Temporal Trends in Outcomes in Patients With Adrenocortical Carcinoma: A Multidisciplinary Referral-center Experience

Authors

Marilyne Daher
Jeena Varghese
Stephen K Gruschkus
Camilo Jimenez
Steven G Waguespack
Sara Bedrose
Lina Altameemi
Hadil Bazerbashi
Aung Naing
Vivek Subaiah
Matthew T Campbell
Amishi Y Shah
Miao Zhang
Rahul A Sheth
Jose A Karam
Christopher G Wood
Nancy D Perrier
Paul H Graham
Jeffery E Lee
Mouhammed Amir Habra

Publication Date

4-19-2022

Journal

The Journal of Clinical Endocrinology and Metabolism

DOI

10.1210/clinem/dgac046

PMID

35092681

PMCID

PMC9016449

PubMedCentral® Posted Date

1-29-2022

PubMedCentral® Full Text Version

Post-print

Published Open-Access

yes

Keywords

Adrenal Cortex Neoplasms, Adrenocortical Carcinoma, Antineoplastic Agents, Hormonal, Humans, Mitotane, Referral and Consultation, Retrospective Studies, adrenocortical carcinoma, recurrence, survival, surgery, mitotane, immunotherapy

Abstract

CONTEXT: Reporting temporal trends in adrenocortical carcinoma (ACC) helps guide management strategies.

OBJECTIVE: This work aimed to report the trends in disease burden and clinical outcomes over time that cannot be adequately captured from individual clinical trials.

METHODS: A retrospective study was held of ACC patients seen at a referral cancer center between February 1998 and August 2019. Clinical outcomes were compared between an early cohort (February 1998-June 2007) and a late cohort (July 2007-August 2019).

RESULTS: A total of 621 patients included with a median age at diagnosis of 49.3 years (range, 0.5-86.6 years). There were 285 (45.9%) patients with hormonal overproduction. More patients in the late cohort had stage IV disease compared to the early cohort (36.8% vs 23.1%; P < .0001). Resection of the primary tumor was performed in 502 patients (80.8%). Complete resection (R0) was more common in the late cohort (165 [60.2%]) than in the early cohort (100 [44.6%]; P = .0005). Of 475 patients with metastatic disease (stage IV or recurrent metastatic disease), 352 (74.1%) received mitotane, 320 (67.4%) received chemotherapy, and 53 (11.2%) received immunotherapy. In the early cohort, 70 (33%) received 2 or more lines of therapy, whereas in the late cohort, 127 (48%) received 2 or more lines of therapy. The 5-year overall survival (OS) rates were 65%, 58%, 45%, and 10% for stage I, II, III, and IV disease, respectively, whereas the 2-year OS rates in patients with stage IV disease was 24% in the early cohort and 46% in the late cohort (P = .01).

CONCLUSION: ACC clinical outcomes improved over the past 2 decades as more patients had complete resection or received more lines of systemic therapy.