Recommendation for standardized surgical management of primary adrenocortical carcinoma.

BACKGROUND: Operative resection is the only potentially curative treatment for primary adrenocortical carcinoma (ACC), but standards of operative care are not defined with regards to the extent of local resection. We propose recommendations for operative management.
METHODS: Anatomic and clinical literature review focusing on local management of ACC, including lymphadenectomy and resection of adjacent organs or large vessels.
RESULTS: First-order drainage nodes of the adrenal gland include the renal hilum lymph nodes, the celiac lymph nodes, and the para-aortic and paracaval lymph nodes, mainly above the renal pedicle and ipsilateral to the adrenal glands. Lymph node involvement occurs in about 20% of patients with ACC, and is an important prognostic factor, but lymphadenectomy is performed infrequently. The adrenal glands and kidneys are contained in the same anatomic space, but systematic en bloc nephrectomy has no proven benefits for survival. Direct invasion of the kidney or adjacent organs is rare, but major venous invasion with tumor thrombus is relatively common. Both are associated with decreased survival, but complete resection can lead to long-term survival.
CONCLUSION: Standardization of regional lymphadenectomy including first-order drainage nodes is proposed. Systematic nephrectomy is not necessary in the absence of gross local invasion, but locally involved organs or large veins should be resected en bloc, with tumor thrombus embolectomy, if R0 resection is possible. Operative standardization improves tumor staging, potentially decreases local recurrence, and may be associated with better survival. Evidence-based standards of operative care and prospective investigations within international collaborating groups are necessary.