17-ketosteroids and Pheochromocytoma

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Acid Phosphatase; Adrenal Gland Neoplasms; Alkaline Phosphatase; Amino Acids; Amylases; Bone Neoplasms; Breast Neoplasms; Carcinoid Tumor; Catecholamines; Chorionic Gonadotropin; Clinical Enzyme Tests; Clinical Laboratory Techniques; Female; Glucose-6-Phosphate Isomerase; Humans; Hydroxyindoleacetic Acid; L-Lactate Dehydrogenase; Liver Neoplasms; Male; Neoplasms; Neoplasms, Nerve Tissue; Neuroblastoma; Nucleotidases; Pancreatic Neoplasms; Pheochromocytoma; Pregnancy; Prostatic Neoplasms; Trophoblastic Neoplasms; Vanilmandelic Acid

Operative management of pheochromocytomas dictates resection of the involved adrenal and exploration-resection of the contralateral gland if enlarged. We describe an exception to this rule.. We report the largest series of patients with adrenocorticotropic hormone (ACTH)-secreting pheochromocytomas and review the world literature.. Four patients presented with findings of adrenocorticoid and catecholamine excess, as well as elevated levels of plasma ACTH, urinary metanephrines, and urinary free cortisol. Abdominal computed tomography scans revealed bilateral adrenal hyperplasia, and magnetic resonance imaging scans showed a unilateral adrenal mass with a bright T2 signal suggesting a pheochromocytoma. Two patients underwent adrenal venous sampling localizing ACTH secretion to the pheochromocytoma. All underwent unilateral adrenalectomy for a benign tumor without morbidity or death, leaving the contralateral hyperplastic adrenal in situ. After operation all patients experienced normalization of their levels of plasma ACTH, urinary metanephrines, and urinary free cortisol with resolution of symptoms. Combining our series with previously reported cases of ACTH-secreting pheochromocytomas, almost all are benign (20 of 21), in contrast to most ACTH-secreting tumors.. ACTH-secreting pheochromocytomas are the exception to the rule; unilateral adrenalectomy is curative and the contralateral hyperplastic adrenal can be preserved. This approach results in resolution of both syndromes of hormone excess and preserves long-term adrenal function.

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenal Gland Neoplasms; Adrenalectomy; Adrenocorticotropic Hormone; Adult; Catecholamines; Female; Humans; Hydrocortisone; Hypertension; Magnetic Resonance Imaging; Male; Middle Aged; Pheochromocytoma; Tomography, X-Ray Computed

At our medical center we have seen 151 patients who have undergone adrenalectomy from 1971 up until June 1992. There has been a recent increase in the number of cases since 99 patients have been seen in the last eight years from 1985. This increase is related to the increase in the number of incidental tumors. Of those 99, 26 have been incidental tumors. Twenty-four of those have been endocrine inactive tumors, and the remaining 2, both active endocrinologically, were pheochromocytoma. We propose that our tentative indications for surgical treatment of incidental tumors are 1) tumors of diameter 3.5 cm or greater, 2) tumors undeniably malignant by imaging diagnosis, 3) tumors that tend to increase in size with every six-month re-examination, even though the original size is less than 3.5 cm in diameter, and 4) tumors endocrinologically active. The main purpose of this study was to make a differential diagnosis of malignant tumors among incidental tumors. Diagnostic modalities included 1) imaging diagnosis such as echography, CT, MRI, and angiography, 2) analysis of DNA ploidy and BrdU uptake utilizing flow cytometry of excised adrenal tumor tissues, and 3) analysis of urinary steroid fraction. It is generally thought that there are currently no methods by which malignant tumors can be differentiated with certainty, even with modern diagnostic technology, and review of the literature, finds approval or disapproval with regard to the methods we selected to evaluate tumors. What has aroused interest in our results is urinary 17KS fraction.(ABSTRACT TRUNCATED AT 250 WORDS)

Topics: 17-Ketosteroids; Adrenal Gland Neoplasms; Adrenalectomy; Biomarkers, Tumor; Diagnosis, Differential; Female; Humans; Magnetic Resonance Imaging; Male; Pheochromocytoma; Tomography, X-Ray Computed

With the wider application of increasingly sensitive computed tomographic scans, more adrenal masses will be discovered incidentally. Because benign lesions of the adrenal are much commoner than malignant ones, an approach is needed to determine which incidentally discovered masses should be removed. The history and physical examination may guide the evaluation. Imaging studies and needle biopsies have limited value. If the history and physical findings do not suggest a diagnosis, an approach using the size of the mass, results of any cyst puncture, and a biochemical assessment may determine which patients should have surgery. This approach is based on the relative prevalence of benign and malignant clinically silent adrenal tumors.

