Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0042755 (masculinization)
 2,562 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

This is a survey of the French South-East Group of Research in Pediatric Surgery (GRECPSE) concerning 18 Solid Adrenal Tumors, observed during a 20-years period (1969-1989) in 11 boys and 8 girls. 13 are tumors of the adrenal cortex (9 adenomas and 4 carcinomas). Clinical virilization is found in 7, cushing's syndrome in 3; while feminizing tumor, Conn's syndrome and non hormone-secreting form are quite exceptional (1 case each, respectively). 5 are adrenal pheochromocytomas (4 benign tumors and 1 malignant with local invasion). Ultrasonography, CT Scan and MIBG Scintigraphy are modern procedures which permit localization of the small and non palpable tumors with high accuracy. They make easier surgical management. Differentiation between benign and malignant tumors of the adrenal glands still remains the main problem. The course of the disease is surely the only distinguishing parameter. However weight tumor and selective histopathological characteristics are useful in predicting the prognosis and behavior of such tumors = Mitotic activity for adrenocortical tumors, extensive areas of necrosis and small cells for pheochromocytomas have the highest discriminating value.
 ...
PMID:[Solid tumors of the adrenal gland in children (excluding neuroblastomas). A study of a series of 18 cases]. 269 66

Tumors are rare, but well-documented causes of precocious puberty in both sexes. The therapeutic and prognostic implications of a diagnosis of cancer require that the presence of a neoplastic process be ruled out in any case of precocious puberty. Granulosa-cell tumor of the ovary and Leydig-cell tumor of the testis are the most frequent gonadal tumors inducing precocious pseudopuberty in the two sexes. Adrenal tumors sustain a variety of endocrine syndromes, the most frequent one being virilization with or without hypercortisolism. Pure feminizing adrenal neoplasms have been described. For reasons not yet well understood, hypothalamochiasmatic glioma (beta-HCG) secreting tumors have almost never been described in association with female precocious puberty. Among these neoplasia, pineal germ-cell tumor inducing sexual maturation must be included. Hypothalamochiasmatic glioma and craniopharyngioma are the two cerebral tumors capable of inducing true precocious puberty. Even if equally distributed between both sexes, these tumors interfere with sexual maturation less frequently in girls than in boys. Hypothalamic hamartoma is considered a benign tumor, since it does represent a space-occupying mass. It more correctly could be called a malformation if its histologic characteristics are recalled. This cerebral lesion is now frequently described in children with true precocious puberty, probably because of improved diagnostic imaging methods.
 ...
PMID:Oncologic causes of precocious puberty. 270 2

Adrenal and gonadal functions were evaluated on two adult cousins with male pseudohermaphroditism due to congenital 3 beta-hydroxysteroid dehydrogenase deficiency (3 beta-HSD) without clinical salt-losing. Both patients had been reared as females since birth. Case 1 presented at age 17 with perineal hypospadias virilization without gynecomastia and a female to male gender role change at puberty. Case 2 had previously undergone bilateral orchidectomy in childhood and presented "primary amenorrhea", absence of virilization and a female gender role at the age of 24. In the basal state, as well as after ACTH and hCG stimulation, 3 beta-hydroxy-5-ene-steroid levels were disproportionately elevated, resulting in abnormal 3 beta-hydroxy-5-ene: 3-oxi-4-ene steroids ratios. Normal basal serum cortisol with inadequate cortisol response to ACTH was observed in both patients. Elevated basal plasma renin activity (PRA) and normal basal serum aldosterone (ALDO) were present in both subjects. After ACTH stimulation serum ALDO rose adequately in Case 1 but subnormally in Case 2. Salt restriction resulted in an increase in serum ALDO and no salt loss in Case 1 whereas in Case 2 the substantial rise in PRA and serum ALDO were unable to prevent slight urinary sodium loss. Case 1 had normal basal serum testosterone with subnormal response to hCG stimulation. Incubation of testicular tissue in vitro with [3H]DHEA resulted in large Androstenediol production but diminished testosterone conversion confirming the 3 beta-HSD deficiency in the testes. We conclude that (1) absence of gynecomastia and a female to male gender role change may be observed in the male pubertal presentation of nonsalt-losing 3 beta-HSD deficiency and (2) the different functional behavior of zona glomerulosa in our patients suggests the presence of variable degrees of 3 beta-HSD deficiency in the zona glomerulosa of the nonsalt-losing form.
 ...
PMID:Male pseudohermaphroditism due to nonsalt-losing 3 beta-hydroxysteroid dehydrogenase deficiency: gender role change and absence of gynecomastia at puberty. 282 19

