1 Department of Medical and Surgical Specialties, Radiological Sciences and Public Health, Medical Oncology Unit, University of Brescia at ASST Spedali Civili, Brescia, Italy
2 Department of Endocrinology and Metabolic Diseases, Hôpital Cochin, Paris, France
3 Department of Internal Medicine, Maxima Medisch Centrum Eindhoven/Veldhoven, Maastricht University, CAPHRI School for Public Health and Primary Care, Ageing and Long-Term Care, Maastricht, the Netherlands
4 The University of Texas MD Anderson Cancer Center, Houston, Texas, USA
5 Erasmus Medical Center, Department of Internal Medicine, Academic Expertise Center for Adrenal and Neuroendocrine Tumors, Rotterdam, the Netherlands
6 Department of Nuclear Medicine and Endocrine Oncology, Gustave Roussy Cancer Center, Villejuif, France
7 Department of Biomedical Sciences Humanitas University and Endocrine and Andrology Unit, Humanitas Clinical and Research, Milan, Italy
8 Section of Pharmacology, Department of Molecular and Translational Medicine, University of Brescia, Brescia, Italy
9 Division of General Internal Medicine, Department of Internal Medicine, Maastricht University Medical Centre+, Maastricht, the Netherlands
10 Internal Medicine, Department of Clinical and Biological Sciences, San Luigi Gonzaga Hospital, Orbassano, University of Turin, Turin, Italy
Correspondence should be addressed to A Berruti; Email: firstname.lastname@example.org
Adrenocortical carcinoma (ACC) is a rare cancer that commonly spreads to the liver, lungs and lymph nodes. Bone metastases are infrequent.
The aim of this report was to describe the clinical characteristics, survival perspective, prognostic factors and frequency of adverse skeletal-related events (SREs) in patients with ACC who developed bone metastasis.
This is a retrospective, observational, multicenter, multinational study of patients diagnosed with bone metastases from ACC who were treated and followed up in three European countries (France, Italy and The Netherlands) and one center in the United States.
Data of 156 patients were captured. The median overall survival was 11 months. SREs occurred in 47% of patients: 17% bone fractures, 17% spinal cord compression, 1% hypercalcemia, 12% developed more than one SRE. In multivariate analysis, cortisol hypersecretion was the only prognostic factor significantly associated with a higher mortality risk (hazard ratio (HR) 2.24, 95% confidence interval (CI): 1.19–4.23, P = 0.013) and with the development of a SREs (of border line significance). The administration of antiresorptive therapies (bisphosphonates and denosumab) was associated with a lower risk of death, even if not significant, and their survival benefit appeared confined in patients attaining serum mitotane levels within the therapeutic range.
Bone metastases in ACC patients are associated with poor prognosis and high risk of SREs. Cortisol hypersecretion was the only prognostic factor suggesting a potential benefit from antisecretory medications. The therapeutic role of bisphosphonates and denosumab to improve patient outcome deserves to be tested in a prospective clinical trial.
Supplementary Table 1. Predictive factors for the time to the occurrence of Pathological Fracture according to univariate Cox analysis
Supplementary Table 2. Predictive factors for the time to the occurrence of Spinal cord compression according to univariate Cox analysis