[An autopsy case of extra-adrenal malignant pheochromocytoma]

An autopsy case of an extra-adrenal malignant pheochromocytoma in a 34-year-old woman is reported. On laparotomy, many advanced stage malignant tumors originating from the paraganglia along the abdominal aorta were found to have invaded the lumbar vertebrae. After a partial resection, Co60 radiation therapy of the paraganglia was instituted, as well as of the metastatic lesions, with little effect. It was found that alpha-methyl-tyrosine was effective in controlling the plasma catecholamine, but had to be discontinued because of an untoward effect (anxiety). The patient subsequently developed intractable hypertension and a paralytic ileus from excess catecholamine secretion. As an alpha 1 adrenergic blocker was not effective, we had to use large doses of phentolamine to control these complications. Despite various intensive therapies, however, the patient died of heart failure resulting from 4 years of severe hypertension.