17-ketosteroids and Adrenogenital-Syndrome

Topics: 17-Ketosteroids; Adrenal Insufficiency; Adrenocortical Hyperfunction; Adrenogenital Syndrome; Anabolic Agents; Hirsutism; Hyperaldosteronism; Hypertrichosis; Hypogonadism; Metabolism; Steroids; Urine

A putative hilus interstitial cell has been proposed as the cell of origin for testicular tumors of adrenogenital syndrome, but its normal histology is not documented. We present hitherto undescribed nodular steroid cell nests associated with the rete testis that are distinctive in their morphology and immunohistochemical profile from Leydig cells and do not have the morphology of typical extra-adrenal cortical rests. These nodules measured 1, 1, 1.8, 2, and 2.5 mm in size with a distinct sinusoidal vasculature. Individual cells were rounded to polygonal with evenly distributed moderate-to-abundant eosinophilic cytoplasm. The nuclei were homogenous and round, with fine chromatin and ocasionally with prominent nucleoli. The differential diagnosis included adrenocortical rests, testicular adnexal Leydig cells, carcinoid tumorlets, paraganglionic rests, and adenomatoid mesothelial proliferation. Immunohistochemistry showed positivity for melan A (5/5), inhibin (3/5), and calretinin (2/4), although the immunoreactivity was distinctively different from the concurrent intratesticular Leydig cells and testicular adnexal Leydig cells in all cases. The unique morphology, immunophenotype, and distinctive location of these cells in the testicular mediastinum raises the possibility that these cells represent testicular hilus steroid cells, the putative histogenetic cell implicated for testicular tumors of adrenogenital syndrome. We propose to name these proliferations rete testis-associated nodular steroid cell nests.

Topics: 17-Ketosteroids; Adrenogenital Syndrome; Adult; Biomarkers; Carcinoma, Embryonal; Epididymitis; Humans; Leydig Cells; Male; Middle Aged; Neoplasms, Germ Cell and Embryonal; Pluripotent Stem Cells; Rete Testis; Testicular Neoplasms; Young Adult

Topics: 17-Ketosteroids; Adrenogenital Syndrome; Amenorrhea; Chiari-Frommel Syndrome; Clomiphene; Drug Therapy; Estrogens; Female; Gonadotropins; Humans; Infertility; Infertility, Female; Ovulation; Pharmacology; Polycystic Ovary Syndrome; Pregnanediol; Stilbenes; Toxicology; Urine

Topics: 17-Ketosteroids; Adolescent; Adrenal Gland Diseases; Adrenal Hyperplasia, Congenital; Adrenogenital Syndrome; Child; Chromatography; Humans; Hyperplasia; Infant; Metabolism; Urine

Topics: 17-Ketosteroids; Adrenal Cortex Hormones; Adrenal Gland Diseases; Adrenalectomy; Adrenocortical Hyperfunction; Adrenogenital Syndrome; Aldosterone; Androgens; Cushing Syndrome; Humans; Hyperplasia; Hypertrophy; Pathology; Pituitary-Adrenal Function Tests; Sympatholytics

Topics: 17-Ketosteroids; Adrenal Cortex; Adrenal Gland Neoplasms; Adrenal Hyperplasia, Congenital; Adrenogenital Syndrome; Anxiety; Cushing Syndrome; Diagnosis, Differential; Hypertension; Liver Neoplasms; Neoplasm Metastasis; Nephrectomy; Pathology; Urine; Urography

Topics: 17-Ketosteroids; Addison Disease; Adrenal Glands; Adrenocorticotropic Hormone; Adrenogenital Syndrome; Asthma; Carcinoma, Bronchogenic; Coccidioidomycosis; Cushing Syndrome; Diabetes Mellitus; Ethinyl Estradiol; Female; Humans; Hydrocortisone; Hyperaldosteronism; Hypopituitarism; Hypotension; Myocardial Infarction; Neurotic Disorders; Pituitary Neoplasms; Polycystic Ovary Syndrome

