pituitrin and Pinealoma

We describe the results of clinical and endocrinological investigations performed on 10 children and adolescents (5 males and 5 females) with a primary central nervous system germinoma. Eight of 10 patients were between 10-20 yr of age at the time of initial presentation. Polyuria (7 of 10) and a decrease in or cessation of linear growth (5 of 10) were the most common presenting symptoms, while only 2 of 10 patients complained of visual problems. Two patients presented with the syndrome of polyuria, adipsia, hypernatremia, profound muscle weakness, and hyperlipidemia. Initial physical exam revealed abnormal eye findings in 60%, short stature (greater than or equal to 2.5 SD) in 50%, and abnormal pubertal development in 30% of the patients. The neoplasm was located in the suprasellar-hypothalamic region in 8, caudate nucleus in 1, and pineal region in 1. Biopsy performed in 7 patients revealed the classic two-cell germinoma in all cases. Assessment of endocrine function before radiotherapy documented pituitary deficits in all patients studied. Antidiuretic hormone was deficient in 8 of 10 patients and was associated with hypoadipsia in 4. GH was deficient in al patients tested (7 of 7). TSH (5 of 8), ACTH (3 of 7), and gonadotropin (1 of 1) deficiencies were also common before treatment. Plasma PRL concentrations were elevated in 5 of 8 patients, all with suprasellar tumors. The hCG values were elevated only in the patient with sexual precocity (1 of 10). Endocrine evaluation during the postirradiation period revealed additional instances of GH (1), ACTH (1), and gonadotropin (5) deficiencies. All 10 patients are alive without evidence of active disease 6 months to 10 yr after radiation therapy (4500-5100 R). Evidence of hypothalamic-pituitary dysfunction is an early and almost universal feature of central nervous system germ cell tumors. The importance of careful evaluation and follow-up of children with acquired anterior or combined anterior and posterior pituitary dysfunction for a suprasellar tumor is stressed.

Topics: Adolescent; Adrenocorticotropic Hormone; Brain Neoplasms; Caudate Nucleus; Child; Child, Preschool; Dysgerminoma; Female; Gonadotropins, Pituitary; Growth Hormone; Humans; Hypothalamic Neoplasms; Male; Pinealoma; Prolactin; Thyrotropin; Tomography, X-Ray Computed; Vasopressins

Clinical aspects with disturbances in fluid and electrolytes metabolism in brain diseases were discussed reviewing 41 cases experienced in our department. These 41 cases were found in 377 patients with diseases of the central nervous system in our hospital during recent 14 months. Hyponatremia was found in 19 cases and aneurysms of A-C, A1 and A2 had the majority of the cases. The cerebral angiography suggested an unstable blood supply to the anterior portion of the hypothalamus, for instance, showing remarkable shift, spasm or obstruction A-C, A1 or A2. The duration of hyponatremia was transient and mostly less than 2 weeks after the last attack of subarachnoid hemorrhage. On the contrary, hypernatremia was seen in 9 cases and 6 of them were found in cases of tumors in the pineal region and A-C, A1 and A2 were intact angiographically. The hypernatremia was continuous and did not response to V-P shunt or any kinds of infusion therapy. The hypernatremia due to cerebral disease is thought to be a result of destruction of the supraoptic and paraventricular nuclei or adjacent area in the anterior potion of the hypothalamus in most of presumed these cases. It might be that the decreased blood supply to the anterior position of the hypothalamus offers an information not of hypoosmolarity but of hypovolemic state, and this information increases the secretion of ADH. This mechanism of hyponatremia could play an important role in S.I.A.D.H.

Topics: Adolescent; Adult; Aged; Brain Diseases; Brain Neoplasms; Child; Female; Humans; Hypernatremia; Hyponatremia; Infant; Intracranial Aneurysm; Male; Middle Aged; Pinealoma; Postoperative Complications; Vasopressins

Topics: Acromegaly; Adenoma, Acidophil; Adenoma, Chromophobe; Adult; Brain Neoplasms; Craniopharyngioma; Diabetes Insipidus; Female; Humans; Luteinizing Hormone; Male; Meningioma; Metyrapone; Pinealoma; Pituitary Hormone-Releasing Hormones; Pituitary Neoplasms; Radioimmunoassay; Thyrotropin; Thyrotropin-Releasing Hormone; Vasopressins

A patient with ectopic pinealoma first presented with apparent anorexia nervosa and hypernatraemic coma. A history of diabetes insipidus two months previously was not known on admission to hospital. The diabetes insipidus was unmasked by the administration of steroids. Neuroendocrinal and neuropathological aspects of the case are discussed with reference to the march of symptoms due to the growth of the tumour. Histochemical evidence is presented supporting the similarity between ectopic pinealoma and seminoma which suggests that they may more properly be referred to as atypical teratomas.

Topics: Acid Phosphatase; Adult; Alkaline Phosphatase; Anorexia Nervosa; Brain Neoplasms; Coma; Diabetes Insipidus; Dihydrolipoamide Dehydrogenase; Dysgerminoma; Electron Transport Complex IV; Esterases; Female; Humans; Hydrocortisone; Hypernatremia; Hypothalamus; Male; Osmolar Concentration; Oxidoreductases; Pinealoma; Sodium; Testicular Neoplasms; Thyroxine; Tuberculosis; Vasopressins

Topics: Adrenocorticotropic Hormone; Adult; Animals; Cattle; Diabetes Insipidus; Histamine Release; Humans; Hypersensitivity, Immediate; Hypopituitarism; Immunoglobulin E; Immunoglobulin G; Leukocytes; Male; Oxytocin; Pinealoma; Pituitary Gland; Rabbits; Sheep; Skin Tests; Swine; Thyrotropin; Vasopressins

Topics: Adult; Dehydration; Humans; Hypernatremia; Hypopituitarism; Hypothalamus; Male; Osmolar Concentration; Pinealoma; Thirst; Vasopressins

Topics: Adolescent; Adrenocorticotropic Hormone; Adult; Craniocerebral Trauma; Diabetes Insipidus; Female; Glioma; Hodgkin Disease; Humans; Hypothalamo-Hypophyseal System; Male; Metyrapone; Middle Aged; Neurotransmitter Agents; Pinealoma; Pituitary Gland; Pituitary-Adrenal Function Tests; Skull Neoplasms; Vasopressins; Water-Electrolyte Balance

Topics: Child; Dehydration; Female; Hormones; Humans; Hypernatremia; Hypothalamus; Pinealoma; Polyuria; Thirst; Vasopressins

Topics: Child; Dehydration; Drug Therapy; Humans; Hypernatremia; Hypothalamus; Hypothyroidism; Metabolism; Neoplasms; Neurosurgery; Pinealoma; Pituitary-Adrenal Function Tests; Prednisone; Thirst; Triiodothyronine; Vasopressins; Water-Electrolyte Balance