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  1. Article ; Online: Adrenocortical carcinoma: Diagnosis, prognostic classification and treatment of localized and advanced disease.

    Libé, Rossella / Huillard, Olivier

    Cancer treatment and research communications

    2023  Volume 37, Page(s) 100759

    Abstract: Adrenocortical carcinoma (ACC) is a rare cancer with an estimated incidence of 0.7 to 2.0 cases per 1 million population per year in the United States. It is an aggressive cancer originating in the cortex of the adrenal gland with a poor prognosis. The 5- ...

    Abstract Adrenocortical carcinoma (ACC) is a rare cancer with an estimated incidence of 0.7 to 2.0 cases per 1 million population per year in the United States. It is an aggressive cancer originating in the cortex of the adrenal gland with a poor prognosis. The 5-year survival rate is less than 15% among patients with metastatic disease. In this article, we review the epidemiology and pathogenesis of ACC, the diagnostic procedures, the prognostic classification of ACC, and the treatment options from localized and resectable forms to advanced disease detailing recent therapeutic developments such as immunotherapy and molecularly targeted therapy.
    MeSH term(s) Humans ; Adrenocortical Carcinoma/diagnosis ; Adrenocortical Carcinoma/therapy ; Prognosis ; Adrenal Cortex Neoplasms/therapy ; Adrenal Cortex Neoplasms/drug therapy ; Immunotherapy ; Antineoplastic Combined Chemotherapy Protocols/therapeutic use
    Language English
    Publishing date 2023-09-08
    Publishing country England
    Document type Review ; Journal Article
    ISSN 2468-2942
    ISSN (online) 2468-2942
    DOI 10.1016/j.ctarc.2023.100759
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  2. Article ; Online: Artistic movements as pitfalls in iconodiagnosis.

    Lefrère, Bertrand / Libé, Rossella / Groussin, Lionel

    Annales d'endocrinologie

    2023  Volume 85, Issue 2, Page(s) 163–165

    MeSH term(s) Humans ; Art
    Language English
    Publishing date 2023-12-13
    Publishing country France
    Document type Letter
    ZDB-ID 299-9
    ISSN 2213-3941 ; 0003-4266
    ISSN (online) 2213-3941
    ISSN 0003-4266
    DOI 10.1016/j.ando.2023.12.002
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  3. Article ; Online: Thyroid swellings in Renaissance illuminations.

    Lefrère, Bertrand / Libé, Rossella / Groussin, Lionel

    Annales d'endocrinologie

    2023  Volume 84, Issue 2, Page(s) 322–324

    MeSH term(s) Humans ; Lighting ; Goiter ; Thyroid Neoplasms
    Language English
    Publishing date 2023-01-12
    Publishing country France
    Document type Letter
    ZDB-ID 299-9
    ISSN 2213-3941 ; 0003-4266
    ISSN (online) 2213-3941
    ISSN 0003-4266
    DOI 10.1016/j.ando.2023.01.001
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  4. Article ; Online: Clinical and molecular prognostic factors in adrenocortical carcinoma.

    Libé, Rossella

    Minerva endocrinologica

    2018  Volume 44, Issue 1, Page(s) 58–69

    Abstract: Introduction: Adrenocortical carcinoma (ACC) is a rare cancer, with an incidence less than 0.7-1.5 per 1 million people per year, with a poor prognosis. The overall survival (OS) depends on the ENSAT stage: in particular in metastatic ACC the OS varies ... ...

