Introduction: Adrenocortical cancer is a rare malignant tumour with a poor prognosis. The incidence is 0.7–2.0/million/year.
Objectives: The aim of this study was to characterise the clinicopathological features and prognostic factors of a large cohort of patients with adrenocortical cancer diagnosed between 1974–2019.
Patients and Methods: The study included eighty patients (22 men and 58 women) with histologically confirmed adrenocortical cancer in our single tertiary referral endocrine unit. Sixty-two and forty-three of the patients were diagnosed after years 2000 and 2010, resp. Descriptive statistics were performed, providing summaries of selected clinical and pathological parameters at the time of diagnosis. The results are given in median (minimum-maximum). Factors contributing to overall survival were analysed. A p value of less than 0.05 was considered statistically significant.
Results: The median age of patients was 52 years (18–84 years) at diagnosis. The majority of cases was diagnosed at ENSAT stage II (41.4%) and stage IV (34.3%), the median tumour size was 9.5 cm (4–30 cm) at diagnosis. In 57 patients (76%) the tumour was hormonally active. Primary surgical tumour resection was performed in 70 patients (87.5%), in 30 patients R0 resection was achieved. The median overall survival and the 5-year survival rate were 23 months (0–274 months) and 20%, respectively. In univariate Cox regression model, older age (>57 years), tumours with stage III-IV, high mitotic activity of the tumour cells (Ki67-index >20%) and R1-R2 surgical resection state were associated with poorer survival. The overall survival of patients achieving therapeutic mitotane plasma concentration (n = 21) was significantly better comparing to those who failed to achieve the therapeutic serum mitotane concentration (n = 22) (38 (13–193) months vs 18 (2–101) months). The time needed to reach the therapeutic range of serum mitotane was 4 (2–12) months. The median age, the distribution of gender, ENSAT stage, resection stage and Ki67-index stratified to 20% did not differ between these two groups. In patients with stage III-IV disease, there was no significant difference in overall survival between those who were treated with mitotane monotherapy compared to those with combined (mitotane plus chemo- therapy (19 (2–193) months, n = 8 vs 18.5 (4–76) months, n = 18).
Conclusion: Our results confirmprevious data that age, disease stage, mitotic activity and the resection stage are the most critical factors influencing the prognosis of adrenocortical cancer. The rapid achievement of therapeutic mitotane concentration has a significant and independent effect on overall survival.
05 Sep 2020 - 09 Sep 2020