Adrenocortical carcinoma: A population-based study on incidence and survival in the Netherlands since 1993

T.M. Kerkhofs, R.H.A. Verhoeven, J.M. van der Zwan, J. Dieleman, M.N. Kerstens, T.P. Links, L.V. van de Poll-Franse, H.R. Haak

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231 Citations (Scopus)


The reported annual incidence of adrenocortical carcinoma (ACC) is 0.5–2.0 cases per million individuals. Updated population-based studies on incidence are lacking. The aim of this nationwide survey was to describe the incidence and survival rates of ACC in the Netherlands. Secondary objectives were to evaluate changes in both survival rates and the number of patients undergoing surgery.
All ACC patients registered in the Netherlands Cancer Registry (NCR) between 1993 and 2010 were included. Data on demographics, stage of disease, primary treatment modality and survival were evaluated.
Included were 359 patients, 196 of whom were female (55%). Median age at diagnosis was 56 years (range 1–91). The 5-year age-standardised incidence rate decreased from 1.3 to 1.0 per one million person-years. Median survival for patients with stage I–II, stage III and stage IV disease was 159 months (95% confidence interval (CI) 93–225 months), 26 months (95% CI: 4–48 months) and 5 months (95% CI: 2–7 months), respectively (P < 0.001). Improvement in survival was not observed, as reflected by the lack of association between survival and time of diagnosis. The percentage of patients receiving treatment within 6 months after diagnosis increased significantly from 76% in 1993–1998 to 88% in 2005–2010 (P = 0.047), mainly due to an increase in surgery for stage III–IV patients.
These nationwide data provide an up-to-date survey of the epidemiology of ACC in the Netherlands. A trend towards a decreasing overall incidence rate was observed. Survival rates did not change during this period despite an increased number of surgical procedures.Keywords: Adrenocortical carcinoma, Incidence, Cancer registry,
Original languageEnglish
Pages (from-to)2579-2586
JournalEuropean Journal of Cancer: Official journal for European Organization for Research and Treatment of Cancer (EORTC)
Issue number11
Publication statusPublished - 2013


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