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.
Included in
Endocrine System Diseases Commons, Endocrinology, Diabetes, and Metabolism Commons, Immunotherapy Commons, Medical Sciences Commons
Comments
This article has been corrected. See J Clin Endocrinol Metab. 2022 May 10;107(8):e3544.
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