Medical Approaches in Adrenocortical Carcinoma

Adrenocortical carcinoma (ACC) represents one of the most aggressive endocrine tumors. In spite of a correct therapeutic strategy based on a multidisciplinary approach between endocrinologist, surgeon and oncologist, the prognosis is often poor. Surgery is the mainstay treatment in ACC. Mitotane, a...

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Main Authors: Rosa Maria Paragliola, Andrea Corsello, Pietro Locantore, Giampaolo Papi, Alfredo Pontecorvi, Salvatore Maria Corsello
Format: Article
Language:English
Published: MDPI AG 2020-11-01
Series:Biomedicines
Subjects:
Online Access:https://www.mdpi.com/2227-9059/8/12/551
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author Rosa Maria Paragliola
Andrea Corsello
Pietro Locantore
Giampaolo Papi
Alfredo Pontecorvi
Salvatore Maria Corsello
author_facet Rosa Maria Paragliola
Andrea Corsello
Pietro Locantore
Giampaolo Papi
Alfredo Pontecorvi
Salvatore Maria Corsello
author_sort Rosa Maria Paragliola
collection DOAJ
description Adrenocortical carcinoma (ACC) represents one of the most aggressive endocrine tumors. In spite of a correct therapeutic strategy based on a multidisciplinary approach between endocrinologist, surgeon and oncologist, the prognosis is often poor. Surgery is the mainstay treatment in ACC. Mitotane, a dichloro-diphenyl-trichloro-ethane derivate, represents the main medical treatment of ACC in consideration of its adrenocytolitic activity and it is mainly employed as adjuvant treatment after complete surgical resection and for the treatment of advanced ACC. However, the use of mitotane as adjuvant therapy is still controversial, also in consideration of the retrospective nature of several studies. The recurrence of disease is frequent, especially in advanced disease at the diagnosis. Therefore, in these contexts, conventional chemotherapy must be considered in association with mitotane, being the combination etoposide, doxorubicin and cisplatin (EDP) the standard of care in this setting. A more modern therapeutic approach, based on the need of a salvage therapy for advanced ACC that progresses through first-line EDP, is focused on molecular-targeted therapies. However, robust clinical trials are necessary to assess the real efficacy of these treatments.
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spelling doaj.art-bbab1b35e16d4b22a4a05433c121734b2023-11-20T22:53:58ZengMDPI AGBiomedicines2227-90592020-11-0181255110.3390/biomedicines8120551Medical Approaches in Adrenocortical CarcinomaRosa Maria Paragliola0Andrea Corsello1Pietro Locantore2Giampaolo Papi3Alfredo Pontecorvi4Salvatore Maria Corsello5Department of Translational Medicine and Surgery, Unit of Endocrinology, Università Cattolica del Sacro Cuore—Fondazione Policlinico “Gemelli” IRCCS, Largo Gemelli 8, 00168 Rome, ItalyDepartment of Translational Medicine and Surgery, Unit of Endocrinology, Università Cattolica del Sacro Cuore—Fondazione Policlinico “Gemelli” IRCCS, Largo Gemelli 8, 00168 Rome, ItalyDepartment of Translational Medicine and Surgery, Unit of Endocrinology, Università Cattolica del Sacro Cuore—Fondazione Policlinico “Gemelli” IRCCS, Largo Gemelli 8, 00168 Rome, ItalyDepartment of Translational Medicine and Surgery, Unit of Endocrinology, Università Cattolica del Sacro Cuore—Fondazione Policlinico “Gemelli” IRCCS, Largo Gemelli 8, 00168 Rome, ItalyDepartment of Translational Medicine and Surgery, Unit of Endocrinology, Università Cattolica del Sacro Cuore—Fondazione Policlinico “Gemelli” IRCCS, Largo Gemelli 8, 00168 Rome, ItalyDepartment of Translational Medicine and Surgery, Unit of Endocrinology, Università Cattolica del Sacro Cuore—Fondazione Policlinico “Gemelli” IRCCS, Largo Gemelli 8, 00168 Rome, ItalyAdrenocortical carcinoma (ACC) represents one of the most aggressive endocrine tumors. In spite of a correct therapeutic strategy based on a multidisciplinary approach between endocrinologist, surgeon and oncologist, the prognosis is often poor. Surgery is the mainstay treatment in ACC. Mitotane, a dichloro-diphenyl-trichloro-ethane derivate, represents the main medical treatment of ACC in consideration of its adrenocytolitic activity and it is mainly employed as adjuvant treatment after complete surgical resection and for the treatment of advanced ACC. However, the use of mitotane as adjuvant therapy is still controversial, also in consideration of the retrospective nature of several studies. The recurrence of disease is frequent, especially in advanced disease at the diagnosis. Therefore, in these contexts, conventional chemotherapy must be considered in association with mitotane, being the combination etoposide, doxorubicin and cisplatin (EDP) the standard of care in this setting. A more modern therapeutic approach, based on the need of a salvage therapy for advanced ACC that progresses through first-line EDP, is focused on molecular-targeted therapies. However, robust clinical trials are necessary to assess the real efficacy of these treatments.https://www.mdpi.com/2227-9059/8/12/551adrenocortical carcinomamitotaneetoposidecisplatindoxorubicinimmune checkpoint inhibitors
spellingShingle Rosa Maria Paragliola
Andrea Corsello
Pietro Locantore
Giampaolo Papi
Alfredo Pontecorvi
Salvatore Maria Corsello
Medical Approaches in Adrenocortical Carcinoma
Biomedicines
adrenocortical carcinoma
mitotane
etoposide
cisplatin
doxorubicin
immune checkpoint inhibitors
title Medical Approaches in Adrenocortical Carcinoma
title_full Medical Approaches in Adrenocortical Carcinoma
title_fullStr Medical Approaches in Adrenocortical Carcinoma
title_full_unstemmed Medical Approaches in Adrenocortical Carcinoma
title_short Medical Approaches in Adrenocortical Carcinoma
title_sort medical approaches in adrenocortical carcinoma
topic adrenocortical carcinoma
mitotane
etoposide
cisplatin
doxorubicin
immune checkpoint inhibitors
url https://www.mdpi.com/2227-9059/8/12/551
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AT alfredopontecorvi medicalapproachesinadrenocorticalcarcinoma
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