17-ketosteroids and Hypertrophy

The experience with adrenocortical neoplasms in childhood is reviewed. During three decades, ten children with adrenocortical neoplasms were seen at the authors' institution. The literature was reviewed, and 209 patients 16.5 years or younger were found. All ten patients at Vanderbilt University Hospital (VUH) presented with endocrine manifestations of the tumor. Three patients had Cushing's, two patients had virilization, and five patients had features of both. In the literature, virilization, alone or with Cushing's, was the most common mode of presentation. Feminizing tumors were uncommon and nonfunctional tumors rare. The majority of patients were female. Features associated with malignancy included 17-KS levels greater than 40 mg/24 hr, diameter greater than 6 cm, weight greater than 500 g, and histologic evidence of diffuse growth pattern, vascular invasion, and tumor cell necrosis. Although uncommon, adrenocortical neoplasms should be suspected in children with Cushing's, virilization, feminization, or a combination of these. There has been an increased incidence reported in patients with hemihypertrophy, Beckwith-Wiedemann syndrome, hemangiomas, and nevi. Following endocrinologic evaluation, imaging studies should be performed. CT scan appears to be the most useful diagnostic tool. A posterior operative approach is recommended for suspected adenomas. An anterior thoracoabdominal approach is favored for suspected malignancies with uncompromised en bloc resection. There is no evidence that adjuvant therapy provides any additional benefit.

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adenoma; Adolescent; Adrenal Cortex Neoplasms; Carcinoma; Child; Child, Preschool; Cushing Syndrome; Female; Hirsutism; Humans; Hypertrophy; Infant; Male; Prognosis

A 3-year-old girl affected by a virilizing tumor of the adrenal gland, without significant elevation in the levels of 17 ketosteroids (17-KS) urinary excretion, was studied clinically. Her symptoms started abruptyly at the age of 2, with progressive enlargement of the clitoris and the appearance of pubic hair. In various tests, the 17-KS levels barely exceeded the upper normal limits and at times remained within normal limits. The retropneumoperitoneum X-ray suggested an enlargement of the right adrenal gland and the presence of a neoplasm, which was actually discovered during surgery. Histopathological examination revealed a well-defined neoplasm, without capsule invasion and with accentuated cell polymorphism. Histoenzymology showed that the tissue lacked the enzymatic system involving 3 beta-hydroxysteroid dehydrogenase (3 beta-HSD). Indoxylesterase (I.EST-A) activity identified the tumor as originating from the internal layers of the adrenal cortex. The histochemical findings were correlated to the clinical picture and the levels of urinary 17-KS.

Topics: 17-Ketosteroids; 3-Hydroxysteroid Dehydrogenases; Adrenal Cortex Neoplasms; Child, Preschool; Clitoris; Female; Hair; Histocytochemistry; Humans; Hypertrophy; Isomerases; Virilism; Vulva

A review of adrenal cortical carcinoma in childhood is presented, including endocrinologic aspects. Electron microscopic features are presented, as well as the finding of hypertrophied spermatic tubules. The importance of serial steroidal determinations is stressed as a "tumor marker" that may help in early detection of recurrent disease and possibly increased long-term survival.

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenal Cortex Neoplasms; Child, Preschool; Female; Gonadal Steroid Hormones; Humans; Hypertrophy; Male; Seminiferous Tubules; Testis; Virilism

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenocorticotropic Hormone; Adult; Biopsy; Chorionic Gonadotropin; Clitoris; Dexamethasone; Face; Female; Humans; Hypertrophy; Laparoscopy; Mestranol; Norethynodrel; Ovary; Polycystic Ovary Syndrome; Pregnancy; Pregnancy Complications; Testosterone; Virilism

Topics: 17-Ketosteroids; Adult; Clitoris; Female; Fetal Diseases; Humans; Hypertrophy; Ovarian Neoplasms; Ovary; Pregnancy; Pregnancy Complications; Protein Binding; Radionuclide Imaging; Testosterone; Thecoma; Virilism

Topics: 17-Ketosteroids; Adrenal Glands; Adrenocorticotropic Hormone; Animals; Castration; Disorders of Sex Development; Follicle Stimulating Hormone; Hypertrophy; Hypophysectomy; Ketosteroids; Luteinizing Hormone; Male; Rats

Topics: 17-Ketosteroids; Adrenal Glands; Adult; Clitoris; Contraceptives, Oral; Dexamethasone; Diagnosis, Differential; Estrogens; Female; Hirsutism; Humans; Hypertrophy; Leydig Cell Tumor; Male; Obesity; Ovary; Testosterone; Virilism

Topics: 17-Ketosteroids; Adolescent; Dehydroepiandrosterone; Diabetes Insipidus; Estradiol; Feedback; Gynecomastia; Histiocytosis, Langerhans-Cell; Humans; Hypertrophy; Hypothyroidism; Leydig Cells; Male; Puberty; Testicular Diseases; Testosterone

Progressive idiopathic masculinization of the clitoris was observed between the ages of 13 and 30 months in a healthy girl, otherwise normally developed except for a patent ductus arteriosus.

