Adrenocortical carcinoma - Clinical and laboratory observations

BACKGROUND. The clinical features and natural history of adrenocortical car cinoma are highly dependent on the type of center reporting their experienc e. Observations from oncology services suggest a high incidence of nonfunct ioning tumors, whereas reports from endocrine clinics emphasize excessive c orticoid and androgen production in the majority of tumors. The incidence r ate and natural history of childhood adrenal carcinoma generally has been u nder emphasized. METHODS. Over the past 17 years, the authors have evaluated and treated 47 patients with adrenocortical carcinoma referred to the University of Sao Pa ulo, 22 of whom were children. RESULTS. There is a bimodal age incidence of adrenal carcinoma, with the di sease peaking in the first and fourth decades of life. Childhood adrenal ca rcinoma is characterized by a high rate of incidence of virilization, marke d overproduction of androgens, and a less aggressive clinical course, and a ppears to be more amenable to surgical and other therapeutic modalities. By contrast, adrenocortical carcinoma occurring in adults presents more commo nly as a mixed Gushing and virilizing syndrome, with overproduction of cort icoids and androgens and a far more aggressive clinical course, leading to rapid death within months or years. Nonfunctioning adrenocortical carcinoma is less common; it generally occurs in older adults and exhibits a rapid d ownhill course. Modern day imaging methods have improved the diagnosis and staging of adrenal carcinoma greatly. In the authors' experience, the histo logic criteria of Weiss appeared to predict tumor prognosis most accurately , whereas immunologic markers, cytoskeletal markers, DNA ploidy, cell phase markers, and oncogenic probes have yielded inconsistent results to date. S urgical removal of a localized tumor remains the best hope for long term su rvival. Medical therapy with mitotane and its successors in patients with S tage III or TV (MacFarlane system as modified by Sullivan et al.) disease a ppear to have added little to longevity or quality of life. CONCLUSIONS, When diagnosed in children, adrenal carcinoma is associated wi th virilism and a less aggressive natural history; however, when it occurs in adults, the disease presents more commonly as a mixed Cushing-virilizing syndrome and has a virulent course. The Weiss histologic criteria appear t o correlate best with disease prognosis, but other histochemical, cell cycl e, and genetic markers have not, to date, aided in disease management. (C) 2000 American Cancer Society.