Efficacy of the EDP-M Scheme Plus Adjunctive Surgery in the Management of Patients with Advanced Adrenocortical Carcinoma: The Brescia Experience

Etoposide, doxorubicin and cisplatin plus oral mitotane (EDP-M) comprise the reference regimen in the management of patients with adrenocortical carcinoma (ACC). In this paper, we described the outcome of 58 patients with advanced/metastatic ACC consecutively treated with EDP-M in a reference center...

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Main Authors: Marta Laganà, Salvatore Grisanti, Deborah Cosentini, Vittorio Domenico Ferrari, Barbara Lazzari, Roberta Ambrosini, Chiara Sardini, Alberto Dalla Volta, Carlotta Palumbo, Pietro Luigi Poliani, Massimo Terzolo, Sandra Sigala, Guido Alberto Massimo Tiberio, Alfredo Berruti
Format: Article
Language:English
Published: MDPI AG 2020-04-01
Series:Cancers
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Online Access:https://www.mdpi.com/2072-6694/12/4/941
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author Marta Laganà
Salvatore Grisanti
Deborah Cosentini
Vittorio Domenico Ferrari
Barbara Lazzari
Roberta Ambrosini
Chiara Sardini
Alberto Dalla Volta
Carlotta Palumbo
Pietro Luigi Poliani
Massimo Terzolo
Sandra Sigala
Guido Alberto Massimo Tiberio
Alfredo Berruti
author_facet Marta Laganà
Salvatore Grisanti
Deborah Cosentini
Vittorio Domenico Ferrari
Barbara Lazzari
Roberta Ambrosini
Chiara Sardini
Alberto Dalla Volta
Carlotta Palumbo
Pietro Luigi Poliani
Massimo Terzolo
Sandra Sigala
Guido Alberto Massimo Tiberio
Alfredo Berruti
author_sort Marta Laganà
collection DOAJ
description Etoposide, doxorubicin and cisplatin plus oral mitotane (EDP-M) comprise the reference regimen in the management of patients with adrenocortical carcinoma (ACC). In this paper, we described the outcome of 58 patients with advanced/metastatic ACC consecutively treated with EDP-M in a reference center for this rare disease in Italy. In this series, EDP-M obtained a partial response in 50% of patients; median progression free survival (PFS) and overall survival were 10.1 months (95% Confidence Interval [CI 95%] 8.1–12.8) and 18.7 months (95% CI: 14.6–22.8), respectively. EDP-M was not interrupted in five patients showing disease progression after two cycles without the appearance of new lesions and mitotane levels below the therapeutic range. In two of them, the disease remained stable at further imaging evaluations and the other three obtained a partial response. Twenty-six responding patients underwent surgery of residual disease and 13 of them became disease free. Surgery identified a pathological complete response (pCR) in four patients (7%) and Ki67 expression in post-chemotherapy tumor specimens, inferior to 15% (median value), was associated with better PFS and survival. In the present study, the EDP-M regimen is confirmed to have a limited efficacy. Early disease progression does not mean treatment inefficacy. Surgery of residual disease in partially responding patients allows for the detection of pCR in few of them and this condition is predictive of long-term survival. Ki67 expression of post-chemotherapy residual disease could be an additional prognostic factor that deserves to be studied further.
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spelling doaj.art-82160d30f209420dab63520b7e304b2c2023-11-19T21:17:49ZengMDPI AGCancers2072-66942020-04-0112494110.3390/cancers12040941Efficacy of the EDP-M Scheme Plus Adjunctive Surgery in the Management of Patients with Advanced Adrenocortical Carcinoma: The Brescia ExperienceMarta Laganà0Salvatore Grisanti1Deborah Cosentini2Vittorio Domenico Ferrari3Barbara Lazzari4Roberta Ambrosini5Chiara Sardini6Alberto Dalla Volta7Carlotta Palumbo8Pietro Luigi Poliani9Massimo Terzolo10Sandra Sigala11Guido Alberto Massimo Tiberio12Alfredo Berruti13Medical Oncology Unit, Department of Medical and Surgical Specialties, Radiological Sciences and Public Health, University of Brescia at ASST Spedali Civili, 25123 Brescia, ItalyMedical Oncology Unit, Department of Medical and Surgical Specialties, Radiological Sciences and Public Health, University of Brescia at ASST Spedali Civili, 25123 Brescia, ItalyMedical Oncology Unit, Department of Medical and Surgical Specialties, Radiological Sciences and Public Health, University of Brescia at ASST Spedali Civili, 25123 Brescia, ItalyMedical Oncology Unit, Department of Medical and Surgical Specialties, Radiological Sciences and Public Health, University of Brescia at ASST Spedali Civili, 25123 Brescia, ItalyMedical Oncology Unit, Department of Medical and Surgical Specialties, Radiological Sciences and Public Health, University of