Surgery for adrenocortical carcinoma: When and how?

• Published in: Baillière's best practice & research. Clinical endocrinology & metabolism Vol. 34; no. 3; p. 101408
• Main Authors: Sinclair, Tiffany J., Gillis, Andrea, Alobuia, Wilson M., Wild, Hannah, Kebebew, Electron
• Format: Journal Article
• Language: English
• Published: Netherlands Elsevier Ltd 01.05.2020
• Subjects: adrenal cancer; Adrenal Cortex Neoplasms - diagnosis; Adrenal Cortex Neoplasms - pathology; Adrenal Cortex Neoplasms - surgery; adrenalectomy; Adrenalectomy - methods; Adrenalectomy - standards; Adrenocortical Carcinoma - diagnosis; Adrenocortical Carcinoma - pathology; Adrenocortical Carcinoma - surgery; CT scan; Humans; Liver Neoplasms - secondary; Magnetic Resonance Imaging; Neoplasm Metastasis; Neoplasm Staging; Positron Emission Tomography Computed Tomography; Prognosis; resectability; surgery; Time Factors; Tomography, X-Ray Computed; tumor margins; adrenalectomy; resectability; AOR; OS; adrenal cancer; CT scan; HU; tumor margins; LAD; surgery
• ISSN: 1521-690X; 1878-1594
• DOI: 10.1016/j.beem.2020.101408

Adrenocortical carcinoma (ACC) is a rare malignancy that is frequently asymptomatic at presentation, yet has a high rate of metastatic disease at the time of diagnosis. Prognosis is overall poor, particularly with cortisol-producing tumors. While the treatment of ACC is guided by stage of disease, complete surgical resection is the most important step in the management of patients with primary, recurrent, or metastatic ACC. Triphasic chest, abdomen, and pelvis computer tomography (CT) scans and 18F flourodeoxyglucose positron emission tomography CT scanning are essential for accurate staging; moreover, MRI may be helpful to identify liver metastasis and evaluate the involvement of adjacent organs for operative planning. Surgical resection with negative margins is the single most important prognostic factor for survival in patients with ACC. To achieve the highest rate of R0 resection, open adrenalectomy is the gold standard surgical approach for confirmed or highly suspected ACC. It is extremely important that the tumor capsule is not ruptured, regardless of the surgical approach used. The best post-operative outcomes (complications and oncologic) are achieved by high-volume surgeons practicing at high-volume centers.