Adrenocortical carcinoma. Our experience.

Adrenocortical carcinoma represents only 0.2% of the causes of deaths from cancer and this extremely low incidence rate implies that few medical centers have sufficient experiences to permit logical conclusions to be drawn regarding its treatment. From 1979 to 1993 we operated on 215 patients for adrenal diseases. The male/female ratio was 1:2.5 and the median age was 47 years (range 13-70). Eighty patients presented a clinical picture of hypercortisolism, 66 had a Conn's syndrome and 38 a pheochromocytoma; 30 had a nonfunctioning cortical or medullary tumor and one a metastatic adrenal mass. Of 141 patients with cortical tumors, 19 (13.5%) had malignancy of which 9 (7 males and one female, mean age 62 years) were nonfunctioning and 10 (3 males and 7 females, mean age 43 years, range 15-65) associated with a Cushing's syndrome. Seventeen patients underwent surgery, and two had multiple CT-FNAB-confirmed liver metastases and received o,p'DDD and chemotherapy treatment only. Tumor staging was the following: 3 (16%) stage I patients (tumor size varying from 4 to 5 cm), 7 (37%) stage II patients (tumor size 6-20 cm, mean 12 cm) and 9 (47%) stage III or IV. In 14 of the 17 operated patients the surgical approach was a lateral extraperitoneal incision with 12th rib partial resection. One patient had postoperative hemorrhage and reoperation was necessary. Operative mortality related to adrenalectomy was 0% although in three cases a nephrectomy was necessary because the mass completely surrounded the kidney. Three patients had a second operation 21-48 months after adrenalectomy for local recurrence of the disease. Overall survival time ranged from 2 to 42 months, the one-year survival rate was less than 40% and only six patients are still alive, 2-18 months after surgery. Tumor size and its endocrine functionality did not seem to correlate with prognosis in our experience.(ABSTRACT TRUNCATED AT 250 WORDS)