Brescia at ASST Spedali Civili, 25123 Brescia, ItalyRadiology Unit, ASST Spedali Civili, 25123 Brescia, ItalyMedical Oncology Unit, Department of Medical and Surgical Specialties, Radiological Sciences and Public Health, University of Brescia at ASST Spedali Civili, 25123 Brescia, ItalyMedical Oncology Unit, Department of Medical and Surgical Specialties, Radiological Sciences and Public Health, University of Brescia at ASST Spedali Civili, 25123 Brescia, ItalyUrology Unit, Department of Medical and Surgical Specialties, Radiological Science and Public Health, University of Brescia at ASST Spedali Civili of Brescia, 25123 Brescia, ItalyPathology Unit, Department of Molecular and Translational Medicine, University of Brescia at ASST Spedali Civili di Brescia, 25123 Brescia, ItalyInternal Medicine, Department of Clinical and Biological Sciences, San Luigi Hospital, University of Turin, Orbassano, 10043 Turin, ItalySection of Pharmacology, Department of Molecular and Translational Medicine, University of Brescia, 25123 Brescia, ItalySurgical Unit, Department of Medical and Surgical Specialties, Radiological Sciences, and Public Health, University of Brescia, ASST Spedali Civili di Brescia, 25123 Brescia, ItalyMedical Oncology Unit, Department of Medical and Surgical Specialties, Radiological Sciences and Public Health, University of Brescia at ASST Spedali Civili, 25123 Brescia, ItalyEtoposide, doxorubicin and cisplatin plus oral mitotane (EDP-M) comprise the reference regimen in the management of patients with adrenocortical carcinoma (ACC). In this paper, we described the outcome of 58 patients with advanced/metastatic ACC consecutively treated with EDP-M in a reference center for this rare disease in Italy. In this series, EDP-M obtained a partial response in 50% of patients; median progression free survival (PFS) and overall survival were 10.1 months (95% Confidence Interval [CI 95%] 8.1–12.8) and 18.7 months (95% CI: 14.6–22.8), respectively. EDP-M was not interrupted in five patients showing disease progression after two cycles without the appearance of new lesions and mitotane levels below the therapeutic range. In two of them, the disease remained stable at further imaging evaluations and the other three obtained a partial response. Twenty-six responding patients underwent surgery of residual disease and 13 of them became disease free. Surgery identified a pathological complete response (pCR) in four patients (7%) and Ki67 expression in post-chemotherapy tumor specimens, inferior to 15% (median value), was associated with better PFS and survival. In the present study, the EDP-M regimen is confirmed to have a limited efficacy. Early disease progression does not mean treatment inefficacy. Surgery of residual disease in partially responding patients allows for the detection of pCR in few of them and this condition is predictive of long-term survival. Ki67 expression of post-chemotherapy residual disease could be an additional prognostic factor that deserves to be studied further.https://www.mdpi.com/2072-6694/12/4/941adrenocortical tumortreatmentEDPmitotane
spellingShingle Marta Laganà
Salvatore Grisanti
Deborah Cosentini
Vittorio Domenico Ferrari
Barbara Lazzari
Roberta Ambrosini
Chiara Sardini
Alberto Dalla Volta
Carlotta Palumbo
Pietro Luigi Poliani
Massimo Terzolo
Sandra Sigala
Guido Alberto Massimo Tiberio
Alfredo Berruti
Efficacy of the EDP-M Scheme Plus Adjunctive Surgery in the Management of Patients with Advanced Adrenocortical Carcinoma: The Brescia Experience
Cancers
adrenocortical tumor
treatment
EDP
mitotane
title Efficacy of the EDP-M Scheme Plus Adjunctive Surgery in the Management of Patients with Advanced Adrenocortical Carcinoma: The Brescia Experience
title_full Efficacy of the EDP-M Scheme Plus Adjunctive Surgery in the Management of Patients with Advanced Adrenocortical Carcinoma: The Brescia Experience
title_fullStr Efficacy of the EDP-M Scheme Plus Adjunctive Surgery in the Management of Patients with Advanced Adrenocortical Carcinoma: The Brescia Experience
title_full_unstemmed Efficacy of the EDP-M Scheme Plus Adjunctive Surgery in the Management of Patients with Advanced Adrenocortical Carcinoma: The Brescia Experience
title_short Efficacy of the EDP-M Scheme Plus Adjunctive Surgery in the Management of Patients with Advanced Adrenocortical Carcinoma: The Brescia Experience
title_sort efficacy of the edp m scheme plus adjunctive surgery in the management of patients with advanced adrenocortical carcinoma the brescia experience
topic adrenocortical tumor
treatment
EDP
mitotane
url https://www.mdpi.com/2072-6694/12/4/941
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