New Findings on Presentation and Outcome of Patients With Adrenocortical Cancer: Results From a National Cohort Study

• Published in: The journal of clinical endocrinology and metabolism Vol. 108; no. 10; pp. 2517 - 2525
• Main Authors: Puglisi, Soraya, Calabrese, Anna, Ferraù, Francesco, Violi, Maria Antonia, Laganà, Marta, Grisanti, Salvatore, Ceccato, Filippo, Scaroni, Carla, Di Dalmazi, Guido, Stigliano, Antonio, Altieri, Barbara, Canu, Letizia, Loli, Paola, Pivonello, Rosario, Arvat, Emanuela, Morelli, Valentina, Perotti, Paola, Basile, Vittoria, Berchialla, Paola, Urru, Sara, Fiori, Cristian, Porpiglia, Francesco, Berruti, Alfredo, Pia, Anna, Reimondo, Giuseppe, Cannavò, Salvatore, Terzolo, Massimo
• Format: Journal Article
• Language: English
• Published: US Oxford University Press 18-09-2023
• Subjects: Adrenal Cortex Neoplasms - diagnosis; Adrenal Cortex Neoplasms - epidemiology; Adrenal Cortex Neoplasms - surgery; Adrenal Gland Neoplasms - drug therapy; Adrenocortical Carcinoma - diagnosis; Adrenocortical Carcinoma - epidemiology; Adrenocortical Carcinoma - therapy; Cohort Studies; Cortisol; Female; Hormones; Humans; Hydrocortisone - therapeutic use; Ki-67 Antigen; Male; Medical prognosis; Mitotane - therapeutic use; Neuroendocrine tumors; Patients; Population studies; Retrospective Studies; Secretion; Surgery; Survival; Tumors; recurrence; mitotane; adrenal incidentaloma; gender disease; survival; surgery
• ISSN: 0021-972X; 1945-7197
• DOI: 10.1210/clinem/dgad199

Abstract
Context
Because of the rarity of adrenocortical cancer (ACC), only a few population-based studies are available, and they reported limited details in the characterization of patients and their treatment.
Objective
To describe in a nationwide cohort the presentation of patients with ACC, treatment strategies, and potential prognostic factors.
Methods
Retrospective analysis of 512 patients with ACC, diagnosed in 12 referral centers in Italy from January 1990 to June 2018.
Results
ACC diagnosed as incidentalomas accounted for overall 38.1% of cases, with a frequency that increases with age and with less aggressive pathological features than symptomatic tumors. Women (60.2%) were younger than men and had smaller tumors, which more frequently secreted hormones. Surgery was mainly done with an open approach (72%), and after surgical resection, 62.7% of patients started adjuvant mitotane therapy. Recurrence after tumor resection occurred in 56.2% of patients. In patients with localized disease, cortisol secretion, ENSAT stage III, Ki67%, and Weiss score were associated with an increased risk of recurrence, whereas margin-free resection, open surgery, and adjuvant mitotane treatment were associated with reduced risk. Death occurred in 38.1% of patients and recurrence-free survival (RFS) predicted overall survival (OS). In localized disease, age, cortisol secretion, Ki67%, ENSAT stage III, and recurrence were associated with increased risk of mortality. ACCs presenting as adrenal incidentalomas showed prolonged RFS and OS.
Conclusion
Our study shows that ACC is a sex-related disease and demonstrates that an incidental presentation is associated with a better outcome. Given the correlation between RFS and OS, RFS may be used as a surrogate endpoint in clinical studies.