Prognostic factors in stage III–IV adrenocortical carcinomas (ACC): an European Network for the Study of Adrenal Tumor (ENSAT) study

Libé, R.; Borget, Isabelle; Ronchi, Cristina L.; Zaggia, Barbara; Kroiß, Matthias; Kerkhofs, Thomas M.A.; Bertherat, Jérôme; Volante, Marco; Quinkler, Marcus; Chabre, Olivier; Bala, Margarita; Tabarin, Antoine; Beuschlein, Felix; Vezzosi, Delphine; Deutschbein, Timo; Borson‐Chazot, Françoise; Hermsen, I G C; Stell, Anthony; Fottner, Christian; Leboulleux, Sophie; Hahner, Stefanie; Mannelli, Massimo; Berruti, Alfredo; Haak, Harm R.; Terzolo, Massimo; Fassnacht, Martin; Baudin, Éric

doi:10.1093/annonc/mdv329

Cited by 210 publications

(174 citation statements)

References 18 publications

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“…Retrospective data suggest that regional lymph node involvement in ACC has a negative impact on overall survival 67 and is frequently the cause of locoregional recurrence 85 -87 . As reported recently by Fassnacht et al 70 , and independently validated in North America in the Surveillance, Epidemiology, and End Results (SEER) registries 88 , patients with stage III tumours and positive lymph nodes can have a 10-year overall survival rate of up to 40 per cent after resection, although this finding has been challenged recently 89 -91 . It is not yet decided whether a modified ENSAT classification should consider node-positive ACC as stage IV disease 89 .…”

Section: Role Of Regional Lymphadenectomymentioning

confidence: 99%

European Society of Endocrine Surgeons (ESES) and European Network for the Study of Adrenal Tumours (ENSAT) recommendations for the surgical management of adrenocortical carcinoma

Gaujoux

Mihai

Ensat

2017

British Journal of Surgery

173

133

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Background: Radical surgery provides the best chance of cure for adrenocortical carcinoma (ACC), but perioperative surgical care for these patients is yet to be standardized. Methods: A working group appointed jointly by ENSAT and ESES used Delphi methodology to produce evidence-based recommendations for the perioperative surgical care of patients with ACC. Papers were retrieved from electronic databases. Evidence and recommendations were classified according to the Grading of Recommendations, Assessment, Development and Evaluation (GRADE) system, and were discussed until consensus was reached within the group. Results: Twenty-five recommendations for the perioperative surgical care of patients with ACC were formulated. The quality of evidence is low owing to the rarity of the disease and the lack of prospective surgical trials. Multi-institutional prospective cohort studies and prospective RCTs are urgently needed and should be strongly encouraged. Conclusion: The present evidence-based recommendations provide comprehensive advice on the optimal perioperative care for patients undergoing surgery for ACC.

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Section: Role Of Regional Lymphadenectomymentioning

confidence: 99%

European Society of Endocrine Surgeons (ESES) and European Network for the Study of Adrenal Tumours (ENSAT) recommendations for the surgical management of adrenocortical carcinoma

Gaujoux

Mihai

Ensat

2017

British Journal of Surgery

173

133

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“…In adults, several studies have shown that older age is associated with decreased OS (5,6,8,9,26,30,32,39). Asare et al have even proposed a novel staging system, where stage I and stage II ACC were differentiated by age (≤55 years for stage I vs >55 years for stage II) and not by size, like it is done in the ENSAT-staging system (40).…”

Section: Agementioning

confidence: 99%

“…The Ki67 immunohistochemistry represents an alternative technique for assessing proliferation in ACC. Several studies have reported the prognostic value of Ki67 index in ACC (30,34,48,49,50,51,52). A large study in localized ACC identified Ki67 as the most powerful prognostic factor of DFS after complete surgery.…”

Section: Tumor Grade: From Mitoses To Ki67mentioning

confidence: 99%

MANAGEMENT OF ENDOCRINE DISEASE: Adrenocortical carcinoma: differentiating the good from the poor prognosis tumors