A 15-year-old girl who presented with primary amenorrhea and virilization had an adrenocortical adenoma that secreted predominantly testosterone. To our knowledge, she is the first peripubertal and second youngest patient with a testosterone-secreting adrenal tumor described. Serum dehydroepiandrosterone sulfate and urinary 17-ketosteroid and 17-hydroxycorticosteroid levels were normal. A tumor was located by a computed tomographic (CT) scan and by uptake of 6-beta-[75Se] selenomethylnorcholesterol. Microscopic examination of the tumor showed typical features of an adrenocortical adenoma with no histologic features characteristic of Leydig cells. Postoperatively, her hirsutism regressed, she rapidly went through puberty, and regular monthly menstruation started four months later. Finding the source of testosterone in a virilized patient can be difficult. Eleven of the 14 previously described patients with testosterone-secreting adrenal tumors initially underwent misdirected surgery on the ovaries. Review of these cases revealed that results of hormone stimulation and suppression tests are unreliable and that these tumors are usually large. Therefore, CT scanning of the adrenal glands is recommended in all patients suspected of having a testosterone-secreting tumor. If the adrenal glands on CT scan are normal, then surgery directed at the ovaries can be undertaken. Adrenal and ovarian vein catheterization is rarely necessary.
 ...
PMID:Testosterone-secreting adrenal adenoma in a peripubertal girl. 331 60

An orally active progestagen, 17 alpha-ethynyl-19-nor-testosterone-17 beta-acetate-3-cyclopeotyl enol-ether (ENTACP) was found to be a potent antiestrual and contraceptive agent in rats. It was evaluated with respect to: 1) progestational activity, 2) pregnancy maintenance, 3) androgenic activity, 4) masculinization of female fetuses, 5) uterotropic activity, 6) effect on adrenal and adrenocortical function, 7) effect on organs and body weight, 8) effect on pregnancy, 9) storage in body fat. ENTACP was twice as effective in producing secretory changes of the endometrium as its parent compound. Pregnancy was not maintained; however, after 22 days, resorption sites were still visable. ENTACP was about 1.5 times as effective as its parent compound in stimulating accessory organs during daily administration. A daily dose of .9 mg orally produced masculinization of the female fetuses, but did not interfere with parturition. Resorption of fetuses occurred more frequently in rats treated with ENTACP than with other compounds tested. ENTACP behaved like an estrogen in stimulating uterine growth. Adrenal weight was significantly increased with ENTACP administration and it reduced the capacity of the adrenal cortical tissue to respond to ACTH. Body weight gain suffered in direct proportion to dose administration. Adrenals, hypophysis, and thyroid increased when expressed in mg/100 gm body weight, and thymus decreased. The oral daily dose of .5 mg/animal from day 1-11 after mating did not prevent pregnancy. At the 5 mg/animal dose nidation was interfered with. Oral administration was not followed by storage of steroid material in body fat.
 ...
PMID:Biological profile of quingestanol acetate. 581 52

Testicular Leydig cell hyperplasia was observed in two brothers presenting with progressive sexual precocity at 2 yr of age. Virilization was shown to result from increased secretion rather than decreased clearance of gonadal testosterone. Testosterone hypersecretion appeared to be gonadotropin independent, as basal and gonadotropin-releasing hormone-induced serum LH concentrations were low by both RIA and bioassay. Adrenal steroidogenesis was demonstrated to be normal by ACTH stimulation, dexamethasone suppression, and split adrenal venous function tests. Testicular histology revealed immature reproductive structures in the 2 yr old, but advanced spermatogenesis in the 3 yr-old brother. The etiology of both Leydig cell hyperplasia and reproductive testicular maturation in the absence of significant gonadotropin secretion remains to be established.
 ...
PMID:Testicular leydig cell hyperplasia as a cause of familial sexual precocity. 678 May 88

A seven year-old boy with non-classic 21-hydroxylase deficiency had transient hormonal evidence of central precocious puberty within three months of beginning glucocorticoid treatment for rapidly progressive somatic virilization. Adrenal-derived sex hormones were normalized promptly by hydrocortisone treatment. GnRH-stimulated LH levels and basal testosterone returned to the prepubertal range without further intervention after six months of glucocorticoid therapy. This is the first report of transient central precocious puberty in the mild non-classic form of congenital adrenal hyperplasia due to 21-hydroxylase deficiency.
 ...
PMID:Transient central precocious puberty in non-classic 21-hydroxylase deficiency. 882 7