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adenoma; Adrenal Cortex Neoplasms; Adrenal Gland Neoplasms; Biopsy, Needle; Carcinoma; Diagnosis, Differential; Female; Humans; Male; Pheochromocytoma; Sex Factors; Tomography, X-Ray Computed

Medullary carcinoma of the thyroid (MCT) is reported to synthesize ACTH. This ACTH is believed to be responsible for the development of Cushing's syndrome in some patients with MCT. To determine the frequency of occurrence of adrenal cortical overactivity in patients with MCT, we measured plasma cortisol concentration and the urinary excretion of 17-hydroxycorticosteroids, 17-ketosteroids and urinary free cortisol in 22 patients with MCT and 7 patients with MCT plus pheochromocytomas. The patients with MCT and MCT plus pheochromocytoma had similar adrenal cortical function to age and sex matched normal subjects. We conclude that adrenal cortical function is usually normal in patients with MCT.

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adolescent; Adrenal Cortex Function Tests; Adrenal Gland Neoplasms; Adrenocorticotropic Hormone; Adult; Carcinoma; Child; Cushing Syndrome; Female; Humans; Hydrocortisone; Male; Middle Aged; Pheochromocytoma; Thyroid Neoplasms

Topics: 17-Ketosteroids; Adrenal Cortex Diseases; Adrenal Cortex Hormones; Adrenal Gland Diseases; Adrenal Gland Neoplasms; Adrenal Medulla; Adult; Cushing Syndrome; Dexamethasone; Epinephrine; Female; Humans; Middle Aged; Norepinephrine; Pheochromocytoma

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenal Gland Neoplasms; Adult; Aged; Aldosterone; Child; Child, Preschool; Cushing Syndrome; Female; Humans; Hyperaldosteronism; Hypertension; Hypertension, Malignant; Hypertension, Renal; Male; Middle Aged; Pheochromocytoma; Potassium; Renin; Sodium; Sweat

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adolescent; Adrenal Gland Neoplasms; Adult; Epinephrine; Female; Humans; Male; Norepinephrine; Pheochromocytoma; Renin

Topics: 17-Ketosteroids; Adrenal Gland Neoplasms; Adrenal Glands; Adult; Angiography; Carcinoma; Child; Cushing Syndrome; Female; Humans; Hypertension; Male; Middle Aged; Neoplasm Recurrence, Local; Neoplasms, Multiple Primary; Pheochromocytoma; Renal Artery; Surgical Procedures, Operative; Thymoma; Urography; Vena Cava, Inferior

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenalectomy; Adrenocorticotropic Hormone; Adult; Carcinoma; Cushing Syndrome; Dexamethasone; Female; Hormones, Ectopic; Humans; Lymphatic Metastasis; Male; Metyrapone; Pheochromocytoma; Thyroid Neoplasms; Thyroidectomy

Topics: 17-Ketosteroids; Adrenal Hyperplasia, Congenital; Adult; Breast Neoplasms; Child; Chromatography, Gas; Cushing Syndrome; Etiocholanolone; Female; Humans; Hypopituitarism; Male; Methods; Pheochromocytoma; Polycystic Ovary Syndrome; Pregnanes; Statistics as Topic; Steroids

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Acromegaly; Addison Disease; Adolescent; Adrenal Cortex Hormones; Adrenal Gland Neoplasms; Adrenal Hyperplasia, Congenital; Adult; Anorexia Nervosa; Child; Child, Preschool; Chromatography; Cushing Syndrome; Disorders of Sex Development; Endocrine System Diseases; Female; Humans; Hydrocortisone; Hyperaldosteronism; Hyperthyroidism; Hypertrichosis; Hypopituitarism; Male; Middle Aged; Myxedema; Obesity; Pheochromocytoma; Pregnancy; Spectrophotometry

Topics: 17-Ketosteroids; Adenoma; Adrenal Gland Neoplasms; Adrenal Hyperplasia, Congenital; Adrenalectomy; Adrenocortical Hyperfunction; Adult; Carcinoma; Child; Child, Preschool; Cushing Syndrome; Female; Humans; Hyperaldosteronism; Hypertension; Intestinal Polyps; Male; Pheochromocytoma

Topics: 17-Ketosteroids; Acute Kidney Injury; Adolescent; Adult; Female; Humans; Hyperventilation; Male; Middle Aged; Nephrosis; Neurofibromatosis 1; Pheochromocytoma

Topics: 17-Ketosteroids; Adrenal Cortex Hormones; Adrenal Gland Diseases; Adrenal Gland Neoplasms; Adrenal Hyperplasia, Congenital; Adrenalectomy; Adult; Breast Neoplasms; Child, Preschool; Cortisone; Cushing Syndrome; Disorders of Sex Development; Female; Humans; Hyperaldosteronism; Infant; Male; Pheochromocytoma; Prednisolone; Virilism

Topics: 17-Ketosteroids; Adrenal Cortex Hormones; Adrenal Gland Neoplasms; Adrenalectomy; Adrenocortical Hyperfunction; Adrenogenital Syndrome; Cushing Syndrome; Humans; Hyperaldosteronism; Pheochromocytoma

Topics: 17-Ketosteroids; Adenoma, Islet Cell; Adrenocorticotropic Hormone; Blood; Cushing Syndrome; Dexamethasone; Endocrinology; Humans; Lung Neoplasms; Mediastinal Neoplasms; Pancreatic Neoplasms; Parotid Neoplasms; Peptides; Pheochromocytoma; Urine