THE ITALIAN REGISTRY FOR ADRENAL-CORTICAL CARCINOMA - ANALYSIS OF A MULTIINSTITUTIONAL SERIES OF 129 PATIENTS

Background. Adrenal cortical carcinoma is an uncommon tumor with a poo r prognosis, The low incidence of this tumor makes it difficult to ach ieve reliable data on clinical manifestations, natural history, and th e impact of therapies. The purpose of this study was to evaluate such aspects in a large series. Methods, A retrospective series of 129 case s (55 men and 74 women, mean age of 49 years) was collected from 18 su rgical institutions. At the time of diagnosis 45.7% of patients had en docrine symptoms. One hundred twenty-four patients underwent surgery, which was considered curative in 91 cases and palliative in 33. Sixty- three patients had local disease 48 had regional disease, and 43 had d istant metastases. Results. This study confirmed a higher incidence in the 40- to 50-year-old population with a female prevalence hormonal h yperincretion was more common in women, but it was not caused by advan ced disease, The overall 5-year survival rate was 35%. Tumor stage and curative resection affected prognosis significantly, The influence of gender, side, age, and hormonal function has not been confirmed. Adju vant therapies were ineffective in prolonging survival. Reoperated pat ients experienced better survival (mean, 41.5 months) than nonreoperat ed cases (mean, 15.6 months). Conclusions, The poor prognosis of adren al cortical carcinoma may be improved by early diagnosis and complete resection. Radical surgery is the sole effective therapy, particularly in early stages. Surgical treatment of recurrence seems to improve su rvival and should be attempted systematically. Adjuvant therapies obta ined contrasting results, and their role should be evaluated in prospe ctive multicentric trials.