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Adrenocortical Carcinoma (adrenocortical + carcinoma)

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Medical Sciences	100%

Selected Abstracts

Adrenocortical carcinoma: Retrospective study of 14 patients experienced at a single institution over 34 years

INTERNATIONAL JOURNAL OF UROLOGY, Issue 7 2007
Sachiyo Nishida
Objective: To review clinical outcome of patients with adrenocortical carcinoma experienced at a single institute over 34 years. Methods: The study included 14 patients who were diagnosed as having the disease and were treated at the Department of Urology, Sapporo Medical University Hospital between 1973 and 2006. Their clinical features and outcomes were reviewed. Results: Of the 14 patients, there were nine men and five women. The median follow-up period was 13.0 months (range, 1,213). Two patients were classified as having stage II disease, seven as stage III and five as stage IV. The disease was completely removed in eight patients and incompletely in three. Two other patients received exploratory laparotomy only. The remaining one patient had no indication for surgery. The median survival periods were 2 months in patients with stage IV and 108 months in those with stages II and III (P = 0.136). Mitotane treatment in the adjuvant setting did not clearly affect the clinical courses of patients without metastasis. However, the treatment was effective for metastasis that was repeatedly developed as late recurrence in one patient. Three patients with metastasis at diagnosis received combination chemotherapy with etoposide, doxorubicin and cisplatin (EDP) with or without mitotane treatment, to which lung metastasis completely responded in one patient. Conclusions: Adrenocortical carcinoma is a rare disease but frequently recurs. The best chance of survival may be achieved by early detection and complete surgical removal. There may be patients who possibly benefit from mitotane treatment with or without EDP, although this remains to be conclusively determined. [source]

Adrenocortical carcinoma with delayed cutaneous metastasis

JOURNAL OF CUTANEOUS PATHOLOGY, Issue 7 2008
Elizabeth K. Satter
Adrenal cortical carcinoma (ACC) is an uncommon and aggressive malignancy. Patients often have metastatic disease at initial presentation, with the most common sites being the liver, local lymph nodes, lungs, peritoneum and bone. Despite a high frequency of metastases, there are only isolated reports of ACC that have metastasized to the skin. Herein, we report a case of an 82-year-old woman who presented with a cyst-like lesion on her back, which on biopsy proved to be ACC metastatic from a primary tumor diagnosed 30 years previously. [source]

Adrenocortical carcinoma in children: A role for etoposide and cisplatin adjuvant therapy?

PEDIATRIC BLOOD & CANCER, Issue 5 2003
Preliminary report
No abstract is available for this article. [source]

ES07 MICROARRAY GENE EXPRESSION ANALYSIS OF HUMAN ADRENOCORTICAL TUMOURS

ANZ JOURNAL OF SURGERY, Issue 2007
P. S. H. Soon
Introduction Adrenal tumours are common, occurring in 7% of patients over the age of 50. Adrenocortical carcinomas, however, are rare, with an incidence of two per million population per year. The management of adrenocortical tumours is complex, compounded by the difficulty in discriminating benign from malignant tumours using conventional histology. A molecular marker which could reliably distinguish between the two groups would be valuable in patient management. Objectives The aim of this study was to identify molecular markers which will discriminate between adrenocortical carcinomas and adenomas using microarray gene expression analysis. Methods This study used RNA from 6 normal adrenal cortices, 16 adrenocortical adenomas and 12 carcinomas. Only samples with an RNA integrity number of 7.5 or greater were used. The samples were hybridised to Affymetrix HGU133plus2.0 genechips. Data analysis was performed with Partek and affylmgui softwares. Results Using a cutoff of B > 2 and M > 2 or <,2, 217 genes were found to be significantly differentially expressed between adrenocortical adenomas and carcinomas. Of these genes, 120 were unpregulated while 97 were downregulated. Seven of these genes have been selected for validation studies with real time reverse transcription polymerase chain reaction. Conclusion In this study, we found 217 genes which were significantly differentially expressed between adrenocortical adenomas and carcinomas. With validation and further studies, these genes will provide further insight into the pathogenesis of adrenocortical tumours as well as possibly proving to be reliable discriminators between adrenocortical adenomas and carcinomas. [source]

The prognostic value of two different histopathological scoring systems for adrenocortical carcinomas

