Hyperandrogenemia and virilization with simultaneous pituitary and adrenal adenomas.

We describe a postmenopausal woman who presented with virilizing hyperandrogenemia and was found to have an intrasellar tumor and a large left adrenal mass. Pathologic studies showed on undifferentiated hypophyseal adenoma with immunostaining for chromogranin only and a benign adrenocortical adenoma. In light of current understanding of the regulation of adrenal androgen secretion and adrenocortical mitogenesis, we postulate that this case may be explained by secretion of adrenal androgen-stimulating and mitogenic factors by the pituitary tumor.

Cushing's disease: dilemmas of diagnosis and management.

Determining the cause of Cushing's disease and correcting the abnormality presents a continuing challenge to the clinician despite remarkable advances in diagnostic and therapeutic techniques. We present seven cases to illustrate 1) the classic disorder cured by pituitary adenomectomy; 2) persistence of the disease after adenomectomy; 3) Cushing's disease manifesting in the puerperium and remitting with dopamine agonist therapy; 4) a patient whose disease relapsed at least five times during 20 years of treatment by adrenalectomy, pituitary radiation, mitotane, and pituitary adenomectomy; 5) the Nelson syndrome; 6) the ectopic adrenocorticotropic hormone (ACTH) syndrome in a patient with dexamethasone suppressible urinary cortisol who had a pituitary adenoma which stained positively for ACTH but who was not cured by total hypophysectomy; and 7) a patient whose ACTH-secreting tumor proved fatal despite repeated surgical, radiologic and pharmacologic measures.

Colon cancer and polyps in acromegaly: increased risk associated with family history of colon cancer.

A cohort of 52 subjects diagnosed with acromegaly in southeastern Michigan and northern Ohio between 1935 and 1985 were followed to determine the incidence of colon cancer and polyps. Medical records were reviewed, subjects or their next-of-kin were interviewed, and screening examinations of the colon were offered to the living patients who were located. Data on demographics, personal histories of cancer and colon polyps, family history of colon cancer, and cure from acromegaly were obtained for both living and deceased subjects.

Hypersecretion of growth hormone and prolactin in McCune-Albright syndrome.

Acromegaly and hyperprolactinemia have been reported in association with the McCune-Albright syndrome, but the pathophysiology of the GH and PRL hypersecretion that occurs in patients with this disorder has not been defined. We studied GH and PRL secretory dynamics in three patients with McCune-Albright syndrome and hypersecretion of these hormones. Each patient had excessive linear growth, glucose-non-suppressible plasma GH concentration, and GH responsiveness to TRH and GHRH.

Marked elevation of serum dehydroepiandrosterone sulphate in Cushing's disease with macronodular adrenocortical hyperplasia.

A 30-year-old man presented with longstanding hypercortisolism and biochemical studies typical of pituitary-dependent Cushing's disease. After unsuccessful transsphenoidal surgery, plasma ACTH transiently became undetectable and adrenal computed tomography (CT) was consistent with macronodular hyperplasia. Serum dehydroepiandrosterone sulphate (DHEA-S) exceeded 10,000 ng/ml (normal 2,000-3,350 ng/ml).

Malignant somatostatinoma presenting with diabetic ketoacidosis.

High circulating levels of somatostatin (SRIF) were detected in a patient with a metastatic tumour after development of diabetic ketoacidosis (DKA). Fasting insulin and C-peptide levels were markedly suppressed, but plasma glucagon was not suppressed below normal. Progressive cachexia ensued; at autopsy a poorly differentiated non-small cell neuroendocrine carcinoma metastatic to liver was found.

Effect of bromocriptine treatment on the aldosterone response to angiotensin II and adrenocorticotropin in idiopathic hyperaldosteronism.

Bromocriptine can prevent an increase in plasma aldosterone during the infusion of angiotensin II in normal subjects and during upright posture in some patients with idiopathic hyperaldosteronism. To determine if bromocriptine prevents the increase in plasma aldosterone concentration during angiotensin II infusion in idiopathic hyperaldosteronism, we infused angiotensin II in five patients with idiopathic hyperaldosteronism, before and after treatment with bromocriptine (2.5 mg, three times daily for 5 days), and measured the resulting plasma aldosterone and angiotensin II concentrations.

Primary polydipsia. Syndrome of inappropriate thirst.

A patient with lifelong severe polyuria and polydipsia had normal serum antidiuretic hormone (ADH) levels and responded to water deprivation with a prompt increase in urine osmolality and maintenance of normal plasma osmolality (less than 290 mOsm/kg), despite extreme thirst. When treated with desmopressin acetate and allowed free access to water, she was able to reduce plasma osmolality below 270 mOsm/kg, and her compelling thirst disappeared. The disorder is interpreted to be the result of excessive fluid intake in response to a thirst stimulus that was not inhibited by normal plasma osmolality.

Failure of angiotensin II to stimulate increases in concentrations of adrenal androgens, 17-hydroxyprogesterone, or adrenocorticotropin in congenital 21-hydroxylase deficiency.

To determine if angiotensin II stimulates an increase in the plasma concentration of androstenedione, dehydroepiandrosterone, 17-hydroxyprogesterone, or ACTH in a patient with congenital 21-hydroxylase deficiency, we measured these plasma concentrations before and after the plasma angiotensin II concentration was increased by upright posture and angiotensin II infusion in a surgically castrate XX adult patient with this disorder. The patient was studied before treatment, after treatment with 1 mg dexamethasone daily for 3 weeks, and after treatment with both dexamethasone and 0.2 mg fludrocortisone daily for 3 weeks.

Pituitary carcinoma mimics the ectopic adrenocorticotropin syndrome.

A middle-aged man presented with weight loss, hypokalemic alkalosis, diabetes, hypertension, and generalized melanosis. Marked elevation of urinary free cortisol (655 micrograms/24 h) and plasma ACTH (2445 PG/ML) SUGGESTED THE DIAGNOSIS OF ECTOPIC ACTH syndrome. The plasma concentrations of cortisol and urinary 17-hydroxycorticosteroids increased paradoxically during the administration of dexamethasone without a corresponding change in the plasma ACTH level.

Acromegaly associated with a bronchial carcinoid tumor: evidence for ectopic production of growth hormone-releasing activity.

A patient with acromegaly, pituitary enlargement, and elevated plasma GH levels also had a bronchial carcinoid tumor. Signs and symptoms of active acromegaly along with elevated GH levels persisted for 11 yr after hypophysectomy and pituitary stalk section. Resection of the bronchial carcinoid reduced plasma GH to barely detectable levels.

Evaluation of thyroid function during growth hormone therapy.

Decrease in the blood levels of PBI, in the thyroidal uptake of iodine, and diminished effect of TRH on TSH release have been reported to occur during growth hormone administration. We assessed thyroid function indices in two groups of growth hormone deficient children before and during long-term HGH therapy. Eight patients were given TSH prior and at 2, 4, and 6 mo of growth hormone treatment.