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. 2013 Oct 23;169(6):891-899.
doi: 10.1530/EJE-13-0519. Print 2013 Dec.

Adrenocortical carcinoma: clinical outcomes and prognosis of 330 patients at a tertiary care center

Montserrat Ayala-Ramirez #  1 , Sina Jasim #  2 , Lei Feng  3 , Shamim Ejaz  1 , Ferhat Deniz  1 , Naifa Busaidy  1 , Steven G Waguespack  1 , Aung Naing  4 , Kanishka Sircar  5 , Christopher G Wood  6 , Lance Pagliaro  7 , Camilo Jimenez  1 , Rena Vassilopoulou-Sellin  1 , Mouhammed Amir Habra  1
Affiliations

Adrenocortical carcinoma: clinical outcomes and prognosis of 330 patients at a tertiary care center

Montserrat Ayala-Ramirez et al. Eur J Endocrinol. .

Abstract

Objective: Adrenocortical carcinoma (ACC) is a rare malignancy with a poor prognosis. Herein, we describe the clinical features and outcomes for a large series of ACC patients.

Design and methods: Retrospective review of ACC patients seen at The University of Texas MD Anderson Cancer Center from 1998 through 2011.

Results: A total of 330 patients with median age at diagnosis of 48.5 years; 12 (3.6%) patients were under 18 years. Hormonally functioning tumors represented 41.8% (n=138) of all cases. Surgical resection for the primary tumor was done in 275 (83.3%) patients (45 at MD Anderson (16.4%)). For those who had surgical resection, the median local-recurrence-free time was 1.04 years. Factors associated with local recurrence included positive surgical margins (P=0.007) and advanced disease stage (P=0.026). Median overall survival time for all patients was 3.21 years. Median survival times were 24.1, 6.08, 3.47, and 0.89 years for stages I, II, III, and IV respectively. In multivariable analysis, older age, functioning tumors, and higher disease stage remained significant prognostic factors associated with poor survival.

Conclusion: ACC prognosis remains poor with the use of currently available treatments. Older age, functioning tumors, and incomplete resections are clinical factors associated with poor survival. Surgical expertise is important to achieve complete resections and to improve outcome.

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Figures

Figure 1
Figure 1. Factors associated with the overall survival of patients with adrenocortical carcinoma
R0, no evidence of tumor; R1, microscopically positive resection margins; R2, macroscopic residual disease ENSAT, European Network for the Study of Adrenal Tumors; VTE, Venous Thromboembolism
Figure 2
Figure 2. A Suggested algorithm for the management of localized adrenocortical carcinoma
R0, no evidence of tumor; R1, microscopic evidence of tumor; R2, macroscopic residual disease; RX, margins unknown. We recommend mitotane for 2-3 years.
Figure 3
Figure 3
An Algorithm for the management of locally advanced/metastatic adrenocortical carcinoma
Figure 4
Figure 4. Overall survival (OS) in patients who achieve mitotane levels of 14 mg/l or higher
(YES, red line) compared with the patients with lower levels (NO, green line).
See this image and copyright information in PMC

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