Virilizing adrenocortical carcinoma. Development in a patient with salt-losing congenital adrenal hyperplasia.
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17-Ketosteroids↗
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Results for “ADRENAL CORTEX NEOPLASMS”
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Adrenal incidentaloma: report of 33 cases.
BACKGROUND AND OBJECTIVES: The aim of our study was to review the imaging characteristics, endocrinologic screening and histologic diagnoses of adrenal incidentaloma cases encountered in our institute. METHODS: Thirty-three adrenal incidentaloma cases that had been evaluated in Hacettepe University Hospital between 1985 and 1995 were reviewed retrospectively. Adrenal masses were discovered incidentally by abdominal ultrasonography (USG) and/or computed tomography (CT). Detailed endocrine evaluation was carried out to rule out a possible functioning mass. RESULTS: Among these incidentaloma cases, 2 silent Cushing syndrome (6.06%) cases, 6 silent pheochromocytoma (18.18%) cases, 2 adrenocortical carcinoma (6.06%) cases, and 2 metastatic masses (6.06%) were diagnosed. CONCLUSIONS: An optimal diagnostic approach to an adrenal incidentaloma would consider the results of the biochemical tests and a review of anatomical qualities depicted on CT or magnetic resonance imaging (MRI) while taking into account the previous clinical epidemiologic data.
Abdomen↗
Class III beta-tubulin isotype (beta III) in the adrenal medulla: II. Localization in primary human pheochromocytomas.
BACKGROUND: The Class III beta-tubulin isotype (beta III) is expressed specifically in central and peripheral nervous system neurons at various stages of neuronal differentiation. We have shown previously that beta III is expressed in a differentiation-dependent manner in human neuroblastomas arising in the adrenal medulla and sympathetic chains (Katsetos et al., Clin Neuropathol 13:241-255, 1994). The neuronal distribution of beta III in the developing and mature human adrenal medullae is detailed in the companion article (Katsetos et al., 1998A). METHODS: We have compared the localization of the neuronal beta III to S-100 protein, a sustentacular cell marker, in 14 formalin-fixed, paraffin-embedded primary human pheochromocytomas of the adrenal medulla and 14 adrenocortical tumors (adenomas and carcinomas). RESULTS: In pheochromocytomas, beta III staining was present in all tumors, but the number of stained cells varied in the two neural neoplastic phenotypes. Although the majority of chromaffin-like cells were beta III-positive, there was a lack of beta III in one-third of the tumor cells. Compared to chromaffin-like phenotypes, neuronal (ganglion-like cells) were invariably beta III-positive. Stromal sustentacular cells, stromal fibroblasts, and tumor blood vessels were beta III-negative. Sustentacular cells in pheochromocytomas were S-100 protein-positive, but beta III-negative. Primary adrenocortical tumors were beta III-negative with the exception of rare beta III-positive cells demonstrated in one case. CONCLUSIONS: The distribution of beta III in human pheochromocytomas of the adrenal gland is differentiation-dependent, closely recapitulating chromaffin cell and neuronal phenotypes of the normal adrenal medulla. Our findings indicate that beta III may be used as one of the adjuvant neural markers in the differential diagnosis of adrenal tumors, i.e., pheochromocytoma versus adrenocortical carcinoma. The occurrence of rare beta III-positive cells in cortical carcinomas is exceptional and probably represents the acquisition of a divergent neuroendocrine phenotype. The significance of the latter is unclear, although it may constitute a marker for malignancy.
Adrenal Cortex Neoplasms↗
Adrenocortical carcinoma associated with adrenogenital syndrome in a child.
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Adrenal Cortex Neoplasms↗
Regression of an adrenal cortical carcinoma and its neovascular bed following mitotane therapy: a case report.
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Adrenal Cortex Neoplasms↗
Virilizing adrenal cortical carcinoma.
A 43-year-old man with a 36-year history of virilization due to an adrenal carcinoma is presented. The initial presentation at age 7 with precocious puberty and epiphyseal bone fusion suggested increased androgen effect at a very early age. The patient's 36-year course before his death suggested either a very slow growing adrenal carcinoma or untreated congenital adrenal hyperplasia that progressed to an adrenal carcinoma. Endocrine evaluation showed markedly increased DHEA and DHEA-sulfate levels. These were associated with elevated plasma and urinary estradiol levels and suppressed LH and FSH plasma concentrations. The 24-hour mean levels of cortisol and testosterone were normal. Studies of the circadian periodicity of cortisol showed a disturbed temporal pattern but a normal 24-hour mean concentration that correlated with a normal cortisol production rate. The 24-hour LH secretory pattern showed a decrease in the normal episodic fluctuation of this hormone over the 24-hour period.
