PubMed Health⌕ Search

SEARCH · PubMed Health

Results for “ADRENOGENITAL SYNDROME”

Explore indexed PubMed citations for clinical trials, systematic reviews and public health research. Read source abstracts and follow each citation to its original PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 55 records · Page 3Linked to original sources
PubMed2006

Bilateral adrenal myelolipoma associated with adrenogenital syndrome.

Adrenal myelolipoma is a rare benign tumor, occasionally reported in association with endocrine disorders. We report herein a case of bilateral adrenal myelolipoma associated with adrenogenital syndrome caused by 21-hydroxylase deficiency. A diagnosis of 21-hydroxylase deficiency was confirmed by mutation analysis of the CYP21 gene. Our case represents only the second case of bilateral adrenal myelolipoma associated with adrenogenital syndrome caused by 21-hydroxylase deficiency.

Adrenal Hyperplasia, Congenital↗
PubMed1985

[Image diagnosis of adrenal disorders. III. "Nonfunctioning" adrenal adenoma, weak mineralocorticoids producing adrenal carcinoma, congenital adrenogenital syndrome due to 21-hydroxylase deficiency--simple virilizing form, and pheochromocytoma].

The image diagnoses of a case of so-called "nonfunctioning" adrenal adenoma, weak mineralocorticoids producing adrenal carcinoma, congenital adrenogenital syndrome due to 21-hydroxylase deficiency--simple virilizing form--, and 5 cases of pheochromocytoma were studied. In a patient with so-called "nonfunctioning" adrenal adenoma (2.3 X 3.0 X 3.3 cm), in which steroids biosynthesis was confirmed, computed tomography (CT) delineated the tumor shadow with extremely low density, and ultrasonography (US) demonstrated the round tumor echo with homogenous and low echogenicity at the superior region of the right renal pole. Adrenal scintigraphy also showed the tumor image. A weak mineralocorticoids-producing left adrenal carcinoma (3.5 X 3.5 X 3.0 cm) was shown as a heterogenous round tumor at the left lateral portion of the vertebra by CT. On adrenal scintigraphy under dexamethasone pretreatment, there was good uptake in the tumor and disappearance of the contralateral. Both bilateral adrenal images on CT in a patient with congenital adrenogenital syndrome were linear-shaped and markedly enlarged. The enlarged right adrenal was clearly demonstrated by US with an electronic sector scanner but not with an electronic linear scanner, although the left one was hardly shown by either US instruments. Three of 4 patients with pheochromocytomas examined by US were correctly detected, while in the remaining one the tumor image was judged to be a retroperitoneal tumor. CT also correctly demonstrated the former 3 pheochromocytomas, but misjudged the latter one as a pancreatic cancer. Good uptake of Adosterol by bilateral adrenals was shown in a case of extra-adrenal pheochromocytoma. Three of 4 cases of adrenal pheochromocytoma showed the isotope uptake of the contralateral normal adrenal alone. In another case of right adrenal pheochromocytoma, isotope accumulation in the colon obscured whether the isotope uptake in the right adrenal was shown or not.

Adenoma↗
PubMed1993

[Histological structure of the peniform clitoris in congenital adrenogenital syndrome].

Peniform clitoris in 50 girls suffering from congenital adrenogenital syndrome were subtotally removed at the age of 4-6 years and studied by biopsy. A corpus spongiosum more or less developed was found in all cases beneath the corpora cavernosa in the bottom part of corpus clitoridis, epithelial urethral rudiments represented by single or multiple short tubules with transitional epithelium and mucinous glands were found in 46 cases; they were localized in corpus spongiosum or close to it in loose connective tissue. Other urethral rudiments occurring in glans clitoridis were represented by long slight pegs of squamous epithelium extending into corpus spongiosum glandis. Control samples from 20 4-6-year-old girls who had deceased from tumours showed but once heterotopic epithelial urethral rudiments-tubules with transitional epithelium and mucinous glands.

