Adjuvant therapies and patient and tumor characteristics associated with survival of adult patients with adrenocortical carcinoma

J Clin Endocrinol Metab. 2014 Feb;99(2):455-61. doi: 10.1210/jc.2013-2856. Epub 2013 Dec 3.


Context: Adrenocortical carcinoma is a rare malignant endocrine neoplasia. Studies regarding outcome and prognostic factors rely on fairly small studies. Here we summarize the experience with patients with a diagnosis of adrenocortical carcinoma from a large tertiary referral center.

Objective: The objective of the study was to identify prognostic factors in patients with adrenocortical carcinoma and evaluate adjuvant treatment strategies.

Design: Patient data were collected in a retrospective single-center study. Epidemiological, patient, and tumor characteristics were analyzed for prognostic factors regarding overall and recurrence-free survival in Cox regression models (multivariable and univariable).

Results: Three hundred ninety-one adult patients with the diagnosis of adrenocortical carcinoma were identified. Median overall survival was 35.2 months. Cortisol production [hazard ratio (HR) 1.4, HR 1.5], tumor stage (HR stage 3 of 2.1 and 2.1, HR stage 4 of 4.8), and tumor grade (HR 2.4 and 2.0) were identified as negative prognostic factors (HR for death, HR for recurrence). Mitotane therapy increases recurrence-free survival, an effect that was significantly further improved by adjuvant radiation therapy but did not impact overall survival. Patients with open adrenalectomy had improved overall survival.

Conclusions: This study increases the evidence for adverse risk factors (cortisol production, high tumor stage, and high tumor grade) and suggests the following therapy approach: adrenocortical carcinoma patients should be treated with open adrenalectomy. Adjuvant therapy, particularly mitotane therapy in conjunction with radiation, should be considered to delay tumor recurrence.

Publication types

  • Research Support, N.I.H., Extramural

MeSH terms

  • Adolescent
  • Adrenal Cortex Neoplasms / mortality*
  • Adrenal Cortex Neoplasms / pathology
  • Adrenal Cortex Neoplasms / therapy*
  • Adrenalectomy
  • Adrenocortical Carcinoma / mortality*
  • Adrenocortical Carcinoma / pathology
  • Adrenocortical Carcinoma / therapy*
  • Adult
  • Aged
  • Aged, 80 and over
  • Antineoplastic Agents, Hormonal / therapeutic use
  • Chemotherapy, Adjuvant
  • Combined Modality Therapy
  • Female
  • Humans
  • Male
  • Middle Aged
  • Mitotane / therapeutic use
  • Neoplasm Recurrence, Local / mortality*
  • Neoplasm Recurrence, Local / pathology
  • Neoplasm Recurrence, Local / prevention & control
  • Prognosis
  • Retrospective Studies
  • Risk Factors
  • Survival Rate
  • Treatment Outcome


  • Antineoplastic Agents, Hormonal
  • Mitotane