ObjectivesThis study aimed to characterise the clinicopathological features and
prognostic factors of a large cohort of Hungarian patients with adrenocortical cancer
diagnosed between 2000-2021.Patients and methodsThis retrospective study
included seventy-four patients (27 men and 47 women) with histologically confirmed
adrenocortical cancer in a single tertiary referral endocrine centre. Descriptive
statistics were performed, providing summaries of selected clinical and pathological
parameters. Clinicopathological factors contributing to overall survival were analysed.ResultsThe
median age of patients was 48,5 years (17-84 years) at diagnosis. The majority of
cases were diagnosed at ENSAT stage II (39,2%) and stage IV (33,8%). At diagnosis,
the median tumour size was 9,0 cm (4,5-20 cm). In 47 patients (71,6%), the tumour
was hormonally active. The median overall survival and the 5-year survival rate were
23,5 months (95% CI, 17-30,5 months) and 18,3%, respectively. Primary tumour resection
was performed in 68 patients (91,8%); R0 surgical resection was achieved in 30 patients.
In univariate Cox regression model, tumours with stages III and IV, high proliferative
activity (Ki67-index > 10%), R1-R2 surgical resection state and hormonal activity
were associated with poorer survival. Cortisol excess, both isolated and combined
with androgen production, was associated with poorer survival. Fifty-five patients
were treated with mitotane. The overall survival of patients achieving therapeutic
mitotane plasma concentration was significantly better compared to those who never
reached it [27.0 (2-175) months vs 18.0 (2-83) months; p<0.05)]. The median age, the
distribution of gender, ENSAT stage, resection state and Ki67-index did not differ
between these two groups. The time needed to reach the therapeutic range of serum
mitotane was 96.5 days (95% CI, 75-133 days).ConclusionOur results confirm
previous data that disease stage, mitotic activity, the resection state and the mitotane
treatment achieving therapeutic concentration are the most critical parameters influencing
the prognosis of adrenocortical cancer. Our data suggest that hormonal activity may
be more frequent than described previously, and it is a strong and independent prognostic
factor of overall survival. To our knowledge, this is the first single-centre study
confirming the prognostic importance of achieving therapeutic mitotane concentration.
