Treatment of malignant pheochromocytoma

Pheochromocytoma is a tumor derived from chromaffin tissue, which secretes catecholamines. Today, about 90 percent of patients with this tumor are cured by surgical procedures. In 8 to 15 percent of patients with this tumor there is unresectable, recurrent or metastatic disease, which causes significant morbidity and mortality. The natural history of metastatic disease includes long-term survivors; many, however, die early of disseminated disease. The most common site of metastatic lesions is the skeleton. Palliation for these lesions can often be achieved with the use of radiation therapy. Other sites are, in general, less responsive to radiation therapy. Chemotherapy has been used in combination with radiation therapy, but the results generally have been disappointing. Chemotherapy with doxorubicin hydrochloride and cyclophosphamide in combination with radiation therapy has provided good palliation for skeletal disease for about five months, when disease progression was again noted. Further information is needed concerning the optimal chