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

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Title: Current clinical management and outcome of patients with adrenal cortical carcinoma (ACC) with rare histological subtypes-an ENSAT cohort study.
Authors: Kimpel O; Division of Endocrinology and Diabetes, Department of Medicine, University Hospital, University of Würzburg, Würzburg, Germany. Electronic address: Kimpel_o@ukw.de., Cosentini D; Department of Medical and Surgical Specialties, Radiological Sciences and Public Health, University of Brescia, ASST-Spedali Civili, Brescia, Italy., Calabrese A; Internal Medicine, San Luigi Gonzaga Hospital, Department of Clinical and Biological Sciences, University of Turin, Turin, Italy., Jouinot A; Université Paris Cité, CNRS, Inserm, Institut Cochin, Paris, France; Department of Endocrinology and National Reference Center for Rare Adrenal Disorders, AP-HP, Hôpital Cochin, Paris, France., Altieri B; Division of Endocrinology and Diabetes, Department of Medicine, University Hospital, University of Würzburg, Würzburg, Germany; Bavarian Cancer Research Center (BZKF), University Hospital, University of Würzburg, Würzburg, Germany., Kircher S; Institute of Pathology, University of Würzburg, Würzburg, Germany., Di Dalmazi G; 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., Elhassan YS; 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., Vassiliadi D; Department of Endocrinology, Diabetes Centre and European Reference Network on Rare Endocrine Conditions (Endo-ERN), Evangelismos Hospital, Athens, Greece., Chrysoula K; Department of Endocrinology, Diabetes Centre and European Reference Network on Rare Endocrine Conditions (Endo-ERN), Evangelismos Hospital, Athens, Greece., Puglisi S; Internal Medicine, San Luigi Gonzaga Hospital, Department of Clinical and Biological Sciences, University of Turin, Turin, Italy., Coscia K; 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., Gallego NV; Department of Internal Medicine, Section of Endocrinology and Nutrition, Hospital Universitario de Cabueñes, Gijón, Spain., Loli P; Endocrinology, Clinica Polispecialistica San Carlo, Milan, Italy., Boudina M; Department of Endocrinology, Theagenio Cancer Hospital, Thessaloniki, Greece., Lagana M; Department of Medical and Surgical Specialties, Radiological Sciences and Public Health, University of Brescia, ASST-Spedali Civili, Brescia, Italy., Cremaschi V; Department of Medical and Surgical Specialties, Radiological Sciences and Public Health, University of Brescia, ASST-Spedali Civili, Brescia, Italy., Pignatelli D; Hospital S Joao and Faculty of Medicine, University of Porto, Porto, Portugal., Rios E; Department of Pathology, ULS São João, Porto, Portugal; Department of Oncology and Pathology, Faculty of Medicine, University of Porto, Porto, Portugal., Villavicencio CA; Division of Endocrinology, Diabetes and Metabolism, Mayo Clinic, Rochester, USA., McKenzie TJ; Division of Endocrinology, Diabetes and Metabolism, Mayo Clinic, Rochester, USA., Rayes N; Department of Visceral, Transplant, Thoracic and Vascular Surgery, University Hospital of Leipzig, Leipzig, Germany., Ronchi CL; 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., Bancos I; Division of Endocrinology, Diabetes and Metabolism, Mayo Clinic, Rochester, USA., Terzolo M; Internal Medicine, San Luigi Gonzaga Hospital, Department of Clinical and Biological Sciences, University of Turin, Turin, Italy., Berruti A; Department of Medical and Surgical Specialties, Radiological Sciences and Public Health, University of Brescia, ASST-Spedali Civili, Brescia, Italy., Assié G; Université Paris Cité, CNRS, Inserm, Institut Cochin, Paris, France; Department of Endocrinology and National Reference Center for Rare Adrenal Disorders, AP-HP, Hôpital Cochin, Paris, France., Kroiss M; Division of Endocrinology and Diabetes, Department of Medicine, University Hospital, University of Würzburg, Würzburg, Germany; Department of Medicine IV, LMU University Hospital, LMU Munich, Munich, Germany., Fassnacht M; Division of Endocrinology and Diabetes, Department of Medicine, University Hospital, University of Würzburg, Würzburg, Germany; Bavarian Cancer Research Center (BZKF), University Hospital, University of Würzburg, Würzburg, Germany; Comprehensive Cancer Center Mainfranken and NCT WERA, University of Würzburg, Würzburg, Germany.