HISTOPATHOLOGY, Issue 2 2007
H P Van't Sant
Aims:, To compare two different multiparameter histopathological scoring indices and determine their prognostic value in patients presenting with adrenocortical carcinoma (ACC). Methods and results:, Seventy-nine adrenal cortical tumours were divided into adenomas (n = 17), non-metastatic carcinomas (n = 24) and carcinomas with metastatic disease and/or local recurrence during follow-up (n = 19) or at time of presentation (n = 19). All cases were scored according to the Weiss revisited index (WRI) and the Van Slooten index (VSI). Both scoring indices yielded a significantly different score (P < 0.005) between adenomas and carcinomas. Non-metastasized carcinomas had a lower score with both indices compared with carcinomas with metastases at the time of presentation (VSI, P = 0.017; WRI, P = 0.019). The VSI also distinguished ACC that had metastasized at any time from those that had not (P = 0.015). Cancer-specific survival in patients with metastasized ACC correlated with the scores for both indices (VSI, P = 0.0078; WRI, P = 0.0025). Time from diagnosis of ACC to development of metastatic disease was correlated with the WRI (P = 0.036, r = ,0.350). Conclusions:, The VSI and the WRI have equal validity in the correct categorization of ACC and adenomas. Furthermore, both indices show a correlation with survival for metastasizing ACC. [source]

Adrenocortical carcinoma: Retrospective study of 14 patients experienced at a single institution over 34 years

Application of cardiopulmonary bypass for resection of renal cell carcinoma and adrenocortical carcinoma extending into the right atrium

INTERNATIONAL JOURNAL OF UROLOGY, Issue 3 2006
TATSUMASA OCHI
Aim:, The application of cardiopulmonary bypass to atrial involvement represents an important advance that has improved the safety and technical efficacy of a difficult surgical undertaking. Our experiences of the management of extended thrombi into the right atrium in patients with retroperitoneal malignancy using a cardiopulmonary bypass were discussed. Methods:, Data were reviewed for five patients (two men and three women; mean age, 60.4 years; range, 49,79 years) with retroperitoneal tumors displaying intracardiac tumor extension. Tumors originated in the right kidney in four patients, and in left adrenal gland in one patient. Cardiopulmonary bypass was used in all cases. Results:, Mean total blood loss was 6059 mL. Mean operative time was 14.7 h. No intra- or postoperative complications due to surgical technique were encountered, and no significant bleeding occurred during incision of the inferior vena cava or after removal of tumor thrombus. The follow-up period ranged from 3 to 20 months with a mean of 12.6 months. Of the five patients, three died of metastatic diseases, one died of liver dysfunction and one remains disease free as of 18 months postoperatively. Conclusions:, Our experience indicates that this procedure can be safely used for atrial involvement. Although superior long-term survival cannot be shown yet, favorable early results and a lack of perioperative complications were identified. [source]

Portless endoscopic adrenalectomy via a single minimal incision using a retroperitoneal approach: Experience with initial 30 cases

INTERNATIONAL JOURNAL OF UROLOGY, Issue 9 2004
YUKIO KAGEYAMA
Abstract Aim: To assess the feasibility of portless endoscopic adrenalectomy via a single minimum incision that narrowly permits extraction of the specimen. Methods: For, 30 cases of adrenal tumor, portless endoscopic surgery through a single flank incision (3,9 cm; mean, 5.6 cm) was performed without gas inflation or trocar port placement. All of the instruments used during surgery were reusable. The cases included primary aldosteronism (12), Cushing's syndrome (6), preclinical Cushing's syndrome (3), pheochromocytoma (1), non-functioning cortical adenoma (6), adrenocortical carcinoma (1) and adrenocortical hemorrhage (1). Results: Resection of the tumor was successfully completed, without complications, in all of the cases. Operative time was between 83 and 240 min (mean, 147 min). Estimated blood loss was 5,470 mL (mean, 139 mL). None of the patients required blood transfusion. Postoperative course was uneventful. Wound pain was mild and walking and full oral feeding were resumed on the first and second postoperative day, respectively, in the majority of cases. Conclusions: Adrenal tumors are good candidates for portless endoscopic surgery, which is safe, cost-effective, minimally invasive and matches favorably with laparoscopic surgery. [source]

Osteopontin stimulates invasion of NCI-h295 cells but is not associated with survival in adrenocortical carcinoma,

THE JOURNAL OF PATHOLOGY, Issue 2 2009
Dirk Weismann
Abstract Gene array studies indicated that osteopontin (OPN) mRNA is highly expressed in adrenocortical carcinomas (ACCs). OPN enhances invasiveness, proliferation, and metastasis formation, and is associated with poor survival in some malignant diseases. Integrin ,v,3 has been shown to mediate OPN effects on invasion. In this study, we demonstrated OPN and integrin ,v,3 expression in normal adrenal glands and benign adenomas, with staining seen exclusively in adrenocortical cells as well as even stronger staining in ACC. Western blot analysis confirmed overexpression of OPN in ACC (p < 0.01). With Matrigel invasion assays, we have shown that OPN greatly stimulates the invasiveness of NCI-h295 cells (>six-fold increase, p < 0.001). Transfection with integrin ,v,3 further increased invasiveness after OPN stimulation (p < 0.001). This increase was reversed by the addition of an anti-integrin ,3 antibody, indicating a functional relationship of OPN and integrin ,v,3 in ACC. With tissue arrays, we confirmed high OPN expression in 147 ACC samples. However, no association with survival was seen in Kaplan-Meier analysis including 111 patients with primary tumours graded for OPN staining and follow-up data available. In conclusion, our in vitro data indicate that OPN and integrin ,v,3 may act as a functional complex facilitating the invasiveness of adrenocortical tumours. This relationship remains of relevance to our understanding of carcinogenesis, but further studies are needed to address the physiological and pathological function of OPN in adrenal tissue. Copyright © 2009 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd. [source]

