Adrenocortical carcinoma: presentation and outcome of a contemporary patient series

Endocrine. 2019 Jul;65(1):166-174. doi: 10.1007/s12020-019-01918-9. Epub 2019 Apr 12.


Background: Adrenocortical carcinoma (ACC) is a rare endocrine carcinoma with poor 5-year 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 years. 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-year 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, characterised 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-year survival of 96% in ENSAT stage I-III compares favourably to previous studies.

Keywords: Adrenocortical carcinoma; Complications; ENSAT stage; Hounsfield units; Mitotane; Surgery; Survival.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adrenal Cortex Neoplasms* / diagnosis
  • Adrenal Cortex Neoplasms* / epidemiology
  • Adrenal Cortex Neoplasms* / genetics
  • Adrenal Cortex Neoplasms* / therapy
  • Adrenalectomy
  • Adrenocortical Carcinoma* / diagnosis
  • Adrenocortical Carcinoma* / epidemiology
  • Adrenocortical Carcinoma* / genetics
  • Adrenocortical Carcinoma* / therapy
  • Adult
  • Antineoplastic Agents, Hormonal / therapeutic use
  • Child
  • Child, Preschool
  • Cohort Studies
  • Combined Modality Therapy
  • DNA Mutational Analysis
  • Female
  • Follow-Up Studies
  • Genes, p53 / genetics
  • Humans
  • Infant
  • Male
  • Middle Aged
  • Neoplasm Recurrence, Local
  • Survival Rate
  • Treatment Outcome
  • Young Adult


  • Antineoplastic Agents, Hormonal