Clinical management and outcomes associated with etoposide, doxorubicin, and cisplatin plus mitotane treatment in metastatic adrenocortical carcinoma: a single institute experience

Masaki Uchihara, Maki Tanioka, Yuki Kojima, Tadaaki Nishikawa, Kazuki Sudo, Tatsunori Shimoi, Emi Noguchi, Akiko Miyagi Maeshima, Kan Yonemori

Research output: Contribution to journalArticlepeer-review

2 Citations (Scopus)

Abstract

Background: Adrenocortical carcinoma (ACC) is a rare and aggressive disease that is often diagnosed at an advanced stage. There is no standard treatment for metastatic ACC; EDP-M (etoposide, doxorubicin, and cisplatin plus mitotane) is one treatment option. A randomized controlled trial (FIRM-ACT) evaluating the efficacy of EDP-M showed progression-free survival (PFS) was 5.0 months, overall survival (OS) was 14.8 months, the response rate was 19%, and adrenal insufficiency occurred in 3.4% of patients. However, the efficacy and safety of this regimen in Asia are not fully reported. Methods: We retrospectively analyzed 43 patients diagnosed with metastatic ACC at the National Cancer Center Hospital between 1997 and 2020. We evaluated PFS, OS, and response in 17 patients who received EDP-M as first-line therapy. Results: The median age at treatment initiation was 45 years (range 18–74). Eight patients (47%) had autonomous hormone production, including six patients with hypercortisolism. The best response of partial response and stable disease was seen in two (12%) and ten (59%) patients, respectively. The median PFS was 6.2 months [95% confidence interval (CI): 4.3–10.0]. The median OS was 15.4 months (95% CI 11.6–not reached). Three patients received only one cycle due to adverse effects associated with hypercortisolism. Grade 3/4 adverse events associated with adrenal insufficiency occurred in three (17%) cases, resulting in EDP-M discontinuation. Conclusions: The EDP-M regimen had similar PFS to that observed in FIRM-ACT. Adrenal insufficiency was more frequent in the current study, but this could be managed with supportive endocrinological care such as cortisol replacement.

Original languageEnglish
Pages (from-to)2275-2281
Number of pages7
JournalInternational Journal of Clinical Oncology
Volume26
Issue number12
DOIs
Publication statusPublished - Dec 2021
Externally publishedYes

Keywords

  • Adrenal insufficiency
  • Adrenocortical carcinoma
  • EDP
  • Hypercortisolism
  • Mitotane

ASJC Scopus subject areas

  • Surgery
  • Hematology
  • Oncology

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