Reoperative adrenal surgery: lessons learnt

ANZ JOURNAL OF SURGERY, Issue 5 2009
Charles T. Tan
With the widespread use of abdominal imaging, the detection and therefore incidence of adrenal tumours is increasing. The laparoscopic approach to primary surgical resection of adrenal tumours has now become the standard of care. There is scarce published literature regarding the management and outcomes of recurrent adrenal tumours. The aim of the present study was therefore to review the authors' experience with reoperative adrenal surgery. A retrospective review of reoperative adrenalectomy cases identified from the prospectively maintained University of Sydney Endocrine Surgical Unit Database from January 1988 to July 2007 was carried out. There were nine (3.5%) reoperative adrenalectomies in six patients. Two were cases of adrenocortical carcinoma, two involved cases of familial phaeochromocytomas and two cases were due to sporadic phaeochromocytomas. Reoperative adrenal surgery is an uncommon event. During the index surgery for adrenal tumours, all adrenal tissue should be removed and knowledge of the vagaries of adrenal anatomy is essential. Reoperative adrenal surgery is a safe procedure and may confer survival benefit or symptom relief. Lifelong follow up is essential for all patients who have had surgery for functional and malignant adrenal tumours. [source]

Nine cases of childhood adrenal tumour presenting with hypertension and a review of the literature

ACTA PAEDIATRICA, Issue 6 2007
Xiumin Wang
Abstract Aim: To describe the clinical features, treatment and outcome of children adrenal tumors presenting with hypertension. Methods: The records of nine children under 16 years of age with adrenal tumours presenting with hypertension were analysed. Details were recorded for family history, clinical presentation, biochemistry, imaging, histology, treatment and outcome. Results: Abdominal mass was palpable only in one patient at diagnosis. Besides hypertension-associated symptoms, Cushing's syndrome was the common presentation form (n = 4). Abdominal computed topography showed adrenal mass in all patients. Tumours were completely resected for each patient. The median tumour weight was 73 g (11,530 g) and the size ranged from 1.5 × 1.5 to 12 × 14 cm2. Pheochromocytoma (n = 2), adrenocortical adenoma (n = 3), adrenocortical carcinoma (n = 1), neuroblastoma (n = 2) and ganglioneuromas (n = 1) were found. In one case, adrenal pheochromocytoma first occurred and non-functioning islet cell tumour successively occurred at pancreas. A better status is common at a median follow-up time of 3.5 years. Conclusions: Childhood adrenal tumours presented with hypertension showed an atypical course, variable presentation. We report a unique case of adrenal pheochromocytoma followed by the occurrence of non-functioning islet cell tumour. Reversal of hypertension by surgery is crucial. Imaging techniques are important to detect adrenal tumours. [source]

What is the best approach to an apparently nonmetastatic adrenocortical carcinoma?

CLINICAL ENDOCRINOLOGY, Issue 5 2010
Martin Fassnacht
Summary In suspected nonmetastatic adrenocortical carcinoma (ACC) a careful preoperative diagnostic work up is needed including comprehensive endocrine analysis as recommended by the European Network for the Study of Adrenal Tumors (http://www.ENSAT.org/ACC.htm). Staging prior surgery, in particular chest CT, is indispensable to exclude distant metastases. Open surgery is still the recommended approach in ACC. However, in localized non-invasive ACC with a diameter <10 cm laparoscopic adrenalectomy by an expert surgeon is probably similarly effective and safe. As many patients will suffer from tumor recurrence after seemingly complete removal of ACC, adjuvant treatment based on the individual risk status is recommended. Key factors for risk assessment are tumor stage, resection status and the proliferation marker Ki67. All patients considered at high risk for recurrence should receive adjuvant mitotane for a minimum of 2 years aiming at a drug level of 14,20 mg/l. In selected patients (e.g. R1 resection) we recommend additional radiotherapy of the tumor bed. Patients with a low/intermediate risk for recurrence should be included in the Adiuvo trial comparing adjuvant mitotane with observation only (http://www.adiuvo-trial.org). In low/intermediate risk patients who cannot be included in this trial observation only can be justified in cases with a tumor diameter of <8 cm and no microscopic evidence for invasion of blood vessels or tumor capsule. In all patients a structured follow-up for 10 years is strongly recommended. [source]