Computed tomography (CT) plays a leading role in the evaluation of nonmalignant disease of the adrenal gland. CT is highly accurate in the localization of adrenal masses in patients with diseases associated with hyperfunctioning adrenal glands such as Cushing syndrome and Cushing disease, Conn syndrome, adrenal tumors leading to virilization or feminization, and pheochromocytomas. CT permits a specific diagnosis of acute or subacute adrenal hematoma and myelolipoma. Hematomas are round to oval and have increased attenuation (50-90 HU) that decreases on follow-up CT scans. Myelolipomas typically manifest as a well-defined suprarenal mass with an attenuation of-30 to -115 HU. Adrenal cysts are usually round to oval and manifest as a hypoattenuating mass with a smooth, thin wall. CT is useful in the evaluation of patients with Addison disease, particularly the subacute form secondary to tuberculosis or disseminated histoplasmosis. Findings typically include bilateral adrenal enlargement with a central necrotic area of hypoattenuation and peripheral enhancement. Thin-section unenhanced CT permits accurate measurement of attenuation and can be used to differentiate adrenal adenoma from metastasis in a cancer patient with an indeterminate mass: Attenuation of 10 HU or less usually indicates adenoma rather than cancer. If the mass is found incidentally at contrast material-enhanced CT, delayed scans obtained as early as 5-15 minutes after intravenous administration of contrast material appear to have comparable accuracy.
 ...
PMID:Spectrum of CT findings in nonmalignant disease of the adrenal gland. 953 86

Discerning malignancy in adrenal tumors largely influences disease management and is, therefore, of utmost importance to both patient and physician. Clinical presentation (e.g. virilization) and baseline hormonal evaluation (e.g. high serum DHEAS) are occasionally of great value but usually provide only limited help in predicting malignancy. The probability of malignancy is clearly related to tumor size, as almost all lesions <3 cm are benign whereas a diameter of >6 cm indicates a high risk of malignancy. Computed tomography (CT) and magnetic resonance imaging (MRI) both contribute significantly to the characterization of adrenal masses. If the attenuation of a homogeneous mass with smooth border is 10 Hounsfield units or less in unenhanced CT the diagnosis of a lipid rich adenoma is established. Similarly, enhancement washout of more than 50% in CT at 10-15 min suggests a benign lesion. In MRI both rapid contrast enhancement after gadolinium followed by rapid washout and signal intensity loss using opposed-phase image in chemical shift analysis also indicate the presence of an adenoma. In contrast, adrenal carcinomas--but also pheochromocytomas--typically present as inhomogeneous lesion with intermediate-to-high intensity on T2 images in MRI. Margins and enhancement after contrast media in CT are irregular in adrenal carcinoma. Other imaging techniques either offer little additional information (NP-59 scintigraphy) or have not yet been fully established (positron emission tomography). Fine needle aspiration/cut biopsy is at present restricted to patients with a known extra-adrenal malignancy and suspected adrenal metastasis as the only evidence of disseminated disease. Adrenal tumors classified as benign undergo follow-up imaging to assess tumor growth. If an increase in diameter of >1 cm is seen, surgical removal is recommended. Even after tumor removal the diagnosis of dignity may remain difficult. Diagnostic scores together with new immunohistological markers are the methods of choice to assess malignancy. In conclusion, an interdisciplinary approach with a structured use of available diagnostic tools is needed to classify adrenal tumors correctly.
 ...
PMID:Adrenal tumors: how to establish malignancy ? 1523 62

Adrenocortical tumors are rare and mostly non-secreting; their discovery is incidental. When secreting, they produce steroid excess and result in a clinical presentation such as the Cushing syndrome, primary aldosteronism, virilization or feminization syndrome. Such tumors are mostly sporadic but can belong to hereditary syndromes predisposing to tumors. The diagnosis of secreting adrenocortical tumors is based upon clinical presentation and biological data associated with specific biological assessments. Adrenal imaging has been considerably improved with the development of CT scan, which can be completed by MRI if necessary. Most of adrenocortical tumors are adenoma, nevertheless some of them can be malignant and the prognosis of such carcinomas is poor. Management of secreting adrenocortical tumors requires surgery in most of the cases and laparoscopic access is now widely used and provides good results in the treatment of benign tumors.
 ...
PMID:[Adrenocortical secretory tumors]. 1548 55


1 2 3 Next >>