Source: ESMO open [ESMO Open] 2026 Mar; Vol. 11 (3), pp. 106092. Date of Electronic Publication: 2026 Mar 10.
Publication Type: Journal Article; Multicenter Study
Journal Info: Publisher: Elsevier Country of Publication: England NLM ID: 101690685 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 2059-7029 (Electronic) Linking ISSN: 20597029 NLM ISO Abbreviation: ESMO Open Subsets: MEDLINE
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  Data: Current clinical management and outcome of patients with adrenal cortical carcinoma (ACC) with rare histological subtypes-an ENSAT cohort study.
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  Data: <searchLink fieldCode="AU" term="%22Kimpel+O%22">Kimpel O</searchLink>; Division of Endocrinology and Diabetes, Department of Medicine, University Hospital, University of Würzburg, Würzburg, Germany. Electronic address: Kimpel_o@ukw.de.<br /><searchLink fieldCode="AU" term="%22Cosentini+D%22">Cosentini D</searchLink>; Department of Medical and Surgical Specialties, Radiological Sciences and Public Health, University of Brescia, ASST-Spedali Civili, Brescia, Italy.<br /><searchLink fieldCode="AU" term="%22Calabrese+A%22">Calabrese A</searchLink>; Internal Medicine, San Luigi Gonzaga Hospital, Department of Clinical and Biological Sciences, University of Turin, Turin, Italy.<br /><searchLink fieldCode="AU" term="%22Jouinot+A%22">Jouinot A</searchLink>; Université Paris Cité, CNRS, Inserm, Institut Cochin, Paris, France; Department of Endocrinology and National Reference Center for Rare Adrenal Disorders, AP-HP, Hôpital Cochin, Paris, France.<br /><searchLink fieldCode="AU" term="%22Altieri+B%22">Altieri B</searchLink>; Division of Endocrinology and Diabetes, Department of Medicine, University Hospital, University of Würzburg, Würzburg, Germany; Bavarian Cancer Research Center (BZKF), University Hospital, University of Würzburg, Würzburg, Germany.<br /><searchLink fieldCode="AU" term="%22Kircher+S%22">Kircher S</searchLink>; Institute of Pathology, University of Würzburg, Würzburg, Germany.<br /><searchLink fieldCode="AU" term="%22Di+Dalmazi+G%22">Di Dalmazi G</searchLink>; 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.<br /><searchLink fieldCode="AU" term="%22Elhassan+YS%22">Elhassan YS</searchLink>; 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.<br /><searchLink fieldCode="AU" term="%22Vassiliadi+D%22">Vassiliadi D</searchLink>; Department of Endocrinology, Diabetes Centre and European Reference Network on Rare Endocrine Conditions (Endo-ERN), Evangelismos Hospital, Athens, Greece.<br /><searchLink fieldCode="AU" term="%22Chrysoula+K%22">Chrysoula K</searchLink>; Department of Endocrinology, Diabetes Centre and European Reference Network on Rare Endocrine Conditions (Endo-ERN), Evangelismos Hospital, Athens, Greece.<br /><searchLink fieldCode="AU" term="%22Puglisi+S%22">Puglisi S</searchLink>; Internal Medicine, San Luigi Gonzaga Hospital, Department of Clinical and Biological Sciences, University of Turin, Turin, Italy.<br /><searchLink fieldCode="AU" term="%22Coscia+K%22">Coscia K</searchLink>; 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.<br /><searchLink fieldCode="AU" term="%22Gallego+NV%22">Gallego NV</searchLink>; Department of Internal Medicine, Section of Endocrinology and Nutrition, Hospital Universitario de Cabueñes, Gijón, Spain.