Adrenocortical carcinoma. Clinical outcome at the end of the 20th century.

BACKGROUND: Adrenocortical carcinoma remains a rare and lethal neoplasm. Effective therapies have not emerged in recent decades. However, medical advances have improved diagnostic techniques and supportive measures; these changes may have a beneficial impact on the natural history of the disease.

METHODS: The authors retrospectively analyzed the clinical outcomes of patients with adrenocortical carcinoma registered at the University of Texas M.D. Anderson Cancer Center focusing on patients who received their diagnosis since 1980 and comparing data from those patients with earlier reports.

RESULTS: Since 1980, 139 patients have registered at M.D. Anderson Cancer Center with the diagnosis of adrenocortical carcinoma. One-third had evidence of hormone hypersecretion, and one-third had localized disease at diagnosis. Men were affected as frequently as women but tended to be older and have larger tumors at diagnosis. The 5-year survival rate was 60% (Kaplan-Meier analysis). The 30 patients with the longest survival (> 5 years) and the 30 patients with the shortest survival (< 11 months) had no significant differences in age, gender, tumor size, or functionality. However, long-term survivors had significantly less extensive disease. A comparison with patients reviewed in earlier reports from the same institution showed no significant differences in gender predilection, tumor function, or extent of disease. Despite these similarities, patients whose disease was diagnosed since 1980 lived much longer than patients observed in earlier decades.

CONCLUSIONS: Despite the lack of significant improvements in early diagnosis and effective therapies, patients with adrenocortical carcinoma are living longer (5-year survival rate, 60%). It is important to revise assumptions regarding the clinical outcomes of patients with this disease.