Browsing by Author "Parviainen, Helka"

Background: Adrenocortical carcinoma (ACC) is a rare endocrine carcinoma with poor 5-yr survival rates of <40%. According to the literature, ACC is rarely an incidental imaging finding. However, presentation, treatment and outcome may differ in modern series. Design and methods: We studied all patients (n=47, four children) from a single centre during years 2002 – 2018. We re-evaluated radiologic and histopathological findings and assessed treatments and outcome. We searched for possible TP53 gene defects and assessed nationwide incidence of ACC. Results: In adults, incidental radiologic finding led to diagnosis in 79% at median age of 61 yrs. ENSAT stage I, II, III and IV was 19%, 40%, 19% and 21%, respectively. Nonenhanced CT demonstrated > 20 Hounsfield Units (HU) for all tumours (median 34 (21-45)), median size 92 mm (20-196), Ki67 17% (1-40%), Weiss score 7 (4-9) and Helsinki score 24 (4-48). ACC was more often found in the left than the right adrenal (p< 0.05). One child had Beckwith-Wiedemann and one a TP53 mutation. In adults, the primary tumour was resected in 88% and 79% received adjuvant mitotane therapy. Median hospital stay was significantly shorter in the laparoscopic vs open surgery group (4 (3-7) vs 8 (5-38) days, respectively; p< 0.001). In 3/4 patients, prolonged remission of >5 to >10 years was achieved after repeated surgery of metastases. Overall 5-yr survival was 67%, and 96% vs 26% for ENSAT stage I-II vs III-IV (p< 0.0001). ENSAT stage and Ki67 predicted survival, type of surgery did not. Mitotane associated with better survival. Conclusions: Contemporary ACC predominantly presents as an incidental imaging finding, characterized by HU>20 on nonenhanced CT but variable tumour size (20-196 mm). Malignancy cannot be ruled out by small tumour size only. The 5-yr survival of 96% in ENSAT stage I-III compares favourably to previous studies