Cases reported "Adrenal Cortex Neoplasms"

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11/61. Cushing's syndrome due to bilateral adrenocortical adenomas with unique histological features.

    Cushing's syndrome due to bilateral cortisol-secreting adenomas rarely occurs. We present a case of Cushing's syndrome due to bilateral adenomas. Both adenomas had distinct cell compositions, and were compared with emphasis on immunohistochemical and enzyme histochemical analysis for cytochrome P450(11beta) and 3beta-hydroxysteroid dehydrogenase (3betaHSD). A 37 year-old female was diagnosed with ACTH-independent Cushing's syndrome based on physical findings and hormonal evaluation. High-resolution CT scan showed bilateral adrenocortical adenomas and atrophied glands. 131I-methylnorcholesterol incorporation into both glands suggested both adenomas were functional. Clinical diagnosis prior to surgery was ACTH-independent Cushing's syndrome due to functioning bilateral adenomas. The left adrenal gland was totally resected, while the right one was partially resected by laparoscopic approach. Both adenomas were black on cut sections, and were comparatively evaluated by immunohistochemical and enzyme histochemical analysis for P450(11beta) and 3betaSD. The left adenoma was 1.6 cm in diameter and had a complex cellular composition and enzyme expression similar to that of primary pigmented nodular adrenocortical disease (PPNAD), while the right adenoma was 1.8 cm in diameter with compact cells typical of a solitary cortisol-producing adenoma. Adjacent bilateral adrenal cortex showed marked atrophy, but contained several micronodules. serum cortisol levels, both at basal and after a low dodexamethasone, normalized thirteen months after surgery. In conclusion, the present case of Cushing's syndrome with bilateral adrenal adenomas demonstrated for the first time the simultaneous occurrence of two distinct adenomas, an ordinary cortisol-producing adenoma and a PPNAD-like adenoma. Further case reports of multiple adrenal adenomas should be well-analyzed to clarify whether the results from this case represent a new subgroup of ACTH-independent Cushing's syndrome.
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12/61. Adrenocortical blastoma.

    We report a previously undescribed virilizing malignant adrenocortical tumor in an 21-month-old infant with elevated serum alphafetoprotein. The tumor consists of a peculiar mixture of immature epithelial and mesenchymal elements as well as slit-like spaces partially lined by primitive epithelial cells. Focally, the tumor has features reminiscent of the normal embryologic development of the adrenal cortex. A panel of immunohistochemical stains revealed only vimentin reactivity. We propose the term "adrenocortical blastoma" for this unusual neoplasm.
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13/61. Pre-Cushing's syndrome: a case report.

    A 67-year-old man affected by prostate cancer was incidentally found to have a nodular enlargement of the left adrenal gland without apparent changes in hormonal status. The adrenal mass was found to be scintigraphically active, the radiolabelled compound being concentrated in its context with a consensual suppression of the contralateral uptake. The patient underwent a resection of the adrenal tumor. Histologically and biochemically, the adrenal mass was found to be a non-functioning adenoma. The radioisotopic uptake along with the non-hormonal activity prompted us to call this tumor "Pre-Cushing's syndrome" of the adrenal cortex.
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14/61. adenomatoid tumor of the adrenal gland with micronodular adrenal cortical hyperplasia.

    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.
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15/61. Pseudoglandular myxoid adenoma of the adrenal gland.

    Myxoid tumors of the adrenal cortex are rare. To the best of our knowledge, only 17 cases have been reported to date, including 10 carcinomas and 7 adenomas. The myxoid areas of these tumors are often evident grossly, and their extent is variable. We report on a case of a myxoid adenoma of the right adrenal gland in a 45-year-old male with a prominent pseudoglandular arrangement in more than 90% of the tumor mass, and with a minor component represented by anastomosing cords of tumor cells floating in pools of myxoid material. In addition, after extensive examination, we found foci of typical adrenocortical adenoma. Grossly, the tumor was yellowish without discernable gelatinous changes. Most of the tumor cells had a moderate amount of clear vacuolated cytoplasm and contained numerous oil-red-O-positive lipid droplets. Extracellularly, in the lumens of pseudoglands and on the background, we noticed myxoid material that stained positively with alcian blue (pH 2.5) and weakly positively with mucicarmine and the PAS method. Immunohistochemical examination showed positivity of the tumor cells for vimentin and cytokeratin CAM5.2. synaptophysin was weakly positive only focally. Cytokeratin AE1/AE3, EMA, and CEA were negative.
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16/61. A case of aldosterone-producing adrenocortical adenoma associated with a probable post-operative adrenal crisis: histopathological analyses of the adrenal gland.

