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PubMed1985

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

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.

Child↗
PubMedDate not supplied

Altered specificity of the blood-cerebrospinal fluid barrier for pituitary hormones in patients with tumoral hypothalamo-hypophyseal diseases as proved by releasing hormones stimulation.

Growth hormone (GH), prolactin (PRL) and gonadotropins (LH and FSH) of cerebrospinal fluid (CSF) and serum were radioimmunologically measured in basal condition in 75 cases and during the stimulation with releasing hormones in 24 of cases, i.e. with TRH plus LRH i.v. Samples were obtained from 75 patients of the following groups: A. control group of 9 patients without endocrine diseases; B. 8 patients with nontumoral diseases, of whom 4 with organic cranial diabetes insipidus and 4 with empty sella syndromes; C. 36 patients with invasive tumors i.e. 6 hypothalamic metastatic carcinomas and 300 invasive pituitary adenomas; D. 22 patients with enclosed tumors, i.e. 1 suprasellar dermoid cyst and 21 enclosed pituitary adenomas. Minimum 3 pituitary hormones were measured in each analyzed sample, and only those hormones, not secreted by the tumoral cells, were analyzed for their differential penetration into the CSF. An increase of the permeability of the blood-CSF barrier (BCB) was frequently observed in the group of invasive tumors and occasionally in the other tumoral or non-tumoral groups. The serum/CSF ratios of gonadotropins become significantly lower than the ratios of GH and PRL in the invasive groups (p less than 0.02), and here the CSF level was correlated to the increases of the serum levels during LRH plus TRH i.v. test only for gonadotropins. These data suggest that the specificity of BCB is not lost, but altered by the tumors of the hypothalamo-hypophyseal region, with an increase of the permeability for gonadotropins more than for GH or PRL.

Adult↗
PubMed1983

[Precocious puberty associated with Von Recklinghausen's disease. Developmental follow-up under cyproterone acetate treatment].

Authors describe a case of a boy aged 8-10/12 years with precocious puberty associated with Von Recklinghausen's disease. He was treated with cyproterone acetate (100 mg/m2/24 h oral doses) and controlled for a period of two years. He shows very few effects of puberal physical signs and improvement of his sexual behavioral area. They have evaluated growth evolution by different parameters and couldn't appreciate any improvement because of the advanced bone age (13-6/12 years) when treatment was started.

Adult↗
PubMed1984

Radiologic findings in a hypothalamic hamartoma.

This report presents a case of a hypothalamic hamartoma in a 4-month-old boy. Ultrasound demonstrated the tumor and its effect on the ventricular system. Computed tomography and ventriculography confirmed these findings and were complementary in the diagnosis. To the author's best knowledge, this is the first ultrasound documentation of a hypothalamic hamartoma.

Cerebral Ventriculography↗
PubMed1984

Treatment of sexual precocity by removal of a luteinizing hormone-releasing hormone secreting hamartoma.

A 1-year-old infant was initially seen with precocious puberty that progressed rapidly and was associated with episodic gonadotropin release. A computed tomographic metrizamide cisternogram showed a pedunculated mass below the tuber cinereum; the mass was resected. His pubertal development regressed, his accelerated growth ceased, and his hormone levels returned to the normal prepubertal range. Immunocytochemical studies of the tissue demonstrated luteinizing hormone-releasing hormone (LHRH) granules in the perikaryon of neurons and axons in the hamartoma and axons connecting the hamartoma to the tuber cinereum. These data suggest that such lesions cause pubertal development by stimulating gonadotropin secretion via episodic release of LHRH. If such lesions are pedunculated, operative resection may be the treatment of choice.

Gonadotropin-Releasing Hormone↗
PubMed1983

[Hypothalamic hamartoma and gelastic crises. Apropos of 7 cases].

Seven cases of hypothalamic hamartomas with gelastic seizures are reported. A precocious puberty was found in 4 cases. The normal neurologic examination and lack of sign of intracranial hypertension were in contrast with the severity of the epileptic seizures, of the mental impairment and of the behavioral disorders. The fact that the presenting symptom may be gelastic seizures is stressed. CT scan is the best means to assess the diagnosis and to follow the evolution of these tumors. Except for the management of the precocious puberty, the treatment is disappointing and neurosurgical indications are quite exceptional.

Adolescent↗
PubMedDate not supplied

Application of reconstruction computed tomography in a case of tuber cinereum hamartoma.

A case of a 2-year-old girl with tuber cinereum hamartoma is evaluated by computed tomography (CT). Special reference is made to the application of CT image reconstruction, which has not yet been reported in the literature. The location, extent, shape and size of the mass lesion and also the anatomical relationship to adjacent structures are clearly demonstrated in sagittal and coronal CT images reconstructed from serial thin-section CT images in conjunction with target imaging and a review program.

Child, Preschool↗
PubMed1983

Surface imaging from computerized tomographic scans.

A series of computer programs were created to display digital data obtained by the computerized tomographic (CT) scanner in a format readily comprehensible to the nonradiologist physician. The software requires no hardware beyond the CT scanner itself, is efficient in computational time and storage, and requires no operator intervention. The program manipulates standard CT axial data into images that simulate photographs of skin, muscle, or bone surfaces from a variety of perspectives. While the images themselves are planar, the surfaces they display are three dimensional. Portions of the images can be made transparent to permit viewing of previously concealed surfaces. Soft tissue and osseous surfaces may be superimposed to assist the localization of deep pathologic processes. Although the program was created to facilitate craniofacial reconstructive surgery, it has now been applied in over 150 patients for the evaluation of intracranial tumors, head and neck malignancies, cervical spine injuries, pelvic fractures, and deformities of the hands and feet. This ability to provide the surgeon with images that allow direct visualization of anatomic details has reduced operative time and improved surgical results in our management of congenital and acquired deformities.

Adolescent↗
PubMedDate not supplied

Clinical manifestations of hypothalamic tumors.

The regulatory function of the central nervous system encompasses diverse endocrine, metabolic, and behavioral processes. Many of these originate, are integrated, or are coordinated through hypothalamic pathways or nuclei. Thus, tumors affecting areas projecting to the hypothalamus, tumors of the hypothalamus, and tumors invading or compressing the hypothalamus can produce abnormalities of hypothalamic function.

Autonomic Nervous System↗