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Child, Preschool; Clitoris; Disorders of Sex Development; Female; Humans; Hypertrophy; Pregnanetriol

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adolescent; Black People; Breast Diseases; Female; Humans; Hypertrophy; Isoniazid; Thioacetazone; Tuberculosis, Pulmonary

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adolescent; Adult; Atrophy; Female; Gonadotropins; Humans; Hypertrophy; Ovarian Cysts; Ovarian Diseases; Ovary; Ovulation; Staining and Labeling

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenal Hyperplasia, Congenital; Androgens; Diagnosis, Differential; Female; Humans; Hypertrophy; Leiomyoma; Middle Aged; Neoplasms, Multiple Primary; Ovary; Uterine Hemorrhage; Uterine Neoplasms; Uterus

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Androgens; Clitoris; Disorders of Sex Development; Female; Humans; Hypertrophy; Pregnanes

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adenoma; Adolescent; Adrenal Gland Neoplasms; Adrenal Hyperplasia, Congenital; Adult; Androgens; Androstanes; Clitoris; Dehydroepiandrosterone; Female; Humans; Hypertrophy; Testosterone; Virilism

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenal Glands; Adrenocorticotropic Hormone; Adult; Aminoglutethimide; Aniline Compounds; Anticonvulsants; Endocrine System Diseases; Female; Humans; Hypertrophy; Metyrapone; Ovary; Pituitary-Adrenal Function Tests; Pyridones; Thyroid Function Tests; Thyroid Gland; Virilism

Topics: 17-Ketosteroids; Adolescent; Age Factors; Child; Child, Preschool; Cryptorchidism; Humans; Hypertrophy; Hypogonadism; Male; Testicular Diseases; Testis

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Abnormalities, Multiple; Adenoma; Adrenal Gland Neoplasms; Carcinoma; Child, Preschool; Cushing Syndrome; Dexamethasone; Female; Growth; Humans; Hydrocortisone; Hypertrophy; Infant; Infant, Newborn; Insulin; Male; Metyrapone; Pituitary-Adrenal Function Tests; Radiography; Sepsis

Topics: 17-Ketosteroids; Adrenal Glands; Adrenalectomy; Animals; Body Weight; Bone and Bones; Chromatography; Dehydroepiandrosterone; Desoxycorticosterone; Estrogens; Female; Hydrocortisone; Hypertrophy; Hypophysectomy; Kidney; Liver; Menopause; Muscles; Organ Size; Pituitary Gland; Rats

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Abdomen; Adrenocortical Hyperfunction; Alopecia; Animals; Asthenia; Dexamethasone; Dog Diseases; Dogs; Feeding and Eating Disorders; Female; Glucocorticoids; Glucose Tolerance Test; Humans; Hyperplasia; Hypertrophy; Liver Diseases; Male; Obesity; Pituitary-Adrenal Function Tests; Radiography; Thirst; Thyrotropin

Topics: 17-Ketosteroids; Acetates; Animals; Carbon Isotopes; Cholesterol; Chromatography, Paper; Dehydroepiandrosterone; Estrone; Female; Fetus; Horses; Hydroxyprogesterones; Hypertrophy; In Vitro Techniques; Male; Pregnancy; Pregnancy, Animal; Testis; Testosterone

Topics: 17-Ketosteroids; Adolescent; Adrenal Hyperplasia, Congenital; Child; Child, Preschool; Clitoris; Disorders of Sex Development; Female; Humans; Hypertrophy; Infant; Laparotomy; Postoperative Complications; Vulva; Water-Electrolyte Balance

Topics: 17-Ketosteroids; Acromegaly; Blood; Bone Development; Carbohydrate Metabolism; Child; Genetics, Medical; Genital Diseases, Female; Gigantism; Hepatomegaly; Hirsutism; Humans; Hypertrichosis; Hypertrophy; Infant; Lipodystrophy; Lipodystrophy, Congenital Generalized; Liver Diseases; Metabolism; Muscular Diseases; Radiography

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; Adrenocorticotropic Hormone; Anabolic Agents; Androgens; Endometrium; Estradiol; Estriol; Estrone; Female; Gonadotropins; Gonadotropins, Pituitary; Humans; Hyperplasia; Hypertrophy; Menstruation; Methyltestosterone; Nandrolone; Ovulation; Pharmacology; Phenylpropionates; Pituitary-Adrenal Function Tests; Pregnanediol; Salpingitis; Steroids; Testosterone; Tuberculosis; Tuberculosis, Female Genital; Uterine Cervical Erosion

Topics: 17-Ketosteroids; Adrenal Gland Diseases; Female; Humans; Hyperplasia; Hypertrophy; Puberty; Puberty, Precocious; Sexual Maturation; Tooth; Urine; Virilism

Topics: 17-Ketosteroids; Adrenal Cortex; Adrenal Cortex Diseases; Adrenal Hyperplasia, Congenital; Familial Mediterranean Fever; Fever; Humans; Hyperplasia; Hypertrophy; Male; Medical Records

Topics: 17-Ketosteroids; Adrenal Cortex; Adrenal Cortex Diseases; Bronchi; Hyperplasia; Hypertrophy; Neoplasms; Steroids; Urine

Topics: 17-Ketosteroids; Adrenal Cortex; Adrenal Cortex Diseases; Adrenocortical Hyperfunction; Hyperplasia; Hypertrophy; Steroids; Urine

Topics: 17-Ketosteroids; Humans; Hypertrophy; Male; Prostatectomy; Prostatic Hyperplasia; Steroids; Urine; Urologic Surgical Procedures

Topics: 17-Ketosteroids; Body Fluids; Humans; Hypertrophy; Lipid Metabolism; Male; Orthopedic Procedures; Prostatic Hyperplasia; Steroids; Vas Deferens; Vasectomy