Adrenal Cortex Neoplasms↗
Genetic and pathologic findings in a kindred with hereditary sarcoma, breast cancer, brain tumors, leukemia, lung, laryngeal, and adrenal cortical carcinoma.
A familial cancer aggregation comprising sarcomas, brain tumors, leukemias, and carcinomas of breast, larynx, lung, adrenal, cortex, and other sites has been studied from a pathologic--genetic standpoint. Based upon sibships segregating for cancer, the genetic segregation parameter is estimated to be 45.6 +/- 11% which is compatible with that expected for a rare deleterious autosomal gene showing complete dominance. Pathologic review of 16 tumors by bright field microscopy revealed variable occurrences of intranuclear cytoplasmic invaginations, intranucleolar bodies, and acidophilic intracytoplasmic inclusions in eight lesions. Two tumors showed both intranuclear cytoplasmic invaginations and intranucleolar inclusions. Morphological findings coupled with the observed pattern and distribution of cancer in the subject kindred suggest that the cancer-prone genotype interacts with one or more exogenous factors in causing this familial tumor association.
Adolescent↗
Adrenal insufficiency secondary to carcinoma metastatic to the adrenal gland.
Four patients are reported in whom adrenocortical insufficiency developed consequent to carcinoma metastatic to the adrenal glands. Recognition and treatment of this entity may lead to palliation of symptoms and prolongation of useful life. The computerized axial tomographic scanner is a valuable diagnostic aid in diagnosing adrenal enlargement.
Adrenal Cortex↗
Evidence of 11 beta-hydroxylase deficiency in childhood adrenocortical tumors. The plasma corticosterone/11-deoxycorticosterone ratio as a possible marker for malignancy.
In search for a biochemical marker to differentiate between adrenocortical carcinoma (AC) and adenoma (AA), plasma levels of the following steroids were studied preoperatively and postoperatively: 11-deoxycorticosterone (DOC), corticosterone (B), 11-deoxycortisol (S), and cortisol (F). Levels were measured by Sephadex LH-20 chromatography and specific radioimmunoassays. The subjects included eight children ages 2 years, 5 months to 9 years, 10 months. There were three girls and 5 boys with pseudoprecocious puberty due to adrenocortical tumors (histologically, four were AC and four, AA). The preoperative showed that DOC and S levels were elevated in all patients, F levels were elevated in four of eight children when compared with age-matched controls, whereas B was normal. Postoperatively, all levels returned to normal. The ratios of B/DOC and F/S as an index of adrenal 11 beta-hydroxylase activity were calculated. The preoperative ratios of B/DOC were markedly decreased in all patients with AC compared to controls (7.7,4.1,5.9,1.9 versus 23.5, median), but normal in three of four patients with AA (16.2, 29.6, 16.1). The F/S ratios were significantly lower in AC and AA when compared with controls. The data indicate a deficiency in 11 beta-hydroxylation in cases of adrenocortical tumors. Despite a still limited number of patients, the decreased B/DOC ratios may possibly indicate malignancy and could be helpful in distinguishing by biochemical means between benign and malignant adrenocortical tumors.
Adenoma↗
Differential expression of menin in various adrenal tumors. The role of menin in adrenal tumors.
BACKGROUND: Adrenocortical tumors occur as sporadic tumors, as part of the multiple endocrine neoplasia type 1 (MEN1) syndrome, or as part of other hereditary disorders. MEN1 is a tumor suppressor gene located on chromosome 11q13 that encodes a 610-amino acid protein called menin, and plays an important role in the development of MEN1 syndrome. Recent reports indicate that heterozygous germline mutations of this gene are responsible for the disease onset of MEN1. METHODS: To investigate the role of menin in sporadic adrenocortical tumors, the authors examined a series of adrenocortical adenoma cases and a single case of carcinoma and adrenomedulary tumors with the corresponding adjacent tumor tissues using reverse transcriptase-polymerase chain reaction (RT-PCR) for menin mRNA and Western blot analysis for menin protein. Both RNA and protein from these tumors were applied to RT-PCR and Western blot analysis, respectively, although they are not truly quantitative. Primers for RT-PCR were designed to amplify the sequence between exons 2 and 3 of the MEN1 gene. A specific antibody against menin was generated in guinea pigs immunized with the recombinant peptide from the amino acid residues 443-535 of menin made by using glutathione-S-transferase gene fusion. RESULTS: Based on the results of RT-PCR and Western blot analysis, both MEN1 mRNA and menin protein appeared to be highly expressed in Cushing syndrome resulting from adrenocortical adenomas and carcinoma. However, their expression was found to be greatly decreased in primary aldosteronism compared with their expression in Cushing syndrome. Although weak expression of MEN1 mRNA also was detected in pheochromocytoma on RT-PCR, menin expression was not detected in any case of pheochromocytoma by Western blot analysis, possibly due to the lower sensitivity of this assay compared with RT-PCR. Neither MEN1 mRNA nor menin protein was detected in any of the corresponding adjacent tumor tissues examined. CONCLUSIONS: The findings of the current study indicate that menin expression appears to be up-regulated in Cushing syndrome, suggesting that adrenocortical proliferation might be one of the primary lesions in the MEN1 syndrome in which menin might play a significant role in some specific cellular functions.
