ; Hypothalamic Neoplasms ; Neurocytoma* ; Radiotherapy, Adjuvant* ; Recurrence

Extra ventricular neurocytoma (EVN) is a rare brain tumor with histologic features similar with a central neurocytoma, but located outside of the ventricular system. In this study, we present an unusual case of hypothalamic EVN in a 14-year-old patient. The patient underwent subtotal removal and had tumor relapse. The patient was then treated using intensity modulated radiation therapy, and the tumor remained stable for 24 months. This case report may be important in that this is the first pediatric case of EVN located in the hypothalamic region. EVN has similar radiologic features with pilocytic astrocytomas and therefore a hypothalamic EVN may be misdiagnosed as a hypothalamic glioma. Also, the pathologic-radiologic-clinical correlation of EVN located in the hypothalamic area may be different from that of EVNs originating from other usual sites.
Adolescent ; Astrocytoma ; Brain Neoplasms ; Glioma ; Humans ; Hypothalamic Neoplasms ; Neurocytoma* ; Radiotherapy, Adjuvant* ; Recurrence

Diencephalic syndrome is a rare cause of failure to thrive in infancy and early childhood. The syndrome is characterized by profound emaciation with normal appetite, loss of cutaneous adipose tissue, hyperactivity, euphoria, and nystagmus. It commonly occurs in association with chiasmatic and hypothalamic gliomas. It has also been described in association with other histologic types. There is the marked increase of serum growth hormone, which may exhibit an inappropriate, even paradoxical response in stimulation test. A male infant of 12 months of age, showed markedly elevated growth hormone but he had failure to thrive findings. Evenly enhanced round mass was seen at suprasella area in brain CT. Its histological findings was "Desmoplastic infantile ganglioglioma", very rare histologic type. Here we report a case of diencephalic syndrome presented by failure to thrive in association with hypothalamic tumors.
Adipose Tissue ; Appetite ; Brain ; Emaciation ; Euphoria ; Failure to Thrive ; Glioma ; Growth Hormone ; Humans ; Hypothalamic Neoplasms ; Infant ; Male

Anatomical lesions of hypothalamic area associated with hypodipsic hypernatremia have been reported only rarely. We report here a case of hypodipsic hypernatremia induced by a hypothalamic lesion. A 25-yr-old man, who had been treated with radiation for hypothalamic tumor 5-yr before, was admitted for evaluation of hypernatremia and hypokalemia. He never felt thirst despite the elevated plasma osmolality and usually refused to drink intentionally. Plasma arginine vasopressin (AVP) level was normal despite the severe hypernatremic hyperosmolar state and urine was not properly concentrated, while AVP secretion was rapidly induced by water deprivation and urine osmolality also progressively increased to the near maximum concentration range. All of these findings were consistent with an isolated defect in osmoregulation of thirst, which was considered as the cause of chronic hypernatremia in the patient without an absolute deficiency in AVP secretion. Hypokalemia could be induced by activation of the renin-angiotensin-aldosterone system as a result of volume depletion. However, inappropriately low values of plasma aldosterone levels despite high plasma renin activity could not induce symptomatic hypokalemia and metabolic alkalosis. The relatively low serum aldosterone levels compared with high plasma renin activity might result from hypernatremia. Hypernatremia and hypokalemia were gradually corrected by intentional water intake only.
Adult ; Argipressin/*secretion ; Case Report ; Human ; Hypernatremia/*etiology ; Hypothalamic Neoplasms/*metabolism ; Male ; Osmolar Concentration ; *Thirst

Various types CNS lesions bring about precocious puberty and mostly these lesions involve the hypothalamus. We experienced two cases of hypothalamic hamartomas and a case of hypothalamic astrocytoma, presenting sexual precocity as the initial symptom. Other clinical findings included gelastic seizure, behavioral disturbance, accelerated bony growth. MRI of the hamartoma showed high signal intensities on T2 weighted spin echo image. The two hypothalamic hamartomas were partially removed and the patients showed some improvements endocrinologically and clinically one month after the operation. Literatures about the hypothalamic hemartoma were reviewed and the problems especially in the extent of surgical removal in the hypothalamic hamartomas were discussed.
Astrocytoma ; Hamartoma ; Humans ; Hypothalamic Neoplasms* ; Hypothalamus ; Magnetic Resonance Imaging ; Puberty, Precocious* ; Seizures

The effects of pulsatile GnRH therapy have been studied for the treatment of different forms of hypogonadotropic hypogonadism, including idiopathic hypogonadotropic hypogonadism and hypogonadism developed as a result of treatment with combination of surgery and irradiation for pituitary or hypothalamic tumor. GnRH was administered subcutaneously in a dose of 10ug every 2 hours with the pulsatile infusion pump. With GnRH therapy, all patients improved secretion of LH, FSH and testosterone. Testicular volumes increased. Spermatogenesis was induced in 4 patients. Pulsatile GnRH therapy is an effective treatment for idiopathic hypogonadotropic hypogonadism and can have a role in hypogonadism previously treated with combination of surgery and irradiation for pituitary or hypothalamic tumor.
Gonadotropin-Releasing Hormone* ; Humans ; Hypogonadism* ; Hypothalamic Neoplasms ; Infusion Pumps ; Male* ; Spermatogenesis ; Testosterone

The effects of pulsatile GnRH therapy have been studied for the treatment of different forms of hypogonadotropic hypogonadism, including idiopathic hypogonadotropic hypogonadism and hypogonadism developed as a result of treatment with combination of surgery and irradiation for pituitary or hypothalamic tumor. GnRH was administered subcutaneously in a dose of 10ug every 2 hours with the pulsatile infusion pump. With GnRH therapy, all patients improved secretion of LH, FSH and testosterone. Testicular volumes increased. Spermatogenesis was induced in 4 patients. Pulsatile GnRH therapy is an effective treatment for idiopathic hypogonadotropic hypogonadism and can have a role in hypogonadism previously treated with combination of surgery and irradiation for pituitary or hypothalamic tumor.
Gonadotropin-Releasing Hormone* ; Humans ; Hypogonadism* ; Hypothalamic Neoplasms ; Infusion Pumps ; Male* ; Spermatogenesis ; Testosterone