    Abstract Introduction: Adrenocortical carcinoma (ACC) is a rare cancer, with an incidence less than 0.7-1.5 per 1 million people per year, with a poor prognosis. The overall survival (OS) depends on the ENSAT stage: in particular in metastatic ACC the OS varies from 10 to 20 months, with a 5-year survival around 10%. ACC has a different behavior, probably due to a different biology. For this reason, a careful prognostic classification is mandatory, in order to stratify the patients and propose a specific management.
    Evidence acquisition: Prognostic factors can be divides in three groups: clinical factors (tumor stage, age, hormone-related symptoms), pathological factors (Weiss Score, mitotic count, Ki-67, SF-1 and AVA2, P53, beta-catenin immunohistochemistry, resection status), molecular factors (chromosomal aberrations, methylation profile, altered gene expression and miRNA expression, gene mutations).
    Evidence synthesis: The best way to stratify ACC patients and propose the best therapeutic option is to combine clinical, pathological and molecular factors.
    Conclusions: Individualizing patients' prognosis and tumor biology appears as a necessary step for personalized medicine. In addition to tumor stage and tumor grade, the genomic classification may precise the risk stratification and thus help defining therapeutic strategy.
    MeSH term(s) Adrenal Cortex Neoplasms/diagnosis ; Adrenal Cortex Neoplasms/therapy ; Adrenocortical Carcinoma/diagnosis ; Adrenocortical Carcinoma/therapy ; Humans ; Molecular Diagnostic Techniques ; Prognosis
    Language English
    Publishing date 2018-09-12
    Publishing country Italy
    Document type Journal Article ; Review
    ZDB-ID 423687-7
    ISSN 1827-1634 ; 0391-1977
    ISSN (online) 1827-1634
    ISSN 0391-1977
    DOI 10.23736/S0391-1977.18.02900-0
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  5. Article: Adrenocortical carcinoma (ACC): diagnosis, prognosis, and treatment.

    Libé, Rossella

    Frontiers in cell and developmental biology

    2015  Volume 3, Page(s) 45

    Abstract: Adrenocortical carticnoma (ACC) is a rare malignancy with an incidence of 0.7-2.0 cases/million habitants/year. The diagnosis of malignancy relies on careful investigations of clinical, biological, and imaging features before surgery and pathological ... ...

    Abstract Adrenocortical carticnoma (ACC) is a rare malignancy with an incidence of 0.7-2.0 cases/million habitants/year. The diagnosis of malignancy relies on careful investigations of clinical, biological, and imaging features before surgery and pathological examination after tumor removal. Most patients present with steroid hormone excess or abdominal mass effects, but 15% of patients with ACC is initially diagnosed incidentally. After the diagnosis, in order to assess the ACC prognosis and establish an adequate basis for treatment decisions different tools are proposed. The stage classification proposed by the European Network for the Study of Adrenal Tumors (ENSAT) is recommended. Pathology reports define the Weiss score, the resection status and the proliferative index, including the mitotic count and the Ki67 index. As far as the treatment is concerned, in case of tumor limited to the adrenal gland, the complete resection of the tumor is the first option. Most patients benefit from adjuvant mitotane treatment. In metastatic disease, mitotane is the cornerstone of initial treatment, and cytotoxic drugs should be added in case of progression. Recently, the First International Randomized (FIRM-ACT) Trial in metastatic ACC reported the association between mitotane and etoposide/doxorubicin/cisplatin (EDP) as the new standard in first line treatment of ACC. In last years, new targeted therapies, including the IGF-1 receptor inhibitors, have been investigated, but their efficacy remains limited. Thus, new treatment concepts are urgently needed. The ongoing "omic approaches" and next-generation sequencing will improve our understanding of the pathogenesis and hopefully will lead to better therapies.
    Language English
    Publishing date 2015-07-03
    Publishing country Switzerland
    Document type Journal Article ; Review
    ZDB-ID 2737824-X
    ISSN 2296-634X
    ISSN 2296-634X
    DOI 10.3389/fcell.2015.00045
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  6. Article ; Online: Adrenocortical carcinoma (ACC)

    Rossella eLibé

    Frontiers in Cell and Developmental Biology, Vol

    diagnosis, prognosis and treatment.

    2015  Volume 3

    Abstract: Adrenocortical carticnoma (ACC) is a rare malignancy with an incidence of 0.7–2.0 cases/million habitants/year. The diagnosis of malignancy relies on careful investigations of clinical, biological and imaging features before surgery and pathological ... ...

