Adrenocortical Carcinoma Treatment in the Netherlands: An Analysis From the Netherlands Cancer Registry From 2014 to 2019

Background: Adrenocortical carcinoma (ACC) is a rare disease with often poor prognosis. Previous research has shown that surgery in a Dutch Adrenal Network (DAN) center increases the chance of survival. We aim to explore the determinants and survival of patients with ACC recently treated in the Netherlands both within and outside DAN centers. Methods: We analyzed retrospectively collected data from 172 adult patients with newly diagnosed ACC and 97 patients with recurrence or new metastases, registered between 2014 and 2019 in the Netherlands Cancer Registry. Differences in survival were analyzed with cox-regression analysis. Results: More than half of the new cases presented with advance disease (25.7% stage III, 34.6% stage IV) and the median survival was 29 months. The majority of treatments occurred within a DAN center (87.2% of surgery, compared to 56.4% between 1999 and 2009; and 94.5% of medical treatment). There were no differences in patient characteristics between the centers apart from a relatively high number of patients with stage IV disease outside DAN centers (47.2% vs. 28.7%). Adrenal resection and mitotane therapy both resulted in a significant survival benefit (resection HR 0.29, CI95%[0.17–0.49]; mitotane HR 0.61, CI95%[0.37–0.99], corrected for stage). Still, a remarkable proportion of patients with advanced disease received no mitotane treatment (39.8%). Due to the small number of patients treated outside DAN centers, survival benefits could not be tested. Conclusions: Centralization of ACC care in the Netherlands has improved since the previous report, but a further improvement in centralization of surgery can be made. Adrenal resection and mitotane treatment remain the main form of treatment, with a clear survival benefit. Further research is necessary to determine why mitotane treatment is withheld in a large proportion of patients with advance disease.