Surgical strategies for non-metastatic adrenocortical carcinoma

Adrenocortical carcinomas (ACC) are rare but highly aggressive tumors. It is very difficult to differentiate small locally limited ACCs from benign adenomas. A spontaneous density > 10 Hounsfield units in non-enhanced CT scan and a slow washout after contrast injection are suspicious of malignancy but with a low specificity. Preoperatively, a hormonal work-up is mandatory for all adrenal tumors. Each patient should be discussed in an interdisciplinary board. For non-metastatic ACCs (ENSAT stages I-III) radical resection is the treatment of choice. R0-resection and avoiding violation of the tumor capsule are the most important prognostic factors for long-term survival. Although discrepant reports regarding the benefits of lymphadenectomy have been published, lymph node dissection at least in the periadrenal area and in the renal hilum (optional extension to paraaortal and paracaval nodes) should be performed in the case of lymph node involvement. The role of prophylactic lymphadenectomy needs to be analyzed in further studies. The gold standard remains the open approach but minimally invasive procedures are also an option, especially in stage I-II tumors, if the principles of oncological surgery are respected. In this case, long-term survival rates are comparable. As local recurrence rates are lower and time to local recurrence is longer in patients who are operated on at a dedicated center (> 10 adrenalectomies/year), adrenalectomy for ACC should be performed by an experienced surgeon.