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

Topics: 17-Ketosteroids; Adrenal Cortex Hormones; Adrenogenital Syndrome; Androgens; Child; Cystic Fibrosis; Diet; Diet Therapy; Galactosemias; Infant; Infant, Newborn; Metabolic Diseases; Phenylketonurias; Prognosis; Urine

Topics: 17-Ketosteroids; Adolescent; Adrenogenital Syndrome; Blood Chemical Analysis; Child; Cortisone; Desoxycorticosterone; Humans; Infant; Infant, Newborn; Infant, Newborn, Diseases; Prednisone; Urine

Topics: 17-Ketosteroids; Adenocarcinoma; Adrenogenital Syndrome; Androgens; Cortisone; Disorders of Sex Development; Estrogens; Female; Humans; Hypertrichosis; Hypogonadism; Hysterectomy; Iatrogenic Disease; North Carolina; Ovarian Neoplasms; Pituitary-Adrenal Function Tests; Progesterone; Urine

Korsakoff's syndrome of obscure etiology was observed in a 34-year-old single woman with an 11-year history of hirsutism and mood swings, and previous hospitalizations for mania three years ago and depression 11 years ago.Recently the virilism had intensified with increased muscularity and coarsening of facial features. The 24-hour urinary 17-ketosteroids ranged between 14.4 mg. and 21.5 mg. and were suppressed by dexamethasone. The 17-hydroxycorticosteroid excretion was normal. These and other findings suggested a diagnosis of adrenal virilism due to adrenocortical hyperplasia. In the absence of other discernible causes it appeared that the adrenal pathology was responsible for the Korsakoff's syndrome. Both conditions responded well to glucocorticoid therapy although low doses were necessary to avoid mania.It is speculated that the encephalopathy was due to an associated adrenal insufficiency. Although hypoadrenalism is accepted as a complication of only the infant form of adrenal virilism, it is noteworthy that this patient had pathological pigmentation of her skin.

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenal Hyperplasia, Congenital; Adrenocortical Hyperfunction; Adrenogenital Syndrome; Amobarbital; Antipsychotic Agents; Barbiturates; Female; Glutethimide; Hirsutism; Humans; Hypertrichosis; Korsakoff Syndrome; Phenothiazines; Prednisone; Thioridazine; Trifluoperazine; Virilism; Wernicke Encephalopathy

Topics: 17-Ketosteroids; Addison Disease; Adrenal Insufficiency; Adrenogenital Syndrome; Androgens; Cholesterol; Cushing Syndrome; Hypoadrenocorticism, Familial; Physiology; Pituitary-Adrenal Function Tests; Progestins; Urine

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenal Cortex Hormones; Adrenal Gland Diseases; Adrenocortical Hyperfunction; Adrenogenital Syndrome; Dexamethasone; Fatigue; Fever; Humans; Hyperplasia; Male; Pituitary-Adrenal Function Tests; Prednisone; Urine

Topics: 17-Ketosteroids; Adrenal Cortex Hormones; Adrenal Hyperplasia, Congenital; Adrenocortical Hyperfunction; Adrenogenital Syndrome; Age Determination by Skeleton; Blood Chemical Analysis; Classification; Cortisone; Desoxycorticosterone; Drug Therapy; Humans; Hydrocortisone; Hyperplasia; Infant; Infant, Newborn; Infant, Newborn, Diseases; Urine

Topics: 17-Ketosteroids; Adrenal Gland Neoplasms; Adrenogenital Syndrome; Estradiol; Estriol; Estrogens; Estrone; Follicle Stimulating Hormone; Gynecomastia; Humans; Hypogonadism; Male; Neoplasm Metastasis; Neoplasms; Pregnanediol; Surgical Procedures, Operative; Testis; Urine

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adolescent; Adrenal Cortex; Adrenal Gland Diseases; Adrenal Hyperplasia, Congenital; Adrenogenital Syndrome; Androsterone; Blood Chemical Analysis; Child; Dehydroepiandrosterone; Humans; Hyperplasia; Pituitary-Adrenal Function Tests; Testosterone; Urine

