Plasma Concentration of o,p'DDD (Mitotane), o,p'DDA and o,p'DDE as Predictors of Tumor Response in Adrenocortical Carcinoma: Results of a Retrospective European Network for the Study of Adrenal Tumors (ENS@T) Multicentre Study.

2010 ◽  
pp. P3-77-P3-77
Author(s):  
IGC Hermsen ◽  
M Fassnacht ◽  
M Terzolo ◽  
S Houterman ◽  
J den Hartigh ◽  
...  
Biomedicines ◽  
2021 ◽  
Vol 9 (8) ◽  
pp. 909
Author(s):  
Giuseppe Cavallaro ◽  
Mariarita Tarallo ◽  
Ambra Chiappini ◽  
Daniele Crocetti ◽  
Andrea Polistena ◽  
...  

Introduction: Adrenocortical carcinoma (ACC) is a rare tumor, often discovered at an advanced stage and associated with poor prognosis. Treatment is guided by staging according to the European Network for the Study of Adrenal Tumors (ENSAT) classification. Surgery is the treatment of choice for ACC. The aim of this review is to provide a complete overview on surgical approaches and management of adrenocortical carcinoma. Methods: This comprehensive review has been carried out according to the PRISMA statement. The literature sources were the databases PubMed, Scopus and Cochrane Library. The search thread was: ((surgery) OR (adrenalectomy)) AND (adrenocortical carcinoma). Results: Among all studies identified, 17 were selected for the review. All of them were retrospective. A total of 2498 patients were included in the studies, of whom 734 were treated by mini-invasive approaches and 1764 patients were treated by open surgery. Conclusions: Surgery is the treatment of choice for ACC. Open adrenalectomy (OA) is defined as the gold standard. In recent years laparoscopic adrenalectomy (LA) has gained more popularity. No significant differences were reported for overall recurrence rate, time to recurrence, and cancer-specific mortality between LA and OA, in particular for Stage I-II. Robotic adrenalectomy (RA) has several advantages compared to LA, but there is still a lack of specific documentation on RA use in ACC.


2022 ◽  
Vol 12 (1) ◽  
pp. 100
Author(s):  
Anja Barac Nekic ◽  
Nikola Knezevic ◽  
Karin Zibar Tomsic ◽  
Ivana Kraljevic ◽  
Annemarie Balasko ◽  
...  

Complete surgical removal of adrenocortical carcinoma (ACC) represents the only chance of long-term cure. In this study, we compared the long-term outcomes of ACC patients depending on whether they had adrenal surgery performed in a high-volume (HVC) or in a low-volume (LVC) center. This retrospective study included 49 patients from the Croatian ACC Registry with the European Network for the Study of Adrenal Tumors (ENSAT) stage I–III ACC, of which 35 underwent surgery in a HVC whereas 14 of them were operated in one of the LVCs. Patients operated in the LVCs had a significantly higher rate of ACC recurrence (57.1% vs. 22.9%; p = 0.02). Accordingly, RFS was significantly longer in patients operated on in HVC (p = 0.04). The difference in RFS remained significant after controlling for age, gender, tumor size, Ki-67 index, Weiss score, and type of surgery (HR 4.55; 95% CI 1.16–17.88; p = 0.03). In addition, there is a tendency towards longer DSS in patients in the HVC group compared to those in the LVC group (p = 0.05). These results point to the centralization of adrenal surgery as a key prerequisite for improving the outcomes of ACC patients.


2020 ◽  
Vol 11 (4) ◽  
pp. 705-710
Author(s):  
Martin K. Walz ◽  
Klaus A. Metz ◽  
Sarah Theurer ◽  
Cathrin Myland ◽  
Pier F. Alesina ◽  
...  

AbstractThe morphological differentiation between benign and malignant adrenocortical tumors is an ongoing problem in diagnostic pathology. In recent decades the complex scoring systems have been widely used to calculate the probability of malignancy in adrenocortical tumors on the basis of a variety of histomorphological parameters. We herewith present a substantially simplified method to diagnose adrenocortical carcinoma by a single histomorphological parameter on a consecutive series of more than 800 adrenocortical tumors. Between January 2000 and May 2019, altogether 2305 adrenalectomies for of all types of diseases were removed, approximately 98% by minimally invasive approaches. After exclusion of pheochromocytomas, adrenal ganglioneuromas, adrenal metastases, Cushing’s disease related specimens, and Conn’s adenomas, the present series finally consisted of 837 adrenocortical tumors. All tumors were analyzed by experienced pathologists of a single institution using standard histopathological methods (Hematoxylin-Eosin and Ki67 stained sections). Clinical and histopathologic data were prospectively collected and retrospectively analyzed. Clinically, 385 patients had 420 functioning tumors (FT), and 417 had non-functioning adrenal tumors (NFT). The mean size of FT was 3.8 ± 1.4 cm (range 0.5–16 cm) and for NFT 4.5 ± 1.6 cm (range 1.5–18 cm). Histomorphologically, 32 adrenal tumors were classified as adrenocortical carcinoma (ACC; 3.8%). In all 32 cases (tumor size 9.1 ± 4.0 cm, range 3–18 cm), confluenting tumor necrosis could be demonstrated. The remaining 805 tumors (control group) completely lacked this highly reproducible single morphological feature. Ki67 levels above 10% were found in 31 of 32 ACCs and never in adrenocortical adenomas (ACA). With a mean follow-up of 8.2 years, 24 out of 32 patients primarily diagnosed as ACC developed distant metastases (75.0%), whereas all patients in the control group remained free of local or distant recurrence. We conclude that a single morphological parameter (confluenting tumor necrosis) is sufficient to predict a poor clinical course in adrenocortical tumors. The histomorphological diagnosis of this parameter is straightforward and highly reproducible.


