1/61. Adrenocortical oncocytoma.The histopathology and ultrastructural features of an adrenocortical oncocytoma are reported. The tumour was discovered incidentally during investigation for hypertension in a 72 year old female. Oncocytic tumours of the adrenal cortex are rare, with only 20 examples described in English language reports. Most have been non-functioning and benign, like the present example. Molecular studies may help assess the significance of oncocytic change in the pathogenesis and behaviour of oncocytic neoplasms.- - - - - - - - - - ranking = 1keywords = cortex (Clic here for more details about this article) |
2/61. Anaesthetic management of a patient with adrenocortical tumour.Adrenocortical tumours in children are rare. They produce many changes in haemodynamics and blood chemistry due to hormones of the adrenal cortex. The details of perioperative management and the need for perioperative steroid supplementation are discussed.- - - - - - - - - - ranking = 1keywords = cortex (Clic here for more details about this article) |
3/61. Primary aldosteronism and adrenal incidentaloma.Adrenal incidentaloma are usually found during the assessment of non adrenal disease. In this paper we report the association between a bilateral adrenal hyperplasia and a macronodule of adrenal cortex (adrenal incidentaloma) which is a rare and misleading cause of primary aldosteronism. In the light of this association even if it is likely to remain rare and of those previously published, its existence is an additional reason for suggesting surgical treatment of primary aldosteronism only to patients who satisfy the following criteria: 1) satisfactory control with spironolactone; 2) poor spironolactone tolerance and poor control with other drugs; 3) accept to be operated on and the risk of a possible error.- - - - - - - - - - ranking = 1keywords = cortex (Clic here for more details about this article) |
4/61. Pediatric adrenocortical tumors: molecular events leading to insulin-like growth factor II gene overexpression.It has been previously shown that adrenocortical tumors (ACT) in adults exhibit structural abnormalities in tumor dna in approximately 30% of cases. These abnormalities involve chromosome 11p15 and include loss of heterozygosity, paternal isodisomy, and overexpression of the gene for insulin-like growth factor ii (IGF2), correlating with dna demethylation at this locus. It has been hypothesized that these events occur late in the tumorigenic process in adults and seem to correlate with a worse prognosis. We present 4 pediatric cases of ACT diagnosed at 2.5 yr, 10 months, 12 yr, and 2.2 yr. All 4 patients presented with virilization, and 1 patient also showed signs and symptoms of glucocorticoid excess. The youngest patient's maternal aunt had surgical excision of a more than 15-cm ACT 18 yr previously, but the aunt is doing well at age 23 yr. They all had surgical removal of their tumors. The 2.5-yr-old child also received chemotherapy and radiotherapy because of capsular rupture and, after 3 local recurrences, died 3.3 yr after initial presentation. We investigated all 4 tumors for chromosome 11 structural abnormalities (11p15.5 to 11q23), IGF2 and H19 expression by competitive RT-PCR analysis, and IGF2 methylation patterns by Southern analysis. All 4 tumors (100%) showed a combination of structural abnormalities at the 11p15 locus with mosaic loss of heterozygosity involving 11p. All tumors also had significantly increased IGF2 messenger ribonucleic acid levels relative to normal adrenal (up to 36-fold) and significant IGF2 demethylation (mean, 87%). H19 messenger ribonucleic acid levels were undetectable in 3 of 4 tumors, explained in part by mosaic loss of the actively expressed maternal allele for this imprinted gene. By immunohistochemistry we were able to confirm increased IGF-II peptide levels within the tumor tissue in 10 pediatric patients, including the 4 patients described above. Concomitantly, we also observed nuclear accumulation of p53, suggesting somatic mutations. For the 10-month-old patient, sequencing revealed a p53 germline mutation. We therefore conclude that in pediatric ACT, structural abnormalities of tumor dna and IGF2 overexpression as well as p53 mutations are very common and are therefore less useful for prognosis than in adults. Our findings support the theory that pediatric ACT, whose IGF2 expression and steroidogenesis evoke the phenotype of the fetal adrenal cortex, may arise because of defective apoptosis.- - - - - - - - - - ranking = 1keywords = cortex (Clic here for more details about this article) |
5/61. Characterization of a newly established cell line derived from human adrenocortical carcinoma.BACKGROUND: ACT-1, a new cell line of human adrenocortical carcinoma, has been established and successfully maintained in culture. This study examined the biological characteristics of the cells. methods: The tumor cells were isolated from a surgical specimen of the tumor thrombus and cultured in monolayer. RESULTS: Histologically, the primary tumor was composed of a solid proliferation of large polygonal cells. A part of the atrophic adrenal cortex remained at the periphery of the tumor. The cultured ACT-1 cells were spindle-shaped in morphology and grew exponentially with an approximate population doubling time of 24 h. A chromosomal analysis revealed a modal number of 61 with consistent structural abnormalities of add(3)(q11), add(9)(p11), and add(16)(ql1). The expression of 3beta-hydroxysteroid dehydrogenase was observed in the ACT-1 cells as well as in normal human adrenal glands. Conclusions: The ACT-1 cell line provides a reproducible model system which gives good insight into the oncogenesis of adrenocortical carcinoma.- - - - - - - - - - ranking = 1keywords = cortex (Clic here for more details about this article) |