Topics: 17-Ketosteroids; Adenoma; Adrenal Gland Diseases; Adrenal Gland Neoplasms; Adrenal Hyperplasia, Congenital; Adrenocorticotropic Hormone; Adrenogenital Syndrome; Cholesterol; Chromatography; Dehydroepiandrosterone; Dexamethasone; Drug Therapy; Gonadotropins; Humans; Hydrocortisone; Lipids; Male; Pathology; Puberty; Puberty, Precocious; Sexual Maturation; Surgical Procedures, Operative; Urine

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Abdomen; Abdomen, Acute; Adrenal Gland Neoplasms; Adrenocortical Hyperfunction; Adrenogenital Syndrome; Betamethasone; Diagnosis; Humans; Neoplasms; Urine

Topics: 17-Ketosteroids; Adrenal Hyperplasia, Congenital; Adrenogenital Syndrome; Blood; Body Fluids; Child; Cortisone; Drug Therapy; Humans; Hydrocortisone; Infant; Infant, Newborn; Pregnanetriol; Progestins; Urine

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenal Cortex; Adrenal Gland Diseases; Adrenal Hyperplasia, Congenital; Adrenocorticotropic Hormone; Adrenogenital Syndrome; Blood; Cushing Syndrome; Estrogens; Humans; Hyperplasia; Pharmacology; Pregnanetriol; Triamcinolone; Urine

Topics: 17-Ketosteroids; Adrenal Cortex Hormones; Adrenal Hyperplasia, Congenital; Adrenogenital Syndrome; Body Fluids; Child; Cortisone; Drug Therapy; Urine

Topics: 17-Ketosteroids; 46, XX Disorders of Sex Development; Adenoma; Adrenal Cortex; Adrenal Cortex Hormones; Adrenal Cortex Neoplasms; Adrenocortical Adenoma; Adrenogenital Syndrome

Topics: 17-Ketosteroids; Adrenal Hyperplasia, Congenital; Adrenogenital Syndrome; Corticosterone; Dexamethasone; Estradiol; Heart Defects, Congenital; Humans; Hydrocortisone; Pregnanediol; Triamcinolone

Topics: 17-Ketosteroids; Adrenal Gland Diseases; Adrenal Hyperplasia, Congenital; Adrenogenital Syndrome; Anatomy; Etiocholanolone; Fever; Hyperplasia

Topics: 17-Ketosteroids; Adrenal Cortex; Adrenal Gland Diseases; Adrenal Insufficiency; Adrenocortical Hyperfunction; Adrenogenital Syndrome; Humans; Hyperplasia; Infant; Infant, Newborn; Infant, Newborn, Diseases; Metabolic Diseases; Sodium Chloride

Topics: 17-Ketosteroids; Adrenal Hyperplasia, Congenital; Adrenogenital Syndrome

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenal Cortex Hormones; Adrenal Gland Diseases; Adrenal Gland Neoplasms; Adrenocorticotropic Hormone; Adrenogenital Syndrome; Hirsutism; Hypertrichosis; Mineralocorticoid Receptor Antagonists; Pituitary-Adrenal Function Tests; Pregnanediol; Urine

Topics: 17-Ketosteroids; Adenoma; Adenoma, Chromophobe; Adolescent; Adrenal Cortex Hormones; Adrenal Hyperplasia, Congenital; Adrenalectomy; Adrenocorticotropic Hormone; Adrenogenital Syndrome; Blood Chemical Analysis; Cushing Syndrome; Dexamethasone; Humans; Male; Progesterone; Testicular Neoplasms; Urine

Topics: 17-Ketosteroids; Adrenal Hyperplasia, Congenital; Adrenocortical Hyperfunction; Adrenogenital Syndrome; Androgens; Androsterone; Cysts; Female; Humans; Ovary; Polycystic Ovary Syndrome; Testosterone; Thyroidectomy

Topics: 17-Ketosteroids; Adolescent; Adrenogenital Syndrome; Chromatography; Dexamethasone; Humans; Hypertrichosis; Retropneumoperitoneum; Urine

