Background. Adrenocortical carcinoma is a rare tumor with a poor progn
osis. This work was aimed at analyzing the clinical outlook and treatm
ent results of 52 patients with this disease. Methods. This study incl
uded patients with adrenocortical carcinoma referred to the Department
of Endocrinology at the Center of Postgraduate Medical Education (War
saw, Poland) during the last 30 years. In 11 patients, the adrenal tum
or was found incidentally by ultrasonographic scan. Hormonal examinati
ons made it possible to define the endocrine activity of the tumors, w
hereas imaging techniques helped to determine their staging. Forty-eig
ht patients underwent surgery, and 36 of them received mitotane. This
drug was administered to 26 patients for a range of 10 months to 10 ye
ars; 13 patients received mitotane immediately after the operation, an
d 13 others after a delay. The patients with severe hypercorticism wer
e pretreated before surgery with aminoglutethimide and mitotane. Resul
ts. The study comprised 10 men and 42 women; hormonally active tumors
were diagnosed in 39 of them. Gushing's syndrome was the most frequent
entity. At diagnosis, 17 cases were classified as localized disease,
15 as regional disease, and 20 as distant disease. Pretreatment with t
he inhibitors of steroidogenesis improved the survival perspectives in
the early postoperative period. As of this writing, there were 12 sur
vivors in the group of 26 patients treated by surgery and long term mi
totane therapy and only 2 survivors of 7 patients treated with surgery
only. Conclusions. Surgery with immediate adjuvant long term mitotane
administration was the most effective form of therapy for patients wi
th adrenocortical carcinoma.