<br /><searchLink fieldCode="AU" term="%22Loli+P%22">Loli P</searchLink>; Endocrinology, Clinica Polispecialistica San Carlo, Milan, Italy.<br /><searchLink fieldCode="AU" term="%22Boudina+M%22">Boudina M</searchLink>; Department of Endocrinology, Theagenio Cancer Hospital, Thessaloniki, Greece.<br /><searchLink fieldCode="AU" term="%22Lagana+M%22">Lagana M</searchLink>; Department of Medical and Surgical Specialties, Radiological Sciences and Public Health, University of Brescia, ASST-Spedali Civili, Brescia, Italy.<br /><searchLink fieldCode="AU" term="%22Cremaschi+V%22">Cremaschi V</searchLink>; Department of Medical and Surgical Specialties, Radiological Sciences and Public Health, University of Brescia, ASST-Spedali Civili, Brescia, Italy.<br /><searchLink fieldCode="AU" term="%22Pignatelli+D%22">Pignatelli D</searchLink>; Hospital S Joao and Faculty of Medicine, University of Porto, Porto, Portugal.<br /><searchLink fieldCode="AU" term="%22Rios+E%22">Rios E</searchLink>; Department of Pathology, ULS São João, Porto, Portugal; Department of Oncology and Pathology, Faculty of Medicine, University of Porto, Porto, Portugal.<br /><searchLink fieldCode="AU" term="%22Villavicencio+CA%22">Villavicencio CA</searchLink>; Division of Endocrinology, Diabetes and Metabolism, Mayo Clinic, Rochester, USA.<br /><searchLink fieldCode="AU" term="%22McKenzie+TJ%22">McKenzie TJ</searchLink>; Division of Endocrinology, Diabetes and Metabolism, Mayo Clinic, Rochester, USA.<br /><searchLink fieldCode="AU" term="%22Rayes+N%22">Rayes N</searchLink>; Department of Visceral, Transplant, Thoracic and Vascular Surgery, University Hospital of Leipzig, Leipzig, Germany.<br /><searchLink fieldCode="AU" term="%22Ronchi+CL%22">Ronchi CL</searchLink>; 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.<br /><searchLink fieldCode="AU" term="%22Bancos+I%22">Bancos I</searchLink>; Division of Endocrinology, Diabetes and Metabolism, Mayo Clinic, Rochester, USA.<br /><searchLink fieldCode="AU" term="%22Terzolo+M%22">Terzolo M</searchLink>; Internal Medicine, San Luigi Gonzaga Hospital, Department of Clinical and Biological Sciences, University of Turin, Turin, Italy.<br /><searchLink fieldCode="AU" term="%22Berruti+A%22">Berruti A</searchLink>; Department of Medical and Surgical Specialties, Radiological Sciences and Public Health, University of Brescia, ASST-Spedali Civili, Brescia, Italy.<br /><searchLink fieldCode="AU" term="%22Assié+G%22">Assié G</searchLink>; Université Paris Cité, CNRS, Inserm, Institut Cochin, Paris, France; Department of Endocrinology and National Reference Center for Rare Adrenal Disorders, AP-HP, Hôpital Cochin, Paris, France.<br /><searchLink fieldCode="AU" term="%22Kroiss+M%22">Kroiss M</searchLink>; Division of Endocrinology and Diabetes, Department of Medicine, University Hospital, University of Würzburg, Würzburg, Germany; Department of Medicine IV, LMU University Hospital, LMU Munich, Munich, Germany.<br /><searchLink fieldCode="AU" term="%22Fassnacht+M%22">Fassnacht M</searchLink>; Division of Endocrinology and Diabetes, Department of Medicine, University Hospital, University of Würzburg, Würzburg, Germany; Bavarian Cancer Research Center (BZKF), University Hospital, University of Würzburg, Würzburg, Germany; Comprehensive Cancer Center Mainfranken and NCT WERA, University of Würzburg, Würzburg, Germany.
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