Adrenocortical carcinoma is an uncommon cancer affecting 1 to 2 persons per 1 million persons.1It has been reported to have a bimodal age of distribution with the first peak occurring in the first decade of life and the second peak occurring between the fourth and fifth decades of life. These tumors are also more prevalent in women (58.6%) than men (41.1%).2,3Although adrenal carcinoma is potentially curable at early stages, only 30% of these malignancies are confined to the adrenal gland at the time of diagnosis.1For patients with localized malignancies, radical surgical excision is the treatment of choice and to date remains one of the only methods by which long-term disease-free survival may be achieved. Overall 5-year survival for tumors resected for cure is approximately 40%.
There are 2 important prognostic factors dictating long-term survival in patients with adrenocortical carcinoma: (1) completeness of resection, and (2) stage of disease. Patients without evidence of invasion into local tissues or spread to lymph nodes have an improved prognosis.4Just as in breast cancer, there has been a push to correlate DNA ploidy with outcome; however, current studies have not shown a correlation between aneuploidy and prognosis.4,5
Staging of adrenocortical carcinoma consists of determining the size of the primary tumor, the degree of local invasion, and spread to regional lymph nodes or distant sites.6Proper staging should include computed tomography of the abdomen.7- 10Magnetic resonance imaging may add specificity to computed tomographic evaluation of an adrenal mass.11Vena caval contrast studies and angiography may provide additional staging information and allow for a more complete preoperative assessment. More than 60% of patients present with disease greater than stage III at the time of diagnosis.3
Current management for stage I and stage II disease is complete surgical removal of the tumor. The long-term survival of patients with nonfunctioning tumors is comparable to that of patients with functioning tumors. The removal of regional lymph nodes that are not clinically enlarged is not indicated. Management for stage III disease is also complete surgical removal of the tumor, with or without regional lymph node dissection. For patients with enlarged regional lymph nodes, a lymph node dissection should be included with the surgery. Because these patients are at high risk for recurrent disease, they should be considered for enrollment in a clinical trial. To our knowledge, there are no published reports on the role of sentinel lymph node biopsy in the management of adrenocortical carcinoma to date. The management of stage IV disease involves temporary palliation with the chemotherapy agent mitotane. Prolonged treatment with mitotane, however, is often limited by gastrointestinal and neurologic toxicity. Local recurrences and selected sites of metastatic disease can sometimes be palliated surgically.12- 14
Due to the overwhelmingly positive response to the Image of the Month, the Archives of Surgery has temporarily discontinued accepting submissions for this feature. It is anticipated that requests for submissions will resume in mid 2008. Thank you.
Correspondence:Stacey D. Moore-Olufemi, MD, Department of Surgery, University of Texas Medical School at Houston, 6431 Fannin St, MSB 4.165, Houston, TX 77030 (firstname.lastname@example.org).
Accepted for Publication:May 16, 2006.
Author Contributions:Study concept and design: Moore-Olufemi and Vercruysse. Acquisition of data: Moore-Olufemi and Cheng. Analysis and interpretation of data: Moore-Olufemi. Drafting of the manuscript: Moore-Olufemi, Cheng, and Vercruysse. Critical revision of the manuscript for important intellectual content: Vercruysse. Administrative, technical, and material support: Moore-Olufemi, Cheng, and Vercruysse. Study supervision: Vercruysse.
Financial Disclosure:None reported.
Image of the Month—Diagnosis. Arch Surg. 2007;142(6):570. doi:10.1001/archsurg.142.6.570