Adrenocortical carcinomas: Surgical trends and results of a 253-patient series from the French Association of Endocrine Surgeons study group

Because of the rarity of adrenocortical carcinoma, survival rates and the p rognosis for patients who have undergone operation are not well known. The purpose of the French Association of Endocrine Surgery was to evaluate thes e factors over an 18-year period. A trend study was associated to assess ch anges in the clinical and biochemical presentations as well as the surgical evolution. A total of 253 patients (158 women, 95 men) with a mean age of 47 years were included. Gushing syndrome was the main clinical presentation (30%), and hormonal studies revealed secreting tumors in 66% of the cases. Altogether, 72% (n = 182) of patients underwent resection for cure, and 41 .5% (n = 105) of them had an extensive resection because of metastatic canc er. A lymphadenectomy was performed in 32.5% (n = 89) of the cases. The ope rative mortality was 5.5% (n = 14). Patients were given mitotane as adjuvan t therapy in 53.8% of the cases (n = 135), The results of staging were stag e I in 16 patients (6.3%), stage II (local disease) in 126 patients (49.8%) , stage III (locoregional disease) in 57 patients (22.5%), and stage IV (me tastases) in 54 patients (21.3%), Neither tumor staging nor the rate of cur ative surgery changed during the study period. More subcostal incisions wer e performed, and the use of mitotane increased significantly. The 5-year ac tuarial survival rates were 38% overall, 50% in the curative group, 66% for stage I, 58% for stage II, 24% for stage III, and 0% for stage TV. Multiva riate analysis showed that mitotane benefited only the group of patients no t operated on for cure. A better prognosis was found in patients operated o n after 1988 (p = 0.04), in those with precursor-secreting tumors (p = 0.00 5), and in those at local stages of the disease (p = 0.0003), Thus mitotane benefited only patients not operated on for cure. Curative resection, prec ursor secretion, recent diagnosis, and local stage were favorably associate d with survival.