17-ketosteroids and Testicular-Neoplasms

Topics: 17-Ketosteroids; Adenoma; Adrenal Cortex Neoplasms; Adrenocorticotropic Hormone; Adult; Androgens; Carcinoma; Child, Preschool; Cushing Syndrome; Estrogens; Female; Feminization; Glucocorticoids; Granulosa Cell Tumor; Humans; Leydig Cell Tumor; Male; Mesenchymoma; Middle Aged; Mitotane; Ovarian Neoplasms; Paraneoplastic Endocrine Syndromes; Pregnancy; Progesterone; Prognosis; Sertoli Cell Tumor; Steroids; Testicular Neoplasms; Thecoma; Virilism

A 28-year-old man with evidence of feminization was demonstrated after 4 years of investigation to have a estrogen-secreting interstitial cell tumor. Such feminizing neoplasms are uncommon, only 37 having been described. They are usually benign and are characterized by gynecomastia, a testicular mass and, with lesser frequency, by decreased libido and potency and poor semen quality. The urinary excretion and plasma levels of estrogen are increased and, by selective testicular catheterization, the site of increased estrogen production can be localized. Secondary histologic changes occur in the nontumorous portions of the testis as well as in the contralateral testis; they are most marked in the area immediately adjacent to the tumor. Postoperatively, the gynecomastia regresses, the excessive levels of estrogen return to normal, libido improves, and the sperm count increases to normal.

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adolescent; Adult; Aged; Biopsy; Child; Erectile Dysfunction; Estrogens; Follicle Stimulating Hormone; Gynecomastia; Humans; Leydig Cell Tumor; Libido; Luteinizing Hormone; Male; Middle Aged; Spermatogenesis; Testicular Neoplasms; Testosterone

Topics: 17-Ketosteroids; Age Factors; Body Height; Brain Diseases; Choriocarcinoma; Dysgerminoma; Ejaculation; Female; Granulosa Cell Tumor; Humans; Hydrocephalus; Male; Menarche; Ovarian Neoplasms; Ovulation; Pregnancy; Prognosis; Psychosexual Development; Puberty, Precocious; Reproduction; Sex Factors; Teratoma; Testicular Neoplasms; Thecoma

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

We present an unusual patient with a Leydig cell tumor to show that greatly elevated serum concentrations of 17-hydroxyprogesterone (17OHP) may not be diagnostic of congenital adrenal hyperplasia (CAH). A 3.5-yr-old boy had a small testicular mass and plasma 17OHP concentrations of 147-333 nmol/L (4,850-11,000 ng/dL), suggesting CAH with adrenal rests. However, normal plasma cortisol values and the unresponsiveness of the 17OHP concentration to dexamethasone suppression or ACTH stimulation suggested a diagnosis of Leydig cell tumor. A 4-fold elevation in plasma 21-deoxycortisol compared with a 200-fold elevation in 17OHP suggested that the elevated 17OHP derived from the normal pathway of testosterone synthesis in the testis. This was proven by normalization of all hormonal values after tumor resection. Compared to the abundance of mRNA for P450c17, the tumor contained unusually large amounts of mRNA for P450scc, the cholesterol side-chain cleavage enzyme, which is the rate-limiting step in steroid hormone synthesis. Increased P450scc activity, which increased the conversion of cholesterol to pregnenolone, apparently permitted the 17,20-lyase activity of P450c17 to become rate limiting, thus accounting for the increased secretion of 17OHP. Thus, Leydig cell tumors can produce quantities of 17OHP previously reported only in CAH due to 21-hydroxylase deficiency. The molecular characterization of steroidogenic mRNAs in this tumor indicates an unusual ratio in the expression of the genes for the steroidogenic enzymes, probably accounting for the unusual pattern of serum steroids.

Topics: 17-alpha-Hydroxyprogesterone; 17-Ketosteroids; Adrenal Hyperplasia, Congenital; Adrenocorticotropic Hormone; Child, Preschool; Cosyntropin; Dehydroepiandrosterone; Dehydroepiandrosterone Sulfate; Humans; Hydrocortisone; Hydroxyprogesterones; Leydig Cell Tumor; Male; Pregnanetriol; Testicular Neoplasms; Testosterone

The levels of steroid (17-HOCS, 17-CS and estrogens) and tropic (FSH, LH and ACTH) hormones were assayed and analysed versus histologic pattern and efficacy of combined therapy of tumor in 39 cases of testicular cancer. Decreased level of sex hormones matched by increased concentration of gonadotropins occurred in most patients: marked rise in FSH was observed in seminoma and that in LH--in teratoblastoma and embryonal cancer. Increased levels of 17-HOCS, estradiol, FSH and LH were observed in cases who did not respond to treatment, while lowered concentrations of FSH and LH--in cases of effective treatment. Tropic hormone and 17-HOCS concentrations were registered in cases of relapse.