    We describe a case of aldosterone-producing adrenocortical adenoma (APA) associated with a probable post-operative adrenal crisis possibly due to subtle autonomous cortisol secretion. The patient was a 46-year-old female who suffered from severe hypertension and hypokalemia. CT and MRI scans revealed a 2-cm diameter adrenal mass. The patient's plasma aldosterone level was increased, and her plasma renin activity was suppressed, both of which findings were consistent with APA. Cushingoid appearance was not observed. Morning and midnight serum cortisol and plasma adrenocorticotropic hormone (ACTH) levels were all within the normal range. Her serum cortisol level was suppressed to 1.9 microg/dl as measured by an overnight 1-mg dexamethasone suppression test, but was incompletely suppressed (2.7 microg/dl) by an overnight 8-mg dexamethasone suppression test. In addition, adrenocortical scintigraphy showed a strong uptake at the tumor region and a complete suppression of the contra-lateral adrenal uptake. After unilateral adrenalectomy, she had an episode of adrenal crisis, and a transient glucocorticoid replacement improved the symptoms. Histopathological studies demonstrated that the tumor was basically compatible with APA. The clear cells in the tumor were admixed with small numbers of compact cells that expressed 17alpha-hydroxylase, suggesting that the tumor was able to produce and secrete cortisol. In addition, the adjacent non-neoplastic adrenal cortex showed cortical atrophy, and dehydroepiandrosterone sulfotransferase immunoreactivity in the zonae fasciculata and reticularis was markedly diminished, suggesting that the hypothalamo-pituitary-adrenal (HPA) axis of the patient was suppressed due to neoplastic production and secretion of cortisol. Together, these findings suggested that autonomous secretion of cortisol from the tumor suppressed the HPA axis of the patient, thereby triggering the probable post-operative adrenal crisis. Post-operative adrenocortical insufficiency should be considered in clinical management of patients with relatively large APA, even when physical signs of autonomous cortisol overproduction are not apparent.
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17/61. Adrenocortical oncocytoma: case report.

    We report the case of an adrenocortical oncocytoma of the left adrenal cortex in a young woman. physical examination revealed a mass in the left upper quadrant of the abdomen using abdomen ultrasound, computed tomography (CT) and magnetic resonance imaging (MRI), which allowed the identification of a large and inhomogeneous mass between the left kidney and the spleen. The lesion was not endowed of any specific radiologic characteristic nor bysided by any biochemical activity that could allow a radiological presurgical diagnosis. Surgical resection led to the diagnosis of adrenocortical oncocytoma, with no aspects revealing malignant potential.
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18/61. Adrenal black adenoma associated with preclinical Cushing's syndrome.

    A black adenoma of the adrenal gland was laparoscopically removed from a 60-year-old man who presented with severe hypertension. Although laboratory findings were indicative of preclinical Cushing's syndrome, there were no clinical features characteristic of Cushing's syndrome. Microscopically, the tumor showed a proliferation of polygonal cells containing numerous brown-pigmented granules. Special staining studies revealed these granules to be lipofuscin. Electron microscopy also identified lipofuscin and lysosomes in these cells. The morphological appearance of the tumor and the adjacent atrophic non-tumorous adrenal cortex supports the assumption that the black adenoma caused preclinical Cushing's syndrome.
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19/61. Virilizing adrenal cortical neoplasm arising ectopically in the thorax.

    We describe a 44-yr-old woman with a 12-yr history of clinical virilization and serum testosterone levels up to 28.1 nmol/L (normal range, 1-3.3 nmol/L) in whom repeated clinical evaluation and surgical procedures failed to reveal the source of androgen production. At the time the patient was referred to the Clinical Center of the NIH, an intrathoracic mass was seen on upper cuts of an abdominal computer-aided tomography scan, confirmed by computer-aided tomography scan and magnetic resonance imaging of the chest. A 6 x 5 x 3.5-cm mass, attached to the posterior pericardium, was removed by thoracotomy. Pathological examination revealed an adrenal cortical neoplasm of uncertain malignant potential that contained testosterone, 11-deoxycortisol, progesterone, and 17-hydroxyprogesterone. After the operation, the patient's serum testosterone levels decreased to the normal range. Ectopic adrenal cortical rests in the thorax and neoplasms arising from these rests are extremely rare, and we are not aware of a similar case previously reported. In women with virilization, radiological studies of the thorax as well as other reported sites of ectopic adrenal cortex should be performed if radiological studies of the abdomen and pelvis fail to locate the source of the neoplasm.
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20/61. Mineralocorticoid excess due to precursors only in a patient with adrenal cortical carcinoma.

    A patient with carcinoma of the adrenal cortex presented with mineralocorticoid excess due to hypersecretion of deoxycorticosterone, which is exceedingly rare. Backache was the only symptom and an unexplained hypokalaemia was the only sign. Because of the abnormal synthesis of steroid precursors in these tumours a urinary steroid profile may be helpful as a diagnostic tool in such cases.
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