[Adrenal cortex carcinoma with right atrium involvement. Surgery with cardiopulmonary bypass]

Primary adrenal carcinoma is a low incidence tumour. About 50% are functional and the majority result in Cushing's syndrome, while detection of the rest is commonly incidental. Surgical management is through radical surgery.

A 36-year old female who presented with a one-month old abdominal pain in the hypochondrium and the right lumbar fossa. Physical examination found a discretely painful mobile mass. Adrenal hormones values were normal. Ultrasound and CT studies detected a 14-cm adrenal tumour with cava vein thrombosis up to the right auricle. The surgical procedure included laparotomy, liver mobilisation and cardiopulmonary by-pass. The thrombus was completely removed by auriculotomy. Therapy with mitotane and 5-fluorouracil was then instituted. Thirteen months after surgery the patient is asymptomatic and tumour free.

Dissemination of adrenal tumours to veins ranges between 6 to 30%. Clinical manifestation of cava vein and auricle involvement is variable but it may develop with no symptoms due to collateral circulation. Pre-operative diagnosis is critical to plan for adequate surgical approach. Ultrasound and computerised tomography can adequately diagnose the process, but nuclear magnetic resonance provides more information on the thrombus extension and location. In the absence of node spread, local extension, or invasion of the cava wall prognosis is similar to that of patients with no vein involvement. The best therapeutic option is surgery with removal of the lesion and cardiopulmonary by-pass. Survival at 5 years is 43%. Post-operative administration of chemotherapy with mitotane is a useful and recommendable choice.