Clinical features and prognostic factors associated with adrenocortical carcinoma: Lahey Clinic Medical Center experience

Adrenocortical carcinoma is a rare tumor associated with a commonly poor pr ognosis. However, data on the natural history and response to therapy of pa tients with this malignancy have often been conflicting. Our objective of t his retrospective study was to evaluate the clinical course and survival of patients with adrenocortical carcinoma and to identify relevant prognostic factors. Between 1966 and 1996, 31 patients with histologically documented adrenocortical carcinoma were observed at the Lahey Clinic Medical Center. Patient information was obtained from chart review. At the time of diagnos is, 48 per cent of patients had endocrine symptoms with compatible hormonal studies,19 per cent had involvement of the inferior vena cava by tumor thr ombus, and 32 per cent had metastatic disease. The median survival time was 17 months (range, 1-205 months) for the entire group, and the 5-year survi val rate was 26 per cent. Age <54 years, absence of metastatic disease at t he time of diagnosis, and completeness of surgical resection were associate d with better prognosis. Evaluation of survival with the Cox proportional h azards model suggested that age <54 years, absence of metastatic disease, a nd nonfunctioning tumor status were independently associated with improved survival. The prognosis of patients with adrenocortical carcinoma is poor b ut appears more favorable in patients <54 years, with localized disease, or nonfunctioning tumor status. Complete tumor resection may be associated wi th improved survival.