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We report a case of an adenomatoid tumor (AT) of an adrenal gland with micronodular adrenal cortical hyperplasia (ACH). A 51-year-old man was found to have newly developed hypertension with clinical evidence of primary aldosteronism. A computerized tomogram of the abdomen revealed a solitary mass in the right adrenal gland. He underwent a right adrenalectomy for a presumptive clinical diagnosis of a solitary aldosterone-producing adrenal cortical adenoma. On histopathologic examination, the adrenal gland demonstrated an AT, diagnosed by the characteristic histological features, immunohistochemical stain results, and electron microscopic findings. The surrounding adrenal cortex showed multiple small hyperplastic cortical nodules. After the adrenalectomy, the patient's blood pressure normalized. Primary AT of the adrenal gland coexisting with micronodular ACH associated with hypertension has not been previously reported.

Distinction between benign adrenal cortical proliferative lesions and adrenal cortical carcinoma has been approached by a combination of histological, immunohistochemical, and macroscopical parameters. Modern imaging studies allow detection of small adrenal cortical lesions that may be incorrectly diagnosed. Differentiation between benign and malignant tumors of the adrenal cortex was attempted by microdissection of nine cases of adrenal cortical hyperplasia, 10 cortical adenomas, and 18 adrenal cortical carcinomas with subsequent polymerase chain reaction (PCR) amplification for loss of heterozygosity (LOH) of five microsatellites of putative tumor suppressor gene loci: p53 gene (17p), the neuroblastoma candidate gene (1p), the p16 gene (9p), the von Hippel Lindau gene (3p), and the retinoblastoma gene (13q). None of the hyperplastic lesions or cortical adenomas showed LOH of any of the gene markers used. Conversely, genetic changes were observed in 61% (11 of 18) of the cases of carcinoma. Forty-four percent of the lesions showed LOH for p53 (7 of 16). LOH of 1p, 3p, and 9p were seen in 22%, 22%, and 26%, respectively. LOH of the retinoblastoma gene was seen in 80% or four of five of the informative cases studied. We conclude that LOH studies may be used to distinguish malignant from nonmalignant adrenal cortical proliferations. Relative infrequency of LOH in 3p may furthermore help to differentiate adrenal lesions from clear cell carcinomas of the kidney.

Primary hyperaldosteronism is a potential cause of hypertension. Unilateral adrenal adenoma and bilateral adrenal cortical hyperplasia are the most common causes of primary hyperaldosteronism. Adrenal venous sampling is employed as the gold standard test to differentiate between these two different causes when the results of other studies in the work-up protocol are non-diagnostic or ambiguous. Adrenal venous sampling can be a challenging procedure, especially in the presence of anomalous venous drainage patterns. Knowledge of normal adrenal venous anatomy, as well as possible variants, is therefore important to ensure a successful procedure. The authors describe an unusual variant of left adrenal venous drainage directly into the IVC.

Vascular tumours are very uncommon in the kidney and most of these have been described in single case reports. Some of these can be diagnosed radiologically so the histological features of many of these tumours are not well recognised. We report a case of a haemangioma arising from within the renal vein in a patient with chronic renal failure and bilateral adrenal cortical hyperplasia. The tumour has an unusual sinusoidal architecture and focal extrame-dullary haematopoiesis reminiscent of the spleen. As expected, the endothelial cells stain positively for CD31 and CD34 but negative for CD8. We will also briefly discuss the clinical and histological features of this unusual tumour based on literature reviews.

Summary Pregnant rats were treated with pharmacologically equivalent doses of cortisone, corticosterone and dexamethasone between the 12th und 22nd day of pregnancy. As there was no interference with parturition, the litters could be divided. The developement of the weigths of body, adrenals and pituitary was examined on the day of birth, the 7th and 14th day of life. Pituitary and body weight show an exuberant increase (rebound effect) as compared with the controls with a peak around the 8th day, the adrenal weight showed a corresponding peak around the 12th day of life. The exuberant increase of weight of the adrenals is correlated with an increased mitotic activity within the zona glomerulosa. Nuclear counts/constant area reveal an increase in the size of the cells of the zona glomerulosa together with the exuberant weight increase on the 7th and 14th day and of the zona fasciculata (only on the 14th day, this increase is apparently delayed) as compared with the controls. This phenomenon suggests an alteration of the functional state with an increase in cytoplasmic structures (mitochondria, deposits, endoplasmic reticulum).

A patient is described with oat cell carcinoma of the lung who had increased total 17-hydroxycorticosteroids and normal total 17-ketosteroids in the urine. The urinary steroid patterns were found to resemble those seen in Cushing's syndrome from bilateral adrenal cortical hyperplasia and in normal subjects after ACTH stimulation. The relationships between nonendocrine tumors and Cushing's syndrome, and between cancer and adrenocortical hyperfunction, are discussed. It is concluded that the most reasonable explanation for the adrenocortical hyperactivity, in the light of existing knowledge, is that the "nonendocrine" tumor produces a substance with ACTH-like activity.

Varying daily doses of a steroid inhibitor of fl-hydroxysteroid dehydrogenase, 2a-cyano-4,4,17atrimethylandrost-5-en-17a-ol-3-one, were administered to pregnant rats on the fifteenth to twentieth days of gestation to determine whether this inhibitor could produce the biologic manifestations of the human disease associated with deficient activity of this enzyme. Infants with this disorder have hyperplastic adrenal cortices deficient in activity of 3fi-HSD, incomplete penile development in males resulting in hypospadias with shortening of the anogenital distance, and clitoral hypertrophy without urethral orificial displacement in females. The external sex (anogenital distance) and gonadal sex (presence of ovaries or testes) of experimental fetuses from females receiving the inhibitor were compared to suitable controls. The experimental fetuses had adrenal cortical hyperplasia (5.9 -2_ 1.32 versus 2.2 -+ 26 rag.), deficient histochemical activity of 3fl-HSD in adrenals, testes, and placentas, and no change in histochemical activity of 3aor 17B-HSD. The anogenital distance in males was reduced from the normal of 2.88 -+ 0.12 to 1.26 ± 0.08 ram. in proportion to dose. The females had clitoral hypertrophy but no change in anogenital distance from the normal of 1 mm. This study thus demonstrates an experimental model for the production of the congenital manifestations of this inborn error of metabolism.