Diagnosis, treatment, and survival analysis of adrenocortical carcinomas: a multicentric study

Pedro Souteiro, Sara Donato, Cláudia Costa, Catarina A. Pereira, Joana Simões-Pereira, Joana Oliveira, Sandra Belo, Ana Paula Santos, Helena Cardoso, Valeriano Leite, Davide Carvalho, Isabel Torres

Research output: Contribution to journalArticlepeer-review

10 Citations (Scopus)


Introduction: Current guidelines specify controversial areas in adrenocortical carcinomas (ACC), such as optimal follow-up time after remission and identification of prognostic markers. We aim to address these topics by analyzing four reference centers in our country. Methods: Cross-sectional multicentric study of 69 patients (mean age: 51.7 ± 16.7 years-old; women, 72.5%). Kaplan-Meier survival curves and Cox regression analysis were used to calculate overall survival and its predictors. Results: Thirty-eight individuals (55.0%) had hormonal autonomous production, and 40.6% of the patients presented with metastasis. Surgery was performed in 84.1% of them. Most of these patients (72.4%) were then assigned to adjuvant therapy, while 27.6% were actively surveilled. Among patients undergoing surgery, those who achieved transient remission presented a longer survival time (66 months) than those who never reached the disease-free status (21 months) (p = 0.021). One patient presented with recurrence more than 7 years after complete tumor resection. The lowest overall survival was observed in patients (n = 11) assigned to palliative care since diagnosis (9 months). Tumor stage was identified as the only independent predictor of survival in our cohort (p = 0.006). Five-year survival was 67% for tumors confined to the adrenal space (stage I/II), 56% for locally advanced disease (stage III), and 0% for metastatic disease (stage IV). Conclusion: This study reinforces the dismal prognosis of ACC, the need for long-term follow-up, and tumor stage as the most important survival predictor. Reviewing medical records in such rare conditions is an opportunity to identify insufficiencies and to improve medical care.

Original languageEnglish
Pages (from-to)197-203
Issue number2
Early online date2019
Publication statusPublished - 1 Jun 2020


  • Adrenalectomy
  • Adrenocortical carcinoma
  • Mitotane


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