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Endocrine Abstracts (2013) 32 P510 | DOI: 10.1530/endoabs.32.P510

ECE2013 Poster Presentations Endocrine tumours and neoplasia (66 abstracts)

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

Thomas Kerkhofs 1 , Rob Verhoeven 2 , Jan-Maarten van der Zwan 3 , Jeanne Dieleman 1 , Michiel Kerstens 4 , Thera Links 4 , Lonneke van de Poll-Franse 2 & Harm Haak 1


1Máxima Medical Centre, Eindhoven/Veldhoven, The Netherlands; 2Comprehensive Cancer Centre South, Eindhoven, The Netherlands; 3Comprehensive Cancer Centre the Netherlands, Utrecht, The Netherlands; 4University Medical Centre Groningen, Groningen, The Netherlands.


Introduction: Adrenocortical carcinoma (ACC) has a reported annual incidence of 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 rate of ACC in the Netherlands. Secondary objectives were to evaluate changes in survival rates and in the number of patients undergoing surgery.

Methods: All ACC patients registered in the Netherlands Cancer Registry between 1993 and 2010 were included. Data on demographics, stage of disease, primary treatment modality and survival were used.

Results: Included were 359 patients, of whom 196 were female (55%). Median age at diagnosis was 56 (range 1–91) years. The 5-year age-standardized 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 was 159 months (95% CI 93–225 months), 26 months (95% CI 4–49 months) and 5 months (95% CI 2–8 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 increased significantly from 76% in 1993–1998 to 88% in 2005–2010 (P=0.047), mainly explained by an increase in surgery in stage III–IV patients.

Conclusion: These nationwide data provide an up to date description of ACC epidemiology 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.

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