INVITED COMMENTARY
Adrenocortical carcinoma (ACC) is a rare but aggressive tumor with a propensity for disease recurrence and metastasis. Unfortunately there is no effective systemic treatment; therefore patients with metastatic disease or incomplete resections have limited treatment options. One of the most common metastatic sites in ACC is the lung, but the management of this group of patients is still controversial given the rarity of the disease and the propensity for disease relapse. Despite small series ob- serving cases of prolonged survival after surgical man- agement of metastatic ACC, a focused review of out- comes after pulmonary metastasectomy in ACC disease has not been available to date.
In this study, Kemp and colleagues [1] describe the largest and most comprehensive series to date of patients who underwent pulmonary metastasectomy for ACC. This study reports the outcome of 26 patients who un- derwent a total of 60 pulmonary metastasectomies over a 30-year period. Twenty-three (88%) patients were ren- dered free of disease in the lungs after the intended pulmonary resections, but only 14 (54%) were rendered completely free of disease. Furthermore, the median disease-free survival was only 6 months for those pa- tients rendered completely free of disease.
However despite the increased incidence of disease recurrence and persistent disease after metastasectomy, the median overall and 5-year actuarial survivals from initial pulmonary metastasectomy were 40 months and 41%, respectively. These are quite impressive numbers for an aggressive disease treated with ineffective systemic therapy. This increased survival may be due in part to aggressive reoperations in patients with pulmonary re- lapse, with 10 (38%) patients undergoing 28 additional thoracotomies. These patients in particular had the best survival, with a 5-year survival of 57% after repeated
resection. This may also be due to more favorable bio- logical characteristics in a very select group of patients with disease relapse only in the lungs, which was ame- nable to resection. Of the prognostic variables for a favorable outcome, only longer disease-free interval after adrenalectomy and a lower T stage of the primary adre- nal tumor were associated with prolonged survival after metastasectomy.
This study is a significant contribution to the thoracic literature and will serve as a benchmark to compare further outcomes in the management of these complex and infrequent patients. Despite the study limitations, it suggests an aggressive surgical approach for patients with ACC and pulmonary metastases, even if it re- quires multiple operations to achieve or maintain disease-free status. Careful patient selection and as- sessment of the disease extent and biological charac- teristics are paramount in order to achieve the best results. More effective systemic treatments may further improve the outcomes of pulmonary metastasectomy for ACC.
Luis J. Herrera, MD
Division of Thoracic Surgery MD Anderson Cancer Center Orlando 1400 S Orange Ave, MP-760 Orlando, FL 32806 e-mail: luis.herrera@orhs.org
Reference
1. Kemp CD, Ripley RT, Mathur A, et al. Pulmonary resection for metastatic adrenocortical carcinoma: the National Cancer Institute experience. Ann Thorac Surg 2011;92:1195-201.