Topics: 17-Ketosteroids; Adenoma; Adrenal Cortex Hormones; Adrenal Hyperplasia, Congenital; Adrenocortical Hyperfunction; Adrenocorticotropic Hormone; Adrenogenital Syndrome; Aldosterone; Cholesterol; Cushing Syndrome; Follicle Stimulating Hormone; Humans; Metabolism; Pituitary-Adrenal Function Tests; Pregnanediol

Topics: 17-Ketosteroids; Adolescent; Adrenal Glands; Adrenal Hyperplasia, Congenital; Adrenogenital Syndrome; Child; Clitoris; Disorders of Sex Development; Female; Humans; Hyperplasia; Infant, Newborn; Pituitary-Adrenal Function Tests; Prednisone; Surgical Procedures, Operative

Topics: 17-Ketosteroids; Adrenocorticotropic Hormone; Adrenogenital Syndrome; Alopecia; Asthenia; Hyperthyroidism; Metyrapone; Mineralocorticoid Receptor Antagonists; Pituitary Gland; Pituitary-Adrenal Function Tests; Rheumatic Heart Disease

Topics: 17-Ketosteroids; Adolescent; Adrenal Hyperplasia, Congenital; Adrenogenital Syndrome; Bone and Bones; Child; Cortisone; Gonadotropins; Growth; Netherlands; Statistics as Topic; Urine

Topics: 17-Ketosteroids; Adrenal Gland Neoplasms; Adrenal Hyperplasia, Congenital; Adrenogenital Syndrome; Child; Cushing Syndrome; Gonads; Humans; Male; Pathology; Surgical Procedures, Operative; Testis; Urine

Topics: 17-Ketosteroids; Adrenal Hyperplasia, Congenital; Adrenogenital Syndrome; Biological Phenomena; Dexamethasone; Growth; Medical Records; Methylprednisolone; Physiological Phenomena; Prednisone

Topics: 17-Ketosteroids; Adrenal Cortex Hormones; Adrenal Hyperplasia, Congenital; Adrenogenital Syndrome; Anovulation; Female; Humans; Infertility; Infertility, Female

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenal Hyperplasia, Congenital; Adrenogenital Syndrome; Steroids

Topics: 17-Ketosteroids; Adrenal Hyperplasia, Congenital; Adrenogenital Syndrome; Chromatography; Humans; Pregnanediol

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenal Cortex Hormones; Adrenocorticotropic Hormone; Adrenogenital Syndrome; Adult; Female; Gonadotropins; Gonadotropins, Pituitary; Humans; Medical Records; Neoplasms

Topics: 17-Ketosteroids; Adrenal Hyperplasia, Congenital; Adrenogenital Syndrome; Female; Humans; Prednisone; Vagina; Vaginal Smears

Topics: 17-Ketosteroids; Adrenal Hyperplasia, Congenital; Adrenogenital Syndrome; Cytodiagnosis; Female; Humans; Vagina

Topics: 17-Ketosteroids; Adrenocorticotropic Hormone; Adrenogenital Syndrome; Biological Transport; Steroids

Topics: 17-Ketosteroids; Adrenal Hyperplasia, Congenital; Adrenogenital Syndrome; Chromatography; Disorders of Sex Development; Follow-Up Studies; Humans; Medical Records; Steroids; Urogenital Abnormalities; Urogenital System

Topics: 17-Ketosteroids; Adrenal Cortex; Adrenal Hyperplasia, Congenital; Adrenogenital Syndrome; Female; Hormones; Humans; Hyperplasia; Virilism

Topics: 17-Ketosteroids; Adrenal Cortex; Adrenal Cortex Diseases; Adrenal Hyperplasia, Congenital; Adrenogenital Syndrome; Cushing Syndrome; Hormones; Hydrocortisone

Topics: 17-Ketosteroids; 46, XX Disorders of Sex Development; Adrenal Hyperplasia, Congenital; Adrenal Insufficiency; Adrenogenital Syndrome; Genetic Diseases, X-Linked; Hormones; Hypoadrenocorticism, Familial; Prednisone

Topics: 17-Ketosteroids; Adrenal Cortex; Adrenal Cortex Diseases; Adrenal Hyperplasia, Congenital; Adrenogenital Syndrome; Steroids; Urine