Cancer ◽  
2003 ◽  
Vol 97 (3) ◽  
pp. 554-560 ◽  
Author(s):  
Bradford J. Wood ◽  
Jame Abraham ◽  
Julia L. Hvizda ◽  
H. Richard Alexander ◽  
Tito Fojo

2021 ◽  
Vol Publish Ahead of Print ◽  
Author(s):  
Sebastian Zimmermann ◽  
Max Kurlbaum ◽  
Stefanie Mayer ◽  
Martin Fassnacht ◽  
Matthias Kroiss ◽  
...  

2019 ◽  
Vol 104 (6) ◽  
pp. 2367-2374 ◽  
Author(s):  
Segolene Hescot ◽  
Maria Curras-Freixes ◽  
Timo Deutschbein ◽  
Anouk van Berkel ◽  
Delphine Vezzosi ◽  
...  

2004 ◽  
Vol 180 (1) ◽  
pp. 125-133 ◽  
Author(s):  
M Watanabe ◽  
S Nakajin

A number of conditions related to sex-reversal in boys and men and precocious puberty in girls are caused by estrogen-secreting adrenal tumors. In these tumors, cytochrome P450 aromatase (aromatase) that is encoded in the CYP19 gene is expressed at high levels. To investigate the molecular mechanism of aromatase expression in these adrenal tumors, we characterized the activity, gene transcript and genomic promoter region of aromatase in the human adrenocortical carcinoma cell line H295R. Aromatase activity and the transcript of the CYP19 gene were highly up-regulated by forskolin, but not by dexamethasone. The results from exon I-specific reverse transcriptase (RT)-PCR and the transfection of reporter constructs suggested that promoter I.3 and promoter II were activated in H295R. Deletion and mutation analysis suggested that cAMP response element-like sequence (CLS) and steroidogenic factor-1 (SF-1) motif, were critical for the activation of promoter II. The results of this work should provide the basis for the molecular analysis of aromatase expression in adrenocortical cells.


2013 ◽  
Vol 169 (1) ◽  
pp. 83-89 ◽  
Author(s):  
T M A Kerkhofs ◽  
R H A Verhoeven ◽  
H J Bonjer ◽  
E J Nieveen van Dijkum ◽  
M R Vriens ◽  
...  

ObjectiveAdrenocortical carcinoma (ACC) is a rare disease with an estimated incidence of one to two cases per 1 million inhabitants. The Dutch Adrenal Network (DAN) was initiated with the aim to improve patient care and to stimulate scientific research on ACC. Currently, not all patients with ACC are treated in specialized DAN hospitals. The objective of the current investigation was to determine whether there are differences in survival between patients operated on in DAN hospitals and those operated on in non-DAN hospitals.DesignThe study was set up as a retrospective and population-based survival analysis.MethodsData on all adult ACC patients diagnosed between 1999 and 2009 were obtained from The Netherlands Cancer Registry (NCR). Overall survival was calculated and a comparison was made between DAN and non-DAN hospitals.ResultsThe NCR contained data of 189 patients. The median survival of patients with European Network for the Study of Adrenal Tumors stages I–III disease was significantly longer for patients operated on in a DAN hospital (n=46) than for those operated on in a non-DAN hospital (n=37, 5-year survival 63 vs 42%). Survival remained significantly different after correction for sex, age, year of diagnosis, and stage of disease in the multivariate analysis (hazard ratio 1.96 (95% CI 1.01–3.81), P=0.047).ConclusionThe results associate surgery in a DAN center with a survival benefit for patients with local or locally advanced ACC. We hypothesize that a multidisciplinary approach for these patients explains the observed survival benefit. These findings should be carefully considered in view of the aim for further centralization of ACC treatment.


2002 ◽  
Vol 13 (2) ◽  
pp. 099-110 ◽  
Author(s):  
Amir Khorram-Manesh ◽  
Håkan Ahlman ◽  
Svante Jansson ◽  
Ola Nilsson

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