Natural history of adrenal cortical carcinoma: a clinicopathologic study of 42 patients.

To study the biologic behavior and natural history of this rare but challenging tumor faced by oncologists, a clinicopathologic study of 42 patients with histologically proven adrenal cortical carcinoma from Roswell Park Memorial Institute (1929--1977) was done. These constituted .04% of all cancer cases and 0.2% of all autopsy cases. Age range was 3--74 years with median of 53 years; female to male ratio was 1.5 to 1. Clinical manifestations were: abdominal mass (36%), metastatic disease (30%), hormonal excess (17%) and weakness with lethargy (17%). Nine of ten functioning tumors were seen in female patients. Tumors arose in left adrenal in 26 patients, right adrenal in 12, and in four the site could not be determined because of bilateral presence of cancer. Median duration of symptoms was six months. At diagnosis, 52% had distant metastases, 41% had locally advanced tumor and 7% had tumor confined to adrenal. Sixteen patients underwent "curative" resection. Tumor diameter ranged from 1--30 cm with median of 10 cm. Of 28 patients who received different chemotherapeutic regimens, three (11%) had objective response; four of ten patients had objective response to radiation therapy. Overall median and five-year survival rates were 14 months and 24%. Prolonged survival (P less than .05) was noted in women, patients who had "curative" resection, a disease-free interval of more than 12 months, and tumor size greater than 10 cm diameter. Patients with functional tumors had longer median survival than those with non-functional ones (28 vs. 12), but P value was greater than .05. A second primary cancer was noted in 22.4% of cases, breast and lymphoma being the most common. At autopsy in 31 patients, the most common metastatic sites were retroperitoneal lymph nodes 68%, lung 71%, liver 42%, and bone 26%. To improve survival, an aggressive surgical approach is recommended to extirpate the tumor with involved organs and retroperitoneal lymph nodes. Adrenal carcinoma should be suspected in patients with metastatic cancer with an occult primary.