Malignant Pheochromocytoma

Abstract

A patient suffering from Recklinghausen's disease and malignant pheochromocytoma was studied. The tumour was extraadrenal, produced mostly norepinephrine and metastasized to the skeleton, lymphnodes, liver and lungs. At autopsy an adenoma of the parathyroid gland was found as well. The initial symptoms are the same in both the benign and malignant cases and are largely related to the production of catechol amines. In the malignant cases the signs and symptoms of metastases are added. It seems probable that malignant tumours can be differentiated from benign ones by measuring the amount of homovanillic acid in the urine. In the malignant cases large amounts are excreted. In this respect–as in its way of metastasizing–the tumour resembles the neuroblastoma. The only form of therapy offering a cure is operation. If the tumour is inoperable or if it has already metastasized, radiation therapy could be tried, but the tumour is not very sensitive to radiation. Furthermore, treatment with agents blocking the synthesis of catechol amines or the adrenergic receptors should be given. The prognosis is bad, the patients surviving about three years after diagnosis. The interesting coincidence of pheochromocytoma with other diseases– Recklinghausen's disease, parathyroid adenoma, and the medullary thyroid cancer–should be kept in mind.