Pheochromocytoma rarely extends locally into the vena cava or the right atrium. We report a case of malignant pheochromocytoma with growth into the inferior vena cava, extending into the right atrium, address clinical aspects of this tumour and review the literature on this malignancy. Pre-operative work-up of this tumour should include measurements of urinary vanillyl mandelic acid and cathecholamine excretion, MRI and spiral CT of the abdomen and thorax. After the diagnosis is made the patient should be treated with catecholamine alpha-receptor blockade and if necessary with subsequent beta-receptor blockade. An aggressive surgical approach is always warranted, even in cases with very large localized tumours, because surgery has been shown to lead to relief of symptoms and to prolong survival in cases otherwise deemed irresectable. The optimal surgical exposure is obtained via a transsternal midline thoraco-laparotomy. If feasible, a combination of cardiopulmonary bypass, hypothermia, cardiac arrest and exsanguination procedures should be used. In case of local of tumour remnants after surgery or distant metastases treatment options are secondary surgery, tumour embolization, or treatment with radioactive labelled drugs, including(131)I-MIBG.
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