6/61. adrenocortical carcinoma with cerebral metastasis in a child: case report and review of the literature.OBJECTIVE AND IMPORTANCE: adrenocortical carcinoma (ACC) is rare in the pediatric population, and brain metastasis seldom occurs. CLINICAL PRESENTATION: The authors report a case of metastatic ACC to the brain in a 9-year-old patient who had an adrenal cortex neoplasm removed at 4 years of age, and was free of symptoms for 5 years. Two weeks before admission she complained of blurred vision in both eyes. INTERVENTION: Examination revealed bilateral papilledema, and a magnetic resonance imaging (MRI) of the brain revealed a mass in the left lateral ventricle with extensive vasogenic edema and hydrocephalus. The tumor was removed, and histopathologic examination demonstrated metastatic ACC. CONCLUSION: Although ACC is a rare neoplasm it must be considered in the differential diagnosis of cerebral lesions in patients with a history of this tumor. Periodic long-term brain imaging is suggested as part of the follow up in patients with adrenocortical neoplasms.- - - - - - - - - - ranking = 1keywords = cortex (Clic here for more details about this article) |
7/61. adrenocortical carcinoma metastatic to the orbit.PURPOSE: To describe the clinical course and histopathologic features of a patient with adrenocortical carcinoma metastatic to the orbit. methods: Case report and literature review. RESULTS: A 24-year-old man first came to medical attention because of symptoms referable to a 4.47-kg, nonfunctioning carcinoma of the left adrenal cortex. Several metastases ensued, including a large tumor to the right superior lateral bony orbit with extension to the brain, temporalis fossa, and orbit proper. The tumor was resected with the use of a combined neurosurgical, ophthalmic, and craniofacial approach. The patient died of widespread metastatic disease 15 months after the orbital operation. CONCLUSIONS: Metastasis to the orbit from adrenocortical carcinoma is rare. Surgical resection is the treatment of choice, with adjunctive radiation therapy and chemotherapy in some cases. The prognosis is poor.- - - - - - - - - - ranking = 1keywords = cortex (Clic here for more details about this article) |
8/61. Primary and adjunctive therapy in carcinoma of the adrenal cortex.The absence of a clinically useful staging system for carcinoma of the adrenal cortex has permitted undue pessimism to develop and prevented effective evaluation of postoperative adjunctive therapy. The records of 15 patients undergoing operation for carcinoma of the adrenal gland were studied, and a staging system is proposed. The correlation between the proposed clinical stages and a patient's survival time was highly significant. An aggressive surgical approach toward both the primary tumor and the isolated metastatic lesion appears warranted. In those patients with functioning tumors, determination of urinary steroid levels provides early evidence of recurrence.- - - - - - - - - - ranking = 5keywords = cortex (Clic here for more details about this article) |
9/61. 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 = cortex (Clic here for more details about this article) |
10/61. Major hyperestrogenism in a feminizing adrenocortical adenoma despite a moderate overexpression of the aromatase enzyme.A 30-year-old male was referred for the rapid development of gynecomastia, and dramatic hyperestrogenemia was assessed: plasma estrone, estradiol but also cortisol were not suppressed by high-dose dexamethasone, while gonadotropin pulsatility was completely abolished. A 60-mm right adrenal tumor was evidenced on computed tomography-scan, and the patient underwent adrenalectomy. The tumor was found to express a moderate increase in aromatase activity compared with adjacent non-neoplastic adrenal tissue. Quantitative RT-PCR also showed a weak and non-significant increase in total aromatase mRNA in the tumor compared with normal adrenal tissue. aromatase transcripts were mainly promoter PII-derived, but different patterns of aromatase minor transcripts were found: promoter I.3- and I.6-derived transcripts were identified in the tumor, while only promoter I.4-derived transcripts were found in normal adrenal. This case report demonstrates that a sharp aromatase overexpression is not a prerequisite for clinical and biochemical hyperestrogenism, and further characterizes the aromatase promoter utilization in this feminizing adrenocortical tumor and in the normal adrenal cortex.- - - - - - - - - - ranking = 1keywords = cortex (Clic here for more details about this article) |
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