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenocorticotropic Hormone; Adult; Combined Modality Therapy; Dysgerminoma; Estrogens; Follicle Stimulating Hormone; Hormones; Humans; Luteinizing Hormone; Male; Teratoma; Testicular Neoplasms

The 15-year old female patient G. G. was referred to the outpatient department for child gynaecology on account of a primary amenorrhoea. The endocrinal parameters found (testosterone, oestradiol, 17 OHCS, 17 KS), chromosome analysis, vaginoscopy and the result of a laparoscopy led to the clinical diagnosis of "testicular feminisation". Satisfactory development of secondary female sex characteristics and sudden increase of complaints caused by an inguinal hernia on the left (gonad situated in the inner inguinal ring) prompted us to perform bilateral extirpation of gonads with simultaneous treatment of the inguinal hernia. Histological examination revealed the existence of a seminoma of the right gonad. This case confirm our stand in respect of extirpation of gonads at the onset of puberty, but it also raised the question as to whether it would be advisable to operate at an even earlier stage in order to avoid the risk of malignant degeneration of intra-abdominally positioned testes.

Topics: 17-Ketosteroids; Adolescent; Amenorrhea; Androgen-Insensitivity Syndrome; Castration; Dysgerminoma; Estradiol; Female; Humans; Hydroxysteroids; Male; Puberty; Testicular Neoplasms; Testosterone; Time Factors

Topics: 17-Ketosteroids; Adult; Humans; Leydig Cell Tumor; Male; Middle Aged; Testicular Neoplasms; Testosterone

Two cases of malignant interstitial cell carcinoma of the testis are reported. The first patient had no evidence of a virilizing syndrome. Basal plasma testosterone (T) was decreased, whereas plasma luteinizing hormone, estrone (E1), and androstenedione were elevated. These findings were diagnostic of a defect in T secretion as a result of a partial 17-hydroxysteroid dehydrogenase deficiency as seen in male pseudohermaphroditism. In the second patient, showing gynecomastia and atrophic testis, endocrine studies revealed high plasma T and estradiol (E2); all measured delta 4 and delta 5 precursors of T were elevated resembling the pattern seen in virilizing adrenal carcinomas. Both patients were treated with radiotherapy without demonstrable effect. Chemotherapy--consisting of a combination of cis-platinum, vinblastine, and bleomycin; then cyclophosphamide, doxorubicin, and vincristine; and finally o,p'-DDD--was unsuccessful in reducing tumor bulk. Since malignant Leydig cell carcinomas are rare, this paper reviews the literature and makes recommendations concerning treatment.

Topics: 17-Ketosteroids; Adrenal Cortex Hormones; Aged; Antineoplastic Agents; Biopsy; Gonadal Steroid Hormones; Humans; Leydig Cell Tumor; Male; Middle Aged; Pituitary Hormones, Anterior; Testicular Neoplasms

The occurrence of a giant myelolipoma of the adrenal gland reported in a patient with congenital adrenal hyperplasia (21-hydroxylase deficiency). Associated significant findings include a massive proliferation of adrenocortical cells as an integral part of the myelolipoma and coincidental tumor of the interstitial cells of the testis. The clinical, radiologic, endocrinologic, and pathologic features of this case are correlated with a review of the literature. The additional myelolipomas are also reported here for the first time. Similar lesions have been induced experimentally in rats and provide further evidence suggesting a hyperplastic rather than a neoplastic nature for this complex lesion, at least in its earlier stages.

Topics: 17-Ketosteroids; Adrenal Cortex; Adrenal Gland Neoplasms; Adrenal Hyperplasia, Congenital; Adrenocorticotropic Hormone; Adult; Age Factors; Chronic Disease; Diagnosis, Differential; Female; Humans; Male; Microscopy, Electron; Middle Aged; Primary Myelofibrosis; Testicular Neoplasms

Topics: 17-Ketosteroids; Adult; Castration; Disorders of Sex Development; Female; Gynecomastia; Humans; Leydig Cell Tumor; Male; Testicular Neoplasms; Testosterone