    Abstract Adrenocortical carticnoma (ACC) is a rare malignancy with an incidence of 0.7–2.0 cases/million habitants/year. The diagnosis of malignancy relies on careful investigations of clinical, biological and imaging features before surgery and pathological examination after tumor removal. Most patients present with steroid hormone excess or abdominal mass effects, but 15% of patients with ACC is initially diagnosed incidentally. After the diagnosis, in order to assess the ACC prognosis and establish an adequate basis for treatment decisions different tools are proposed. The stage classification proposed by the European Network for the Study of Adrenal Tumors (ENSAT) is recommended. Pathology reports define the Weiss score, the resection status and the proliferative index, including the mitotic count and the Ki67 index. As far as the treatment is concerned, in case of tumor limited to the adrenal gland, the complete resection of the tumor is the first option. Most patients benefit from adjuvant mitotane treatment. In metastatic disease, mitotane is the cornerstone of initial treatment, and cytotoxic drugs should be added in case of progression. Recently, the First International Randomised (FIRM-ACT) Trial in metastatic ACC reported the association between mitotane and etoposide/ doxorubicin/cisplatin (EDP) as the new standard in first line treatment of ACC. In last years, new targeted therapies, including the IGF 1 receptor inhibitors, have been investigated, but their efficacy remains limited. Thus, new treatment concepts are urgently needed. The ongoing omic approaches and next-generation sequencing will improve our understanding of the pathogenesis and hopefully will lead to better therapies.
    Keywords Mitotane ; prognosis ; adrenocortical carcinoma (ACC) ; ENS@T staging ; target therapy ; Biology (General) ; QH301-705.5
    Subject code 610
    Language English
    Publishing date 2015-07-01T00:00:00Z
    Publisher Frontiers Media S.A.
    Document type Article ; Online
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  7. Article: Laparoscopic or Open Adrenalectomy for Stage I-II Adrenocortical Carcinoma: A Retrospective Study.

    Gaillard, Martin / Razafinimanana, Meva / Challine, Alexandre / Araujo, Raphael L C / Libé, Rossella / Sibony, Mathilde / Barat, Maxime / Bertherat, Jérôme / Dousset, Bertrand / Fuks, David / Gaujoux, Sebastien

    Journal of clinical medicine

    2023  Volume 12, Issue 11

    Abstract: Surgical resection of adrenocortical carcinoma (ACC) is the only curative treatment. Even in localized (I-II) stages, open adrenalectomy (OA) is the gold standard, though laparoscopic adrenalectomy (LA) can be proposed in selected patients. Despite the ... ...

    Abstract Surgical resection of adrenocortical carcinoma (ACC) is the only curative treatment. Even in localized (I-II) stages, open adrenalectomy (OA) is the gold standard, though laparoscopic adrenalectomy (LA) can be proposed in selected patients. Despite the postoperative benefits of LA, its role in the surgical management of patients with ACC remains controversial regarding oncologic outcomes. The aim of this retrospective study was to compare the outcomes of patients with localized ACC submitted to LA or OA in a referral center from 1995 to 2020. Among 180 consecutive patients operated on for ACC, 49 presented with localized ACC (19 LA and 30 OA). Baseline characteristics were similar between groups, except for tumor size. Kaplan-Meier estimates of 5-year overall survival were similar in both groups (
    Language English
    Publishing date 2023-05-26
    Publishing country Switzerland
    Document type Journal Article
    ZDB-ID 2662592-1
    ISSN 2077-0383
    ISSN 2077-0383
    DOI 10.3390/jcm12113698
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  8. Article ; Online: Letter to the Editor from Berthon: "Cardiac Myxoma Caused by Fumarate Hydratase Gene Deletion in Patient With Cortisol-Secreting Adrenocortical Adenoma".

    Berthon, Annabel / Libe, Rossella / Bertherat, Jérôme / Stratakis, Constantine A

    The Journal of clinical endocrinology and metabolism

    2020  Volume 105, Issue 11

    MeSH term(s) Adrenal Cortex Neoplasms/genetics ; Adrenocortical Adenoma/diagnostic imaging ; Adrenocortical Adenoma/genetics ; Fumarate Hydratase/genetics ; Gene Deletion ; Humans ; Hydrocortisone ; Myxoma/diagnostic imaging ; Myxoma/genetics
    Chemical Substances Fumarate Hydratase (EC 4.2.1.2) ; Hydrocortisone (WI4X0X7BPJ)
    Language English
    Publishing date 2020-08-18
    Publishing country United States
    Document type Letter ; Comment
    ZDB-ID 3029-6
    ISSN 1945-7197 ; 0021-972X
    ISSN (online) 1945-7197
    ISSN 0021-972X
    DOI 10.1210/clinem/dgaa530
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  9. Article ; Online: Outcome of adrenocortical carcinoma patients included in early phase clinical trials: Results from the French network ENDOCAN-COMETE.

