Adrenocortical carcinoma with intracaval extension

We describe the presenting features, treatment approach and prognosis of adrenocortical carcinoma with intracaval extension of tumor thrombus.

In addition to 3 patients with adrenocortical carcinoma associated tumor thrombus treated at our institution, we reviewed an additional 26 patients described in the literature from 1972 to 1997 with regard to presentation, management and outcome.

We identified 23 female and 6 male patients 6 to 77 years old (mean age 41.3). Of the lesions 24 originated in the right adrenal gland. Mean tumor size was 10.1 cm. and 89% of lesions were at least 9 cm. in greatest dimension. Tumor thrombus extended to the atrium in 15 patients, retrohepatic cava in 7 and subhepatic cava in 7. Flank or abdominal discomfort was the most common presenting complaint and abnormal steroid metabolism was documented in 76% of patients. Cardiac bypass techniques were used in 14 patients and none of the 3 intraoperative mortalities, 2 thromboemboli and 1 exsanguination, occurred using this approach. Eight patients received postoperative mitotane, 6 of whom had no evidence of residual disease at the time of case description.

All patients with large adrenal tumors, especially those arising from the right gland, should undergo careful evaluation of the vena cava for thrombus. The best chance for survival is via complete surgical extirpation which is facilitated by the use of cardiac bypass techniques. There is evidence to support the early use of postoperative mitotane if there is a suspicion of residual or recurrent disease.