Adrenal Hyperplasia, Congenital↗
PubMed1989

[Nerve-sparing, feminizing genital reconstruction to correct genital virilism: Experience of 25 children with adrenogenital syndrome].

The reconstruction of the virilized genitalia in females with adrenogenital syndrome (AGS) is carried out sparing the dorsal neurovascular bundle either through clitoral recession or reduction with simultaneous vaginoplasty and clitoroplasty. Within seven years, 25 girls aged 6 months to 16 years (mean 2 1/2 years) with AGS had a nerve-sparing genital reconstruction with resection of the clitoral shaft in one session as described by Praetorius. 23 children had a C21-defect and 1 a C11 beta-hydroxylase defect; one child had an external virilisation. All children were followed over an average period of 50 months: 21 had a good cosmetic result, the glans had a normal blood supply and was sensitive to a prick test; in 2 girls nerve integrity was demonstrated by somato-sensory evoked potentials. 2 children had small disturbing skin folds of the newly created labia and 2 girls had a somewhat scrotal appearance of the labia. The introitus caliber measured 14 F in girls operated before 6 years of age; however, a 14- and 16-year-old required intermittent bougienage.

Adolescent↗
PubMed1986

[Morphology of the adrenal cortex in the adrenogenital syndrome with salt loss].

Adrenals were studied in 17 children with adrenogenital syndrome with a salt loss. Adrenals from 9 children who died from mechanical asphyxia served as control. Adrenal weight, the width of adrenal zones were measured, the volumes of nuclei and nucleoli were calculated after having been drawn on millimeter paper. The dilatation of both fascicular and reticular zones, the persistence of a fetal zone were found. Nuclear polymorphism, hyperchromatism and in 2 cases-mitotic figures were observed in the fascicular zone. An increase of nuclear and nucleolar volume is noted in cells of glomerular and fascicular zones. The zones of an increased functional activity (inner part of a glomerular zone and external part of a fascicular one) are distinguished. Alterations if cortex cells are the evidence of their hypertrophy and, to a lesser extent, their hyperplasia.

Adrenal Cortex↗
PubMed1997

Testicular "tumor" of the adrenogenital syndrome: a case report of an unusual association with myelolipoma and seminoma in cryptorchidism.

BACKGROUND: Males with congenital adrenal hyperplasia may develop bilateral testicular masses in early adult life. These are not malignant and generally regress with corticosteroid therapy. The authors report a case occurring in a 44-year-old man with associated seminoma and myelolipoma in an undescended testis. METHODS: The testicular tumors were analyzed by histologic, flow cytometric, and ultrastructural techniques. RESULTS: The tumors in both testes were comprised of polygonal cells with abundant granular eosinophilic cytoplasm, occasionally with brown (lipochrome) pigment and round nuclei of various sizes with prominent nucleoli. These cells were grouped into nodules by dense and sometimes thick fibrous trabeculae in the right testis. The areas corresponding to the fibrous trabeculae in the left (intraabdominal) testis were replaced by mixture of hematopoietic (myeloid) and fatty tissue in various proportions characteristic of myelolipoma. The left testis also had a well demarcated tumor that was diagnostic of seminoma. Electron microscopy demonstrated abundant smooth endoplasmic reticulum, a moderate number of mitochondria with tubulovesicular cristae, lipid droplets, and lipofuscin granules in the polygonal cells. No Reinke's crystals were observed. The patient received corticosteroids for his adrenocorticoid deficiency and also underwent external beam irradiation to the retroperitoneum for seminoma. CONCLUSIONS: This case illustrates an unusual presentation of a testicular tumor in a patient with the adrenogenital syndrome as well as with myelolipoma and seminoma in a cryptorchid testis. The possibility of an associated neoplasm that could be potentially fatal should be considered whenever a testicular tumor of the adrenogenital syndrome continues to grow despite adequate hormonal treatment.

Adrenal Gland Neoplasms↗