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Management of Adrenocortical Carcinoma

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Prof Gary D Hammer, USA    20 November 2018

  1. Adrenal-focused imaging is recommended in all patients with suspected adrenocortical carcinoma (ACC).
  2. Histopathologic diagnosis and grading – Use of Weiss system is recommended. Ki67 immunohistochemistry is recommended for every adrenocortical tumor.
  3. In patients with advanced ACC at the time of diagnosis not qualifying for local treatment, either mitotane monotherapy or mitotane + EDP is recommended depending on prognostic parameters.
  4. Owing to the high rate of recurrence and metastatic disease, treatment often relies on systemic therapies.
  5. Cytotoxic therapy in unresectable ACC - EDP-M is recommended as first-line treatment for recurrence <6 months, rather than repeat loco-regional measures. In patients who progress under mitotane, addition of EDP is recommended.
  6. It is recommended that adrenal surgery for suspected/confirmed ACC should be performed only by surgeon experienced in adrenal and oncological surgery.
  7. Complete en bloc resection is recommended.
  8. Open surgery is the standard surgical approach for confirmed or highly suspected ACC.
  9. It is suggested that routine loco-regional lymphadenectomy should be performed.
  10. For adrenal tumors with uncertain malignant potential, adjuvant therapy is not recommended.
  11. There is a suggestion for adjuvant mitotane treatment without macroscopic residual tumor but with high risk of recurrence.
  12. Radiation can be considered in addition to mitotane therapy on an individualized basis therapy in patients with R1 or Rx resection or in stage III.
  13. Adjuvant chemotherapy can be considered in selected patients with very high risk for recurrence.

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