Management of adrenocortical carcinoma in Slovenia: A real-life analysis of histopathologic markers, treatment patterns, prognostic factors, and sur-vival

Management of adrenocortical carcinoma in Slovenia

Abstract

Background.

Adrenocortical carcinoma (ACC) is a rare cancer that presents significant diagnostic and therapeutic challenges. We analyzed the management and estimated survival of ACC patients in Slovenia over a 17-year period. 

Patients and methods.

Patients registered in the National Cancer Registry and treated from 2000 to 2017 were included. The survival and prognostic factors were assessed using the Kaplan-Meier method and Cox regression, respectively.  

Results.

48 patients, 34 of whom had inoperable, recurrent or primary metastatic disease, were included. At the time of diagnosis, 6%, 42%, 25% and 27 % were in ENSAT stages I, II, III and IV, respectively. Adjuvant treatment with mitotane was assigned to 18 of 34 potentially eligible patients. Half of those treated adjuvantly (or 9 patients) had ENSAT II, 38.9% (or 7) had III and 11.1% (or 2) had stage I. The median duration of adjuvant mitotane was 29.9 (3– 73) months. Six patients received adjuvant radiotherapy.  

Eleven patients underwent first-line therapy with etoposide, doxorubicin, cisplatin and mitotane (EDP-M). The median progression-free survival (mPFS) and the median overall survival (mOS) were 4.4 and 15.8 months, respectively. Nine patients continued with the second line treatment. The resulting mPFS and mOS were 2.3 months and 10.0 months, respectively. A third line therapy was prescribed to 4 patients. The mOS of the entire cohort was 31.2 months. The 5-year survival ratee for stage I/II and III/IV were 60.9 % and 24%, respectively. Only 12 of 35 operated patients were free of recurrences. The prognostic value of ENSAT stage and Helsinki score regarding survival was determined with the multivariate analysis.  

Conclusions.

The overall survival of our ACC patients was comparable to some other contemporary cohorts. A multidisciplinary approach with optimal usage of validated prognostic markers and adjuvant therapies might have led to improved outcomes.