    Hescot, Ségolène / Debien, Véronique / Hadoux, Julien / Drui, Delphine / Haissaguerre, Magalie / de la Fouchardiere, Christelle / Vezzosi, Delphine / Do Cao, Christine / Libé, Rossella / Le Tourneau, Christophe / Baudin, Eric / Massard, Christophe / du Rusquec, Pauline

    European journal of cancer (Oxford, England : 1990)

    2023  Volume 189, Page(s) 112917

    Abstract: Background: At metastatic stage, treatment of adrenocortical carcinoma (ACC) relies in first line on mitotane therapy, combination of mitotane with locoregional therapies or cisplatin-based chemotherapy according to initial presentation. In second line, ...

    Abstract Background: At metastatic stage, treatment of adrenocortical carcinoma (ACC) relies in first line on mitotane therapy, combination of mitotane with locoregional therapies or cisplatin-based chemotherapy according to initial presentation. In second line, ESMO-EURACAN recommendations favour enrolment of patients in clinical trials investigating experimental therapies. However, the benefit of this approach remains unknown.
    Methods: The aim of our retrospective study was to analyse the inclusion and outcomes of all patients of the French cohort ENDOCAN-COMETE included in early clinical trials between 2009 and 2019.
    Results: Of the 141 patients for whom a local or national multidisciplinary tumour board recommended, as first choice, to look for clinical trial, 27 patients (19%) were enroled in 30 early clinical trials. Median progression-free survival (PFS) was 3.02 months (95% confidence interval [95% CI]; 2.3-4.6) and median overall survival (OS) was 10.2 months (95% CI; 7.13-16.3) while the best response, evaluable in 28 of 30 trial participants according to RECIST 1.1 criteria, was partial response for 3 patients (11%) stable disease for 14 patients (50%) and progressive disease for 11 patients (39%), resulting in a disease control rate of 61%. Median growth modulation index (GMI) in our cohort was 1.32, with a significantly prolonged PFS in 52% of the patients compared to the previous line. The Royal Marsden Hospital (RMH) prognostic score was not associated with OS in this cohort.
    Conclusion: Our study suggests that patients with metastatic ACC benefit from inclusion in early clinical trials in second line. As recommended, if a clinical trial is available, it should be the first choice for suitable patients.
    MeSH term(s) Humans ; Adrenal Cortex Neoplasms/drug therapy ; Adrenal Cortex Neoplasms/pathology ; Adrenocortical Carcinoma/drug therapy ; Adrenocortical Carcinoma/pathology ; Antineoplastic Combined Chemotherapy Protocols/therapeutic use ; Mitotane/adverse effects ; Retrospective Studies ; Treatment Outcome
    Chemical Substances Mitotane (78E4J5IB5J)
    Language English
    Publishing date 2023-05-16
    Publishing country England
    Document type Clinical Trial ; Journal Article
    ZDB-ID 82061-1
    ISSN 1879-0852 ; 0277-5379 ; 0959-8049 ; 0964-1947
    ISSN (online) 1879-0852
    ISSN 0277-5379 ; 0959-8049 ; 0964-1947
    DOI 10.1016/j.ejca.2023.05.006
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  10. Article ; Online: An Ectopic Parathyroid Adenoma Mimicking a Carotid Body Paraganglioma.

    Libé, Rossella / Calvani, Julien / Cottereau, Anne-Ségolène / Connan, Tatiana Lecot / Gaujoux, Sebastien / Groussin, Lionel / Wartski, Myriam

    Journal of the Endocrine Society

    2020  Volume 4, Issue 12, Page(s) bvaa143

    Language English
    Publishing date 2020-11-20
    Publishing country United States
    Document type Journal Article
    ISSN 2472-1972
    ISSN (online) 2472-1972
    DOI 10.1210/jendso/bvaa143
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