pituitrin and Pituitary-ACTH-Hypersecretion

Cerebral salt-wasting (CSW), or renal salt-wasting (RSW), has evolved from a misrepresentation of the syndrome of inappropriate secretion of antidiuretic hormone (SIADH) to acceptance as a distinct entity. Challenges still confront us as we attempt to differentiate RSW from SIADH, ascertain the prevalence of RSW, and address reports of RSW occurring without cerebral disease. RSW is redefined as 'extracellular volume depletion due to a renal sodium transport abnormality with or without high urinary sodium concentration, presence of hyponatremia or cerebral disease with normal adrenal and thyroid function.' Our inability to differentiate RSW from SIADH lies in the clinical and laboratory similarities between the two syndromes and the difficulty of accurate assessment of extracellular volume. Radioisotopic determinations of extracellular volume in neurosurgical patients reveal renal that RSW is more common than SIADH. We review the persistence of hypouricemia and increased fractional excretion of urate in RSW as compared to correction of both in SIADH, the appropriateness of ADH secretion in RSW, and the importance of differentiating renal RSW from SIADH because of disparate treatment goals: fluid repletion in RSW and fluid restriction in SIADH. Patients with RSW are being incorrectly treated by fluid restriction, with clinical consequences. We conclude that RSW is common and occurs without cerebral disease, and propose changing CSW to RSW.

Topics: Animals; Atrial Natriuretic Factor; Biomarkers; Brain; Diagnosis, Differential; Extracellular Fluid; Fluid Therapy; Humans; Hyponatremia; Kidney; Natriuretic Peptide, Brain; Pituitary ACTH Hypersecretion; Sodium; Terminology as Topic; Uric Acid; Vasopressins; Water-Electrolyte Balance

Tumors of hypothalamic neurons that produce vasopressin are rare. We retrieved all cases of vasopressin-positive tumors in the sellar region from the database of the Department of Pathology. Five cases fulfilled the selection criteria, representing the first series of such tumors. Clinical, radiologic, and pathologic features were reviewed. Four tumors classified as neurocytomas were identified in 3 females and 1 male patient; the ages at onset of symptoms ranged from 17 to 40 years. All were large sellar masses with suprasellar extension and/or invasion of the parasellar sinuses. Three patients had the syndrome of inappropriate antidiuresis; in one of these, a 6-year history was initially considered to be idiopathic. One patient died of progressive disease; 3 had incomplete resections and are being followed. In contrast to these patients with neurocytoma, a 65-year-old woman had Cushing disease and a 0.8 cm mass that was completely resected at transsphenoidal surgery; this tumor was a gangliocytoma producing vasopressin associated with corticotroph hyperplasia. We postulate that the small amount of vasopressin secreted by this mature gangliocytic tumor was locally bound to corticotrophs, resulting in hyperplasia and Cushing disease, without sufficient overproduction to cause systemic effects of vasopressin excess. Hypothalamic neurocytoma is a tumor that can mimic pituitary neuroendocrine tumors and olfactory neuroblastoma but is distinguished by positivity for neurofilaments, NeuN, and TTF-1 and negative staining for adenohypophysial biomarkers. Our cases illustrate that neurocytoma and gangliocytoma are 2 variants of tumors of hypothalamic neurons that can produce vasopressin. The morphologic and proliferative features of these 2 tumor types represent 2 ends of a spectrum; their function also can result in divergent clinical manifestations, one characterized by reduced urine output and the other by the more insidious features of glucocorticoid excess.

Topics: Adolescent; Adult; Aged; Diuresis; Female; Ganglioneuroma; Humans; Hypothalamic Neoplasms; Male; Neurocytoma; Pituitary ACTH Hypersecretion; Vasopressins

We performed an analysis of early factors influencing the outcome of Cushing's disease treated by transsphenoidal pituitary surgery.. Prospective study of 29 patients who underwent transsphenoidal pituitary surgery for Cushing's disease. The prognostic value of preoperative and operative variables, histological findings and serum cortisol (measured at 8:00 a.m. the day after surgery) were analyzed.. Of the 29 patients included in this study, 26 achieved postoperative remission while in 3 patients treatment failed. Tumor was identified at histology in 92.3% patients in the remission group and in 33.3% in the failure group, this difference being significant (p = 0.03). Median postoperative cortisol levels were 95.8 nmol/l in the remission group and 676 nmol/l in the failure group, this difference being significant (p = 0.024). Serum cortisol of 600 nmol/l correctly classified the remission and failure groups with a sensitivity of 100% and a specificity of 96%.. In our experience, no identification of an adenoma at histology and an early postoperative cortisol level higher than 600 nmol/l after transsphenoidal pituitary surgery for Cushing's disease was associated with a high risk of failed treatment.

Topics: Adenoma; Adolescent; Adrenocorticotropic Hormone; Adult; Aged; Aged, 80 and over; Child; Female; Humans; Hydrocortisone; Male; Middle Aged; Pituitary ACTH Hypersecretion; Pituitary Neoplasms; Postoperative Period; Prognosis; Remission Induction; Risk Factors; Sensitivity and Specificity; Treatment Failure; Vasopressins