Adenoma↗
Rat adrenocortical carcinoma 494: an integrated structural, stereological, and biochemical analysis.
Snell adrenocortical tumor 494 was implanted into male Sprague-Dawley rats and recovered 7, 14, 21, 28 or 35 days following initial detection by palpation (7-10 days following transplantation). Electron microscopic, stereological and biochemical analyses of the tumor were compared to adrenals of normal animals to serve as a baseline for further studies of the effects of chemotherapeutic agents on tumor cells. Tumor cells possessed oval or elongated mitochondrial profiles with tubular cristae, one or two very large (greater than 5 micrometer) lipid droplets, abundant ribosomes and coated vesicles, and sparse rough and smooth endoplasmic reticulum. Stereologic evaluation revealed that tumor lipid volume was 41% and mitochondrial volume 29% that of the normal adrenal controls. Tumor nuclei were 2.5 times larger than adrenocortical nuclei while cellular volumes were similar. On a net weight basis, tumor cholesterol was 55%, cholesterol ester 2.2%, and lipid phosphate 25% of respective mean values for normal adrenal glands. The tumor cholesterol: cholesterol ester ratio progressively decreased with time but remained 18-fold greater than the normal adrenal. Plasma corticosterone levels in tumor-bearing rats were elevated 3-fold by 14 days and initial detection. The adrenals of the tumor-bearing host exhibited marked involution, the extent of which was directly related to tumor size.
Adrenal Cortex Neoplasms↗
Real-time ultrasound of normal adrenal glands and small tumors.
The adrenals were studied prospectively with real-time ultrasound in a series of 60 healthy individuals and 13 patients with small adrenal masses sized 8-20 mm in diameter. In only one of the 60 healthy subjects was a normal adrenal gland delineated as a distinct hypoechoic structure. In all other instances only the highly echogenic suprarenal fat could be displayed. Visualization of small tumors was successful in 12 of 13 patients. The suprarenal area and small lesions were best demonstrated by a lateral intercostal approach using longitudinal and transverse scanning planes. The best access was through the 9th/10th intercostal space at the junction with the anterior and middle axillary lines on the right and the posterior axillary line on the left.
Adrenal Cortex Neoplasms↗
Ectopic adrenocorticotropin (ACTH) and corticotropin-releasing hormone (CRH) production in the adrenal gland: basic and clinical aspects.
The hypothalamic-pituitary-adrenal (HPA) axis is integrated in the human stress system and controls the metabolism of many cell systems in the body. Therefore, hypofunction or hyperfunction of the HPA axis potentially threatens the life of the whole organism. Noncontrolled overproduction of its key regulators, CRH and ACTH, causes dysfunction of the stress system. Ectopic secretion of these compounds may be part of extraadrenal paraneoplastic syndromes caused by various benign or malignant tumors. However, ectopic ACTH and CRH may originate from the adrenal itself. A local CRH/ACTH system exists in the normal human adrenal medulla. Overproduction of CRH and ACTH has been documented in pheochromocytomas causing Cushing's syndrome. Finally, ectopic production of ACTH causing Cushing's syndrome has also been demonstrated in adrenocortical cells. This suggests a marked plasticity within the HPA axis and the neuroendocrine cell system.
Adrenal Cortex Neoplasms↗
From incidentaloma to adrenocortical carcinoma: the surgical management of adrenal tumors.
In this article we review the management of benign and malignant adrenal tumors, with an emphasis on oncologic concerns. Concise, logical guidelines for the diagnosis and operative treatment of incidentalomas, aldosteronomas, adrenal Cushing syndrome, virilizing and feminizing adrenal tumors, isolated adrenal metastases, and adrenocortical carcinoma are provided. We also discuss the choice of optimal surgical approach for performing adrenalectomy (laparoscopic, open, hand-assist).
Adrenal Cortex Neoplasms↗
Surgery in patients with unsuspected pheochromocytomas.
