National Treatment Practice for Adrenocortical Carcinoma: Have They Changed and Have We Made Any Progress?

J Clin Endocrinol Metab. 2019 Dec 1;104(12):5948-5956. doi: 10.1210/jc.2019-00915.


Background: Adrenocortical carcinoma (ACC) is a rare malignancy with a dismal prognosis. Two landmark trials published in 2007 and 2012 showed efficacy for adjuvant mitotane in resectable ACC and etoposide/doxorubicin/cisplatin plus mitotane for unresectable ACC, respectively. In this study, we used the National Cancer Database to examine whether treatment patterns and outcomes changed after these trials.

Methods: The National Cancer Database was used to examine treatment patterns and survival in patients diagnosed with ACC from 2006 to 2015. Treatment modalities were compared within that group and with a historical cohort (1985 to 2005). χ2 tests were performed, and Cox proportional hazards models were created.

Results: From 2006 to 2015, 2752 patients were included; 38% of patients (1042) underwent surgery alone, and 31% (859) underwent surgery with adjuvant therapy. Overall 5-year survival rates for all stages after resection were 43% (median, 41 months) in the contemporary cohort and 39% (median, 32 months) in the historical cohort. After 2007, patients who underwent surgery were more likely to receive adjuvant chemotherapy (P = 0.005), and 5-year survival with adjuvant chemotherapy improved (41% vs 25%; P = 0.02). However, survival did not improve in patients with unresectable tumors after 2011 compared with 2006 to 2011 (P = 0.79). Older age, tumor size ≥10 cm, distant metastases, and positive margins were associated with lower survival after resection (hazard ratio range: 1.39 to 3.09; P < 0.03).

Conclusions: Since 2007, adjuvant therapy has been used more frequently in patients with resected ACC, and survival for these patients has improved but remains low. More effective systemic therapies for patients with ACC, especially those in advanced stages, are desperately needed.

Publication types

  • Evaluation Study

MeSH terms

  • Adrenal Cortex Neoplasms / mortality*
  • Adrenal Cortex Neoplasms / therapy
  • Adrenalectomy / mortality*
  • Adrenocortical Carcinoma / mortality*
  • Adrenocortical Carcinoma / therapy
  • Aged
  • Antineoplastic Agents / therapeutic use*
  • Antineoplastic Combined Chemotherapy Protocols
  • Chemotherapy, Adjuvant / mortality*
  • Combined Modality Therapy
  • Databases, Factual
  • Disease-Free Survival
  • Female
  • Humans
  • Male
  • Middle Aged
  • Mitotane / therapeutic use
  • Prognosis
  • Treatment Outcome


  • Antineoplastic Agents
  • Mitotane