pituitrin and Dysgerminoma

A case of a patient with the syndrome of chronic hypernatremia and hypodispia due to hypothalamic tumor was studied to evaluate the change of ADH response to plasma osmolality during the clinical course. A 23-year-old man was admitted for investigation of anorexia, hypodipsia and gait disturbance. Examination showed memory disturbance and generalized muscle weakness. Investigation showed marked hypernatremia (177 mEq/l) and hypopituitarism. Water loading test showed that ADH was not stimulated by hyperosmolality but continued to be secreted at a more or less constant level approximating normal basal state. CT scan revealed hypothalamic tumor. The tumor was suspected to be germinoma due to its radiosensitivity and high serum hCG level. After irradiation, the tumor lesion disappeared. ADH secretion came to be responsive to changes in osmolality but the response of the system was markedly reduced compared with the normal response, and hypodipsia and hypernatremia still remained. We conclude that the adipsia and complete destruction of the osmoreceptor in the patient caused marked hypernatremia and the destruction of ADH osmostat improved partially after irradiation. We believe it very useful for analyzing the disturbance of osmoregulatory system to evaluate the relationship of plasma ADH to plasma osmolality.

Topics: Adult; Dysgerminoma; Humans; Hypernatremia; Hypothalamic Neoplasms; Male; Osmolar Concentration; Thirst; Vasopressins

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

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: Adolescent; Adult; Blood Urea Nitrogen; Brain Neoplasms; Chlorides; Craniopharyngioma; Diabetes Insipidus; Drinking Behavior; Dysgerminoma; Female; Humans; Hydrocortisone; Hypernatremia; Hypothalamus; Male; Middle Aged; Osmolar Concentration; Potassium; Sella Turcica; Sodium; Thirst; Thyroid Function Tests; Triiodothyronine; Vasopressins; Water-Electrolyte Balance