In four of the 37 patients operated on for a pheochromocytoma in the last 12 years at Duke Medical Center, the tumor was unsuspected. The first of the four patients had stable blood pressure and pulse during the operative and postoperative period. Although the second patient was thought to have a normal response to surgery, in retrospect her blood pressure showed significant variation. In contrast, the other two patients had wide variations in blood pressure and pulse during the operative and postoperative period. Electron microscopy of the tumor demonstrated numerous dense-core neurosecretory granules suggestive of pheochromocytoma in all four patients. In three of the patients in which measurements could be made, there was a high concentration of norepinephrine (NE) in the tumors that was diagnostic of a pheochromocytoma. Although tissue from the fourth patient was not available for biochemical analysis, his urine and blood tests as well as his ultimate demise from the tumor confirmed that he had a malignant pheochromocytoma. Based on a negative chromaffin reaction--which might be anticipated in a NE-containing pheochromocytoma--one of the patients with the smooth operative course was incorrectly diagnosed as an adrenocortical adenoma. We conclude that although patients with unrecognized pheochromocytomas do not inevitably have wide swings in blood pressure during surgery, this cannot be predicted in a given patient. Thus one should carefully evaluate all patients with suspicious retroperitoneal or intraabdominal masses for possible pheochromocytomas prior to resection of the masses.
Abdominal Neoplasms↗
Expression of DAX-1, the gene responsible for X-linked adrenal hypoplasia congenita and hypogonadotropic hypogonadism, in the hypothalamic-pituitary-adrenal/gonadal axis.
DAX-1, an orphan member of the nuclear hormone receptor superfamily, is responsible for X-linked adrenal hypoplasia congenita (AHC) and the frequently associated hypogonadotropic hypogonadism (HH). The entire DAX-1 genomic region has been sequenced and a putative steroidogenic factor-1 response element has been identified in the promoter region of the gene. The purpose of these investigations was to determine if DAX-1 was expressed in the central nervous system, particularly the hypothalamus and pituitary, in order to better understand the relationship of mutations in this gene to HH associated with AHC. We used Northern blot analysis and reverse transcription PCR to demonstrate that DAX-1 was expressed in the hypothalamus and the pituitary, and to confirm its expression in adrenal cortex and gonads. The expression of DAX-1 in these tissues indicates the involvement of DAX-1 in the development of the reproductive system at multiple levels within the hypothalamic-pituitary-adrenal/gonadal axis. We also observed the expression of DAX-1 in a human adrenocortical carcinoma cell line, NCI-H295, that has features characteristic of the fetal adrenal cortex. Therefore, NCI-H295 cells will be a useful cellular model for investigating the involvement of DAX-1 in the regulation of steroidogenesis.
Adrenal Cortex↗
Expression profiles of COUP-TF, DAX-1, and SF-1 in the human adrenal gland and adrenocortical tumors: possible implications in steroidogenesis.
Chicken ovalbumin upstream promoter-transcription factor (COUP-TF), DAX-1, and steroidogenic factor-1 (SF-1) are orphan members of the nuclear hormone receptor superfamily. COUP-TF and DAX-1 have been shown to negatively regulate the transcriptional activity of SF-1, a steroidogenic cell-specific activator of various steroidogenic cytochrome P450 genes. We therefore examined the expression levels and immunolocalization of COUP-TF, DAX-1, and SF-1 in human adrenal gland (NL) and adrenocortical adenomas, and compared the results with CYP17 expression levels and its enzyme activities to study their potential correlation with adrenocortical steroidogenesis. In NL (n = 10), expressions of COUP-TF, DAX-1, and SF-1 were detected in the nuclei of adrenocortical cells, but not in the medulla. In cortisol-producing adenomas causing Cushing syndrome (CS, n = 20), CYP17 expression was upregulated (298 +/- 2% vs NL 98 +/- 4%), whereas expression levels of both COUP-TFs (COUP-TFI, 52 +/- 5% vs NL 98 +/- 4%; COUP-TFII, 18 +/- 4% vs NL 98 +/- 4%) and DAX-1 (42 +/- 4% vs NL 100 +/- 4%) were reduced. In deoxycorticosterone-producing adenomas (DOC, n = 2), on the other hand, CYP17 expression was extremely reduced (8 and 12% vs NL 98 +/- 4%), whereas DAX-1 expression increased markedly (350 and 360% vs NL 100 +/- 4%). Expression levels of SF-1 did not differ between NL (100 +/- 8%) and CS (106 +/- 10%), but its expression appeared to be decreased in DOC (25 and 20%). These results showed CYP17 expression to be upregulated and downregulated in CS and DOC, respectively, in a manner reciprocal to that of its repressors, COUP-TF and/or DAX-1. In summary, the results indicate that co-localization of COUP-TF, DAX-1, and SF-1 in NL was lost in adrenocortical tumors and that these orphan receptors play an important role in the regulation of steroidogenesis in human adrenals.
Adenoma↗
Ultrastructural pathology of the adrenal glands in Cushing's syndrome.
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Adenoma↗