A 22-year-old male with bilateral testicular tumors and the 21-hydroxylase variety of congenital adrenal hyperplasia (CAH) was studied. Preoperatively, on his usual glucocorticoid regimen, his urinary pregnanetriol excretion was increased (8.0-23.5 mg/day), serum LH and FSH were normal to increased (14.3-28.7 mIU/ml and 13.2-19.5 mIU/ml, respectively) and testosterone (T) was normal to decreased (176-600 ng/dl). At surgery, testicular vein concentrations of 17-alpha-hydroxyprogesterone (17-OHP) and adnrostenedione (delta) were increased (30.1 mug/dl and 38.3 mug/dl respectively) while T was decreased (1,503 ng/dl); a positive peripheral vein--testicular vein gradient was not seen for these steroids. Following injection of 10 U of crystalline ACTH into the testicular artery; testicular vein concentrations of 17-OHP, delta and T increased to 729 mug/dl, 2,390 mug/dl and 9,660 ng/dl respectively. Microscopic examination of the testes revealed multinodular tumors composed of polygonal or rounded eosinophilic cells, arranged in cords, nests and clusters. The tumors extended from the hilus and compressed the adjacent testicular tissue. Electron microscopic examination of the tumors showed features, common to steroid-secreting tissues, with abundant smooth endoplasmic reticulum in close proximity to mitochondria which was moderate in number. The adjacent testicular tissue was composed of immature tubules with normally developed Leydig cells in the interstitial tissues. From these data and a survey of previous works, it was postulated that these tumors were dependent upon ACTH for growth and steroid secretion. In view of the high serum LH concentration seen in association with incomplete suppression of adrenal steroid secretion in this study and the association of evidence of gonadotropin secretion with testicular tumors in other CAH patients, LH may also have contributed to the growth of these tumors.

Topics: 17-Ketosteroids; Adrenocortical Hyperfunction; Adrenocorticotropic Hormone; Adult; Androstenedione; Follicle Stimulating Hormone; Humans; Hydroxyprogesterones; Luteinizing Hormone; Male; Microscopy, Electron; Pregnanetriol; Testicular Neoplasms; Testis; Testosterone

Topics: 17-Ketosteroids; Abdominal Neoplasms; Adult; Aged; Animals; Dogs; Humans; Leydig Cell Tumor; Liver Neoplasms; Lymphatic Metastasis; Male; Middle Aged; Neoplasm Metastasis; Testicular Neoplasms; Testosterone

Systematic investigations of the chorionic gonadotropin excretion were performed in 79 patients with testicular tumours. The influence of the hormonal activity in diagnosis and prognosis of metastatic testicular tumours was investigated. It was discovered, that the part of hormonal active testicular tumours is relatively small and that the evidence of the chorionic gonadotropin excretion is only possible in a few cases before the clinical and radiological evidence of metastases. The hormonal activity of testicular tumours is without influence on the survival time of tumour carriers.

Topics: 17-Ketosteroids; Adolescent; Adult; Aged; Antineoplastic Agents; Choriocarcinoma; Chorionic Gonadotropin; Dysgerminoma; Hormones, Ectopic; Humans; Male; Sarcoma; Teratoma; Testicular Neoplasms

Topics: 17-Ketosteroids; Body Height; Body Weight; Child; Estrogens; Humans; Leydig Cell Tumor; Male; Pregnanetriol; Puberty, Precocious; Testicular Neoplasms; Testosterone

Topics: 17-Ketosteroids; Animals; Benz(a)Anthracenes; Carcinogens; Copper; Cryptorchidism; Follicle Stimulating Hormone; Gonadotropins, Pituitary; Guinea Pigs; Luteinizing Hormone; Male; Methylcholanthrene; Neoplasms, Experimental; Rats; Sulfates; Testicular Neoplasms; Zinc

Topics: 11-Hydroxycorticosteroids; 17-Ketosteroids; Animals; Benz(a)Anthracenes; Castration; Cryptorchidism; Follicle Stimulating Hormone; Guinea Pigs; Hexestrol; Luteinizing Hormone; Male; Methylcholanthrene; Neoplasms, Experimental; Pituitary Gland; Rats; Sulfates; Testicular Neoplasms; Testosterone; Zinc

Topics: 17-Ketosteroids; Androstanes; Child; Chorionic Gonadotropin; Dehydroepiandrosterone; Humans; Hydrocortisone; Leydig Cell Tumor; Male; Pregnanetriol; Puberty, Precocious; Sulfates; Testicular Neoplasms; Testis; Testosterone

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenal Hyperplasia, Congenital; Adrenocorticotropic Hormone; Adult; Age Factors; Aldosterone; Body Height; Glucocorticoids; Humans; Hydrocortisone; Male; Puberty, Precocious; Testicular Neoplasms; Testosterone

Topics: 17-Ketosteroids; Adult; Age Factors; Child; Child, Preschool; Germany, West; Gynecomastia; Humans; Leydig Cell Tumor; Male; Puberty, Precocious; Testicular Neoplasms

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenal Glands; Adrenocorticotropic Hormone; Adult; Androgen-Insensitivity Syndrome; Chorionic Gonadotropin; Estradiol; Estriol; Estrogens; Estrone; Follicle Stimulating Hormone; Gynecomastia; Humans; Leydig Cells; Luteinizing Hormone; Male; Medroxyprogesterone; Metabolism, Inborn Errors; Radioimmunoassay; Testicular Diseases; Testicular Neoplasms; Testis; Testosterone; Veins; Vena Cava, Inferior

