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Publications

Journal Article Ahead of print

PubMed: 41812315 DOI: 10.1016/j.esmoop.2026.106092

Current clinical management and outcome of patients with adrenal cortical carcinoma (ACC) with rare histological subtypes-an ENSAT cohort study.

ESMO Open 11(3):106092

Authors: O Kimpel, D Cosentini, A Calabrese, A Jouinot, B Altieri, S Kircher, G Di Dalmazi, Y S Elhassan, D Vassiliadi, K Chrysoula, S Puglisi, K Coscia, N V Gallego, P Loli, M Boudina, M Lagana, V Cremaschi, D Pignatelli, E Rios, C A Villavicencio, T J McKenzie, N Rayes, C L Ronchi, I Bancos, M Terzolo, A Berruti, G AssiƩ, M Kroiss, M Fassnacht

Affiliations

  • Division of Endocrinology and Diabetes, Department of Medicine, University Hospital, University of WĆ¼rzburg, WĆ¼rzburg, Germany.
  • Department of Medical and Surgical Specialties, Radiological Sciences and Public Health, University of Brescia, ASST-Spedali Civili, Brescia, Italy.
  • Internal Medicine, San Luigi Gonzaga Hospital, Department of Clinical and Biological Sciences, University of Turin, Turin, Italy.
  • UniversitĆ© Paris CitĆ©, CNRS, Inserm, Institut Cochin, Paris, France; Department of Endocrinology and National Reference Center for Rare Adrenal Disorders, AP-HP, HĆ“pital Cochin, Paris, France.
  • Division of Endocrinology and Diabetes, Department of Medicine, University Hospital, University of WĆ¼rzburg, WĆ¼rzburg, Germany; Bavarian Cancer Research Center (BZKF), University Hospital, University of WĆ¼rzburg, WĆ¼rzburg, Germany.
  • Institute of Pathology, University of WĆ¼rzburg, WĆ¼rzburg, Germany.
  • Division of Endocrinology and Diabetes Prevention and Care, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy; Department of Medical and Surgical Sciences (DIMEC), Alma Mater Studiorum University of Bologna, Bologna, Italy.
  • Department of Metabolism and Systems Science, College of Medicine and Health, University of Birmingham, Birmingham, UK; Department of Endocrinology, Queen Elizabeth Hospital, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK.
  • Department of Endocrinology, Diabetes Centre and European Reference Network on Rare Endocrine Conditions (Endo-ERN), Evangelismos Hospital, Athens, Greece.
  • Department of Internal Medicine, Section of Endocrinology and Nutrition, Hospital Universitario de CabueƱes, GijĆ³n, Spain.
  • Endocrinology, Clinica Polispecialistica San Carlo, Milan, Italy.
  • Department of Endocrinology, Theagenio Cancer Hospital, Thessaloniki, Greece.
  • Hospital S Joao and Faculty of Medicine, University of Porto, Porto, Portugal.
  • Department of Pathology, ULS SĆ£o JoĆ£o, Porto, Portugal; Department of Oncology and Pathology, Faculty of Medicine, University of Porto, Porto, Portugal.
  • Division of Endocrinology, Diabetes and Metabolism, Mayo Clinic, Rochester, USA.
  • Department of Visceral, Transplant, Thoracic and Vascular Surgery, University Hospital of Leipzig, Leipzig, Germany.
  • Division of Endocrinology and Diabetes, Department of Medicine, University Hospital, University of WĆ¼rzburg, WĆ¼rzburg, Germany; Department of Medicine IV, LMU University Hospital, LMU Munich, Munich, Germany.
  • Division of Endocrinology and Diabetes, Department of Medicine, University Hospital, University of WĆ¼rzburg, WĆ¼rzburg, Germany; Bavarian Cancer Research Center (BZKF), University Hospital, University of WĆ¼rzburg, WĆ¼rzburg, Germany; Comprehensive Cancer Center Mainfranken and NCT WERA, University of WĆ¼rzburg, WĆ¼rzburg, Germany.

Abstract

BACKGROUND

In 2022, the World Health Organization (WHO) classified adrenal cortical carcinoma (ACC) into four subtypes: conventional, oncocytic, myxoid, and sarcomatoid. The differences in prognosis among these subtypes remain unclear. This European Network for the Study of Adrenal Tumours (ENSAT) multicentre study examines the differences not only in outcomes but also in the clinical management of patients with different ACC subtypes.

PATIENTS AND METHODS

Patient characteristics and survival data were retrospectively collected and analysed using Kaplan-Meier, multivariate Cox regression, and matched propensity score analyses (1 : 3 ratio) to compare recurrence-free survival (RFS) and overall survival (OS) for each subtype against conventional ACC. RFS was investigated only in patients after R0 resection, whereas OS was evaluated in the whole cohort. Matching was done for sex, age, and ENSAT tumour stage.

RESULTS

The study included 1098 conventional, 179 oncocytic, 28 myxoid, and 10 sarcomatoid ACCs. RFS (in 653 conventional, 127 oncocytic, 15 myxoid, and 6 sarcomatoid ACCs) and OS (in all patients) were significantly longer in oncocytic ACC compared with the other groups, which was confirmed by multivariate Cox regression analyses {hazard ratio (HR) for RFS using conventional ACC as reference: oncocytic 0.47 [95% confidence interval (CI) 0.34-0.66], myxoid 0.92 (0.49-1.71), and sarcomatoid 0.83 (0.13-2.13) and for OS: oncocytic 0.37 (0.22-0.61), myxoid 1.02 (0.50-2.1), and sarcomatoid 0.82 (0.11-5.94)}. Similar results were seen after propensity score matching. RFS and OS were significantly longer in oncocytic ACC compared with the matched conventional ACC (RFS HR 0.41, 95% CI 0.29-0.55, P < 0.001; OS HR 0.35, 95% CI 0.24-0.52, P < 0.001).In metastatic oncocytic ACC, mitotane was similarly effective as in conventional tumours, but platinum-based chemotherapy seemed to result in longer progression-free survival.

CONCLUSION

Stage-adjusted outcome in oncocytic ACC is better than in other subtypes.

MeSH

Neoplasms, Cohort Studies, Propensity Score, Adrenocortical Carcinoma, Prognosis, Adrenal Gland Neoplasms