Jouinot

Bertherat

2018

European Journal of Endocrinology

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Adrenocortical carcinoma (ACC) is a rare malignancy with a poor prognosis, the five-years overall survival being below 40%. However, there is great variability of outcomes and we have now a better view of the heterogeneity of tumor aggressiveness. The extent of the disease at the time of diagnosis, best assayed by the European Network for the Study of Adrenal Tumors (ENSAT) Staging Score, is a major determinant of survival. The tumor grade, including the mitotic count and the Ki67 proliferation index, also appears as a strong prognostic factor. The assessment of tumor grade, even by expert pathologists, still suffers from inter-observer reproducibility. The emergence of genomics in the last decade has revolutionized the knowledge of molecular biology and genetics of cancers. In ACC, genomic approaches - including pan-genomic studies of gene expression (transcriptome), recurrent mutations (exome or whole-genome sequencing), chromosome alterations, DNA methylation (methylome), miRNA expression (miRnome) - converge in a new classification of ACC, characterized by distinct molecular profiles and very different outcomes. Targeted measurements of a few discriminant molecular alterations have been developed in the perspective of clinical routine, and thus, may help defining therapeutic strategy. By individualizing patients' prognosis and tumor biology, these recent progresses appear as an important step forward towards precision medicine.

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“…Of 1523 eligible publications, nineteen (eighteen cohort studies, one RCT (1,4,7,17,18,19,20,21,22,23,24,25,26,27,28,29,30, 31, 32)) were included, representing a total of 3814 patients ( Fig. 1 for flow-chart of study inclusion).…”

Section: Study Inclusion and Study Characteristicsmentioning

confidence: 99%

“…Adrenocortical carcinoma (ACC) is a rare tumour with an incidence of less than 2 per million population. The prognosis remains poor with a 5-year overall survival of less than 50% and in metastatic disease less than 20% (1,2,3,4,5). Recurrences are frequent, even after apparent complete surgical resection.…”

Section: Introductionmentioning

confidence: 99%

Influence of hormonal functional status on survival in adrenocortical carcinoma: systematic review and meta-analysis

Vanbrabant

Fassnacht

Assié

et al. 2018

European Journal of Endocrinology

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Objective: Adrenocortical carcinoma (ACC) is a malignancy with a poor prognosis. Many publications in ACC report on risk factors for a poor outcome; one risk factor studied is hormonal hypersecretion (cortisol, sex-hormones, steroid precursors or aldosterone). The aim of this systematic review was to study the association between hormonal secretion and recurrence or mortality in ACC. Design: Systematic review and meta-analysis. We searched PubMed, EMBASE and The Cochrane library (January 2018) for cohort studies examining the association between hormonal secretion on overall or recurrence-free survival in ACC. Methods: A random-effects model meta-analysis was performed to obtain a weighted relative risk comparing cortisolsecreting and/or androgen-secreting ACCs to non-secreting tumours regarding overall and recurrence-free survival. Risk of bias assessment was performed for all studies included. Results: Nineteen publications were included representing a total of 3814 patients. Most studies were generally considered low/intermediate risk of bias. Meta-analysis showed higher mortality risk for cortisol-secreting ACCs, weighted relative risk 1.71 (95% CI: 1.18-2.47) combining studies that adjusted for tumour stage; also a higher recurrence risk was found for cortisol producing ACCs, relative risk 1.43 (95% CI: 1.18-1.73). Androgen secretion was not clearly associated with survival (RR: 0.82, 95% CI: 0.60-1.12). Conclusion: This systematic review and meta-analysis show that cortisol-secreting ACCs are associated with a worse overall survival; future research is needed to establish whether this association points to negative effects of cortisol action, whether it signifies a more aggressive ACC subtype or whether cortisol is merely a prognostic marker.

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Prognostic factors in stage III–IV adrenocortical carcinomas (ACC): an European Network for the Study of Adrenal Tumor (ENSAT) study

Abstract: The mENSAT classification and GRAS parameters (Grade, R status, Age and Symptoms) were found to best stratify the prognosis of patients with advanced ACC.

Cited by 210 publications

References 18 publications

European Society of Endocrine Surgeons (ESES) and European Network for the Study of Adrenal Tumours (ENSAT) recommendations for the surgical management of adrenocortical carcinoma

European Society of Endocrine Surgeons (ESES) and European Network for the Study of Adrenal Tumours (ENSAT) recommendations for the surgical management of adrenocortical carcinoma

MANAGEMENT OF ENDOCRINE DISEASE: Adrenocortical carcinoma: differentiating the good from the poor prognosis tumors

Influence of hormonal functional status on survival in adrenocortical carcinoma: systematic review and meta-analysis

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