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 2,562 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Adrenal cortical carcinomas (ACC) are rare, typically aggressive malignant neoplasms with a reported incidence of 1-2 cases per 1 million population and account for 0.05-0.2 % of all malignancies. The majority of these tumors are functional with approximately 60 % of patients experiencing endocrine symptomatology typically characterized by Cushing's syndrome (40 %) or a mixed hormonal picture of Cushing syndrome seen in association with virilization. Rarely, patients present with a pure hormonal syndrome of feminization or hyperaldosteronism, 6 and 2.5 %, respectively. We report a case of a 76-year-old woman presenting with recently diagnosed hypertension secondary to primary hyperaldosteronism. The patient underwent laparoscopic converted to an open adrenalectomy and a diagnosis of adrenocortical carcinoma (aldosteronoma clinical) was rendered. This case and review of the literature highlight that while rare, aldosterone-secreting adrenal cortical carcinomas may occur. In this case report, we discuss the clinical presentation, pathologic findings, and review the literature for adrenal cortical carcinomas and aldosterone-secreting adrenal cortical carcinomas.
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PMID:Aldosterone-secreting adrenal cortical carcinoma. A case report and review of the literature. 2468 57

Investigation of early human fetal tissue has helped us elucidate the onset of the activation of the pituitary-adrenal axis during human development. Adrenal steroidogenesis and ACTH secretion from the pituitary starts at 7-8 weeks postconception, providing the rationale for prenatal treatment using dexamethasone offered to fetuses at risk of 21-hydroxylase deficiency (21-OHD). Fluctuation of 3beta-hydroxysteroid dehydrogenase (HSD3B2) in human fetal adrenal has several significant meanings. Its activity during early gestation is essential for inhibiting androgen production in the adrenal and safeguarding normal female sexual development. The enzyme may be reduced during mid-gestation in order to maintain pregnancy and to prevent preterm labor. Its reappearance in late gestation is also crucial for fetal maturation and parturition at term. Late-onset circulation failure observed in extremely low birth weight newborns may be associated with the paucity of HSD3B2 in their adrenals. In fetuses with 21-OHD, a proportion of increased 17alpha-hydroxyprogesterone may be converted to dihydrotestosterone through the backdoor pathway and contribute to the virilization of female fetuses.
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PMID:Pituitary-adrenal axis during human development. 2479 Mar 43

Adrenal oncocytoma is an extremely rare neoplasm, which is mostly non-functional. Only five cases of childhood adrenal oncocytoma have been described so far, all of which were hormonally active. Currently, guidelines for management and follow-up are not available. We report a 9-year-old girl with benign adrenal oncocytoma, presenting with severe short-term virilization. After diagnostic work-up the patient underwent laparoscopic unilateral adrenalectomy. For the first 2 weeks following surgery she suffered marked mood swings, irritability and fatigue. There were no other clinical and/or laboratory abnormalities except the rapid drop-down of androgen levels to normal values. Follow-up showed no signs of recurrence and in the absence of signs of adrenal insufficiency, we speculate that, the rapid drop of androgen levels after removal of the tumor might be the reason for the deteriorated psychoemotional condition of our patient.
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PMID:Virilizing adrenal oncocytoma in a 9-year-old girl: rare neoplasm with an intriguing postoperative course. 2551 24

Adrenal cortical tumors are rare in children. Secondary tumors associated with untreated congenital adrenal hyperplasia (CAH) have also been reported in pediatric population. It is difficult for pediatricians to differentiate these 2 lesions.We described a 4.5-year-old girl who presented with symptoms and signs of virilization. Bone age was 9.5 years. Genetic analysis of CYP21A2 and CYP11B1 revealed a heterozygous mutation of CYP11B1 at c.1157C>T (A386V). No germline p53 gene mutation including R337H was detected.The patient was first misdiagnosed as CAH and treated with hydrocortisone for 3 months. Diagnosis of an adrenal cortical tumor was confirmed by laboratory data and abdominal computed tomography. After resection of the tumor, serum steroids normalized and clinical signs receded. The child received no additional treatment and remains disease free after 12 months of close observation. Histological examination showed neoplasia cells with predominantly eosinophilic cytoplasm and few atypical mitotic figures. The proliferation-associated Ki-67 index was <1% detected by immunohistochemistry.Neoplasm is a rare but significant cause of precocious puberty (PP). The possibility of neoplasms should always be considered early to avoid delayed cancer diagnosis and treatment in cases of PP.
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PMID:A Case Report of Adrenocortical Adenoma Mimicking Congenital Adrenal Hyperplasia in a Young Girl. 2610 77

The differential diagnosis of female virilization and infertility can be significantly narrowed using routine laboratory testing. The case presented herein is an example of a 28 year old Caucasian female patient with amenorrhea, hirsutism, and infertility in the context of markedly elevated serum testosterone levels. This case highlights the use of bilateral ovarian vein sampling for testosterone as a means to localize the ectopic testosterone production and to guide future surgical procedures. Adrenal vein sampling procedures are relatively more common than other methods. Ovarian vein sampling is less common, yet in this case, it proved diagnostic. This case demonstrates the needed cooperation of the clinical laboratory and the patient care team performing the catheterization, for this type of testing to be useful. In this unique case, we discovered bilateral production of androgens.
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PMID:A Dozen Testosterone Samples From One Patient, on One Day? 3253 23


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