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A 21 year old hypertensive woman was admitted because of a one month history of lower limb oedema. On examination, her blood pressure was 160/110 mm Hg and she had a tachycardia of 95 beats/min. She had hepatomegaly and a large palpable abdominal mass. Abdominal computed tomography (CT) (top) showed a retroperitoneal mass of 15 × 10 cm that was attached to the left kidney, with venous tumour thrombus extension in the inferior vena cava (IVC) up to the right atrium and involving both renal veins. Urgent echocardiography (bottom) showed complete filling of the right atrium with thrombus; blood flow was limited to a 2 mm channel along the interatrial septum. Chest radiography and abdominal CT showed liver and lung metastases. Steroid metabolism was not studied; urinary excretion of vanillymandelic acid and metanephrines had been normal six days previously.
Because of the intracardiac extension of the thrombus, cardiac bypass surgery was performed with en bloc extraction of the atrial and IVC tumour thrombus. The primary tumour, which was found to be of adrenal origin, was removed. Postoperatively, the patient developed an areflexic coma with extensive cerebral oedema related to cerebral metastases. She died some hours later. Histological analysis showed an adrenocortical carcinoma.