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. 2020 Apr 10;12(4):941.
doi: 10.3390/cancers12040941.

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

Marta Laganà  1 , Salvatore Grisanti  1 , Deborah Cosentini  1 , Vittorio Domenico Ferrari  1 , Barbara Lazzari  1 , Roberta Ambrosini  2 , Chiara Sardini  1 , Alberto Dalla Volta  1 , Carlotta Palumbo  3 , Pietro Luigi Poliani  4 , Massimo Terzolo  5 , Sandra Sigala  6 , Guido Alberto Massimo Tiberio  7 , Alfredo Berruti  1
Affiliations

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

Marta Laganà et al. Cancers (Basel). .

Abstract

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.

Keywords: EDP; adrenocortical tumor; mitotane; treatment.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
Progression-free survival.
Figure 2
Figure 2
Overall survival.
Figure 3
Figure 3
Thorax CT scan series of a patient attaining a pathological complete response after six cycles of etoposide, doxorubicin and cisplatin plus oral mitotane (EDP-M).
Figure 4
Figure 4
Progression-free survival according to post-EDP surgery.
Figure 5
Figure 5
Overall survival according to post-EDP surgery.
Figure 6
Figure 6
Progression-free survival according to Ki67 after post-EDP surgery.
Figure 7
Figure 7
Overall survival according to Ki67 assessed after post-EDP surgery.
See this image and copyright information in PMC

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