Strategic surgical planning for resection of a large adrenal cortical carcinoma

Mark Fairweather, Brenessa Lindeman, Tingsong Yang, Jiping Wang, Gerard Doherty


Adrenal cortical carcinoma (ACC) is a rare malignancy with an annual incidence of approximately 0.7 cases per million in the United States (1). While these tumors can develop at any age, there is a recognized bimodal age distribution for ACC with the first peak occurring before age 5 years and the second peak in the 4th–5th decade of life (2). Despite accounting for only 0.2% of cancer-related deaths per year in the United States, ACC has a very poor prognosis (3). Over half of patients present with advanced disease and overall survival remains poor even with surgical resection (4,5). Management of ACC requires a multidisciplinary treatment approach. Surgical resection offers the only potentially curative treatment option even though recurrences will still occur in up to 70–80% (6,7). Age older than 55 years, positive resection margin, lymph node positive disease, resection of a contiguous organ, poorly-differentiated tumors, and distant metastatic disease are independent predictors of worse prognosis in patients who undergo surgical resection (4). Due to the proximity of the adrenal gland to surrounding anatomic structures, the need for combined resection ACC with liver, pancreas, kidney are common. Hence, surgical resection of ACC carries significant mortality and morbidity.