Topics: 17-Ketosteroids; Adult; Humans; Male; Sertoli Cell Tumor; Testicular Neoplasms

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adult; Aged; Chromatography, Gas; Dysgerminoma; Estrogens; Humans; Isoenzymes; L-Lactate Dehydrogenase; Male; Methods; Steroids; Teratoma; Testicular Neoplasms; Testis

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adolescent; Adult; Age Factors; Aged; Humans; Male; Middle Aged; Prostatic Neoplasms; Testicular Neoplasms

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Castration; Child, Preschool; Humans; Leydig Cell Tumor; Male; Puberty, Precocious; Testicular Neoplasms

Topics: 17-Ketosteroids; Breast Neoplasms; Castration; Female; Genital Neoplasms, Female; Humans; Male; Ovarian Neoplasms; Prostatic Neoplasms; Testicular Neoplasms; Uterine Cervical Neoplasms; Uterine Neoplasms; Vaginal Neoplasms

Topics: 17-Ketosteroids; Adrenal Rest Tumor; Child; Child, Preschool; Diagnosis, Differential; Estrogens; Gynecomastia; Humans; Leydig Cell Tumor; Male; Puberty, Precocious; Testicular Neoplasms

Topics: 17-Ketosteroids; Adrenal Hyperplasia, Congenital; Androsterone; Chromatography, Gas; Cushing Syndrome; Dehydroepiandrosterone; Disorders of Sex Development; Etiocholanolone; Female; Hirsutism; Humans; Klinefelter Syndrome; Male; Polycystic Ovary Syndrome; Pregnanediol; Pregnanetriol; Testicular Neoplasms; Turner Syndrome

Topics: 17-Ketosteroids; Adolescent; Adrenal Gland Neoplasms; Adrenocortical Hyperfunction; Adult; Androstanes; Androsterone; Carcinoma; Chemistry, Clinical; Chromatography, Thin Layer; Cushing Syndrome; Dehydroepiandrosterone; Etiocholanolone; Female; Humans; Kidney Neoplasms; Lung Neoplasms; Male; Methods; Ovarian Diseases; Testicular Neoplasms

Topics: 17-Ketosteroids; Aldosterone; Antineoplastic Agents; Dichlorodiphenyldichloroethane; Estrogens; Gonadotropins; Humans; Liver Neoplasms; Male; Middle Aged; Neoplasm Metastasis; Testicular Neoplasms

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Aged; Chorionic Gonadotropin; Chromatography, Gas; Chromatography, Thin Layer; Humans; Leydig Cell Tumor; Male; Radiometry; Spectrophotometry; Testicular Neoplasms; Testosterone

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Blood; Choriocarcinoma; Chorionic Gonadotropin; Chromatography; Female; Gonadotropins; Growth Hormone; Gynecomastia; Humans; Iodine Isotopes; Male; Placental Hormones; Placental Lactogen; Pregnancy; Radioimmunoassay; Testicular Neoplasms; Urine

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenal Hyperplasia, Congenital; Aldosterone; Child; Child, Preschool; Desoxycorticosterone; Humans; Leydig Cell Tumor; Male; Puberty, Precocious; Testicular Neoplasms; Testosterone; Urine

Topics: 17-Ketosteroids; Androgens; Carcinoma, Embryonal; Estrogens; Gonadotropins; Gynecomastia; Humans; Liver Neoplasms; Lung Neoplasms; Male; Neoplasm Metastasis; Pathology; Retroperitoneal Neoplasms; Testicular Neoplasms; Urine

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adenoma; Adenoma, Acidophil; Carbon Isotopes; Child; Endocrine Gland Neoplasms; Humans; Hydrocortisone; Hydroxyindoleacetic Acid; In Vitro Techniques; Male; Metabolism; Surgical Procedures, Operative; Testicular Neoplasms; Urine

Topics: 17-Ketosteroids; Carcinoma, Embryonal; Chorionic Gonadotropin; Female; Gonadotropins; Gonadotropins, Pituitary; Humans; Male; Pregnancy; Radiotherapy, High-Energy; Surgical Procedures, Operative; Testicular Neoplasms; Trophoblastic Neoplasms

Topics: 17-Ketosteroids; Androgens; Androsterone; Chromatography; Dehydroepiandrosterone; Dysgerminoma; Etiocholanolone; Female; Humans; Male; Neoplasms, Germ Cell and Embryonal; Ovarian Neoplasms; Seminoma; Teratoma; Testicular Neoplasms; 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; Adolescent; Adrenal Gland Neoplasms; Androsterone; Estradiol; Estriol; Estrone; Etiocholanolone; Eunuchism; Gynecomastia; Humans; Klinefelter Syndrome; Male; Steroids; Testicular Neoplasms; Urine