1/8. food-dependent androgen and cortisol secretion by a gastric inhibitory polypeptide-receptor expressive adrenocortical adenoma leading to hirsutism and subclinical Cushing's syndrome: in vivo and in vitro studies.Aberrant gastric inhibitory polypeptide (GIP) receptor expression in bilaterally hyperplastic adrenals or unilateral adrenal adenomas is a rare form of adrenal hyperfunction. So far, only few cases have been described. In all these cases, cortisol was the predominant steroid released in a food-dependent manner, leading to the development of non-ACTH-dependent Cushing's syndrome. In the present study, we describe a novel case of a GIP receptor-expressive adrenocortical adenomatous nodule, detected incidentally by computed tomography scanning in a 41-yr-old lady with hirsutism but no clinical signs of Cushing's syndrome, on physical examination. Hormonal investigations in morning fasting samples showed slightly elevated androgen levels, low-normal baseline cortisol, normal suppression of cortisol after dexamethasone administration, and ACTH levels that were not suppressed and did stimulate after CRH administration. The elevated urinary free cortisol excretion, in conjunction with an atypical cortisol diurnal rhythm, raised the possibility of an aberrant stimulation of cortisol production by the adrenal tumor. Further studies demonstrated food-dependent secretion of cortisol, which was abolished by prior octreotide administration. Notably, substantial amounts of adrenal androgens were also secreted after food consumption. Removal of the tumor resulted in undetectable cortisol and androgen levels that did not respond to food consumption. Histological examination of the excised tumor revealed an adrenocortical adenomatous nodule originating from the inner zona reticularis, consisting mainly of compact cells. A steroidogenic secretory pattern, indicating the concomitant release of adrenal androgens and cortisol, was also observed in vitro from tumor cells cultured in the presence of GIP. The in vitro secretory response to GIP was higher for the adrenal androgen DHEA, compared with cortisol. The expression of the GIP receptor in tumor cells, but not in the adjacent normal adrenal, was demonstrated by RT-PCR), using specific oligonucleotide probes for this receptor. In summary, we describe a patient with a GIP-expressive cortisol and androgen oversecreting adrenocortical nodule with the unusual presentation of hirsutism and not the typical clinical signs of Cushing's syndrome. It is of note that food intake in this patient provoked a substantial increase in both adrenal androgen and cortisol levels that, together with the histological appearance of this nodule, was compatible with a zona reticularis-derived tumor. Thus, aberrant expression of the GIP receptor does not exclusively involve cells of a zona fasciculata phenotype, as previously reported, but may also occur in other types of differentiated adrenocortical cells.- - - - - - - - - - ranking = 1keywords = physical examination, physical (Clic here for more details about this article) |
2/8. Myxoid adrenal cortical adenoma.Myxoid adrenal cortical adenoma is a rare tumor and, to our knowledge, only 16 cases have been reported. We present the case of a 56-year-old Japanese man who was admitted to hospital because of a right adrenal mass that was discovered during a routine physical examination. The resected mass was well circumscribed and contained canary yellow multinodular regions that were surrounded by a brown gelatinous region. Histologically, the multinodular regions resembled a conventional adrenal cortical adenoma, being composed of solid aggregates of large clear or eosinophilic cells. In the gelatinous region, anastomosing small eosinophilic or vesicular cells were visible within a myxoid stroma that contained large amounts of acidic mucopolysaccharides. light-microscopic findings were consistent with a diagnosis of adenoma. Immunohistochemical staining revealed that a small number of tumor cells were positive for vimentin, and the MIB-1 labeling index was less than 1%. flow cytometry demonstrated that cells were diploid. At the ultrastructural level, many fat droplets were found in the large clear cells in the multinodular regions. Small eosinophilic cells in the myxoid region contained many mitochondria but few fat droplets. There were no findings suggestive of malignancy. Although the adrenal cortex might have the potential to produce connective tissue-type mucin as a consequence of its mesodermal origin, the mechanism of production of acidic mucopolysaccharides in a myxoid adrenal cortical tumor remains to be clarified.- - - - - - - - - - ranking = 1keywords = physical examination, physical (Clic here for more details about this article) |
3/8. Primary non-functional extra-adrenal adrenocortical carcinoma.We present the case of a 52-year-old Jordanian man who was admitted to hospital due to a left sided abdominal mass that was discovered during a routine physical examination. The huge abdominal tumor was initially suspected to be a retroperitoneal sarcoma based on the ultrasound and computed tomography findings. Cytological analysis of the fine needle aspiration smears was unable to define the origin of this mass, and suggested a sarcoma. During laparotomy, the mass was found not connected to the adrenal gland. Only histopathological and immunohistochemical examination of the surgical specimen allowed the diagnosis of adrenocortical carcinoma. This tumor most likely has risen from an extra-adrenal rest. From a review of the medical literature, this case appears to be unique as the first well documented histologically and immunohistochemically reported case of extra-adrenal adrenocortical carcinoma.- - - - - - - - - - ranking = 0.068059351464016keywords = physical (Clic here for more details about this article) |
4/8. 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.- - - - - - - - - - ranking = 0.068059351464016keywords = physical (Clic here for more details about this article) |
5/8. Adrenocortical tumor in a boy: final height is not impaired despite a severe advancement of bone age.The long-term sequelae on the growth pattern in successfully resected virilizing adrenal tumors (ACT) have not been clearly defined. We report on 10 years follow-up of a boy with virilizing ACT until the attainment of final height. This is the first clinical description in a boy with a marked advancement of bone age, indicating that despite advanced physical and skeletal maturity the prognosis on growth is good, provided that regression of virilization is obtained.- - - - - - - - - - ranking = 0.068059351464016keywords = physical (Clic here for more details about this article) |
6/8. 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.- - - - - - - - - - ranking = 0.068059351464016keywords = physical (Clic here for more details about this article) |
7/8. Congenital adrenocortical adenoma: case report and review of literature.Congenital ardrenocortical neoplasms are exceedingly rare. Our review of the medical literature revealed 23 reported cases of adrenocortical neoplasm including this one. Eighteen of these cases were adrenocortical carcinoma and four were grouped as adrenocortical tumor. We have not found any reported case with a histological diagnosis of a congenital adrenocortical adenoma. We present this case of a congenital adrenocortical neoplasm with histological findings consistent with an adrenocortical adenoma in a premature infant aged 27 weeks and 4 days who had a prenatal sonogram showing a cystic right abdominal mass and a physical examination demonstrating a palpable mass.- - - - - - - - - - ranking = 1keywords = physical examination, physical (Clic here for more details about this article) |
8/8. Feminizing adrenal carcinoma.A 50-year old female patient was referred to our clinic. She had systolic-diastolic arterial hypertension, oedemas, physical asthenia, weight loss and a large tumor in the left hypochondrium. Hormone assay showed markedly high values of 17 OHCS, 17 KS and testosterone, and high values of estrogens. The tumor was diagnosed as functional adrenal carcinoma. Removal of the tumor and administration of OP'DDD led to the disappearance of all clinical symptoms and normalization of hormonal values.- - - - - - - - - - ranking = 0.068059351464016keywords = physical (Clic here for more details about this article) |