Malignant Extra-adrenal Phaeochromocytoma

Three cases of malignant phaeochromocytoma (paraganglioma) are described. Two of them were metastasising, the third was widely infiltrating and therefore inoperable. All three tumours were extra-adrenal, two were pharmacologically inactive, one (the inoperable case) had typical clinical signs of phaeochromocytoma such as attacks of hypertension and increased excretion of urinary catecholamines. All three patients were given radiotherapy, two of them post-operatively and one as the only treatment. The response to irradiation was fairly poor. One of the patients died, two are alive three and two years after therapy. Attention is drawn to the protective amines excreted by pharmacologically active phaeochromocytomas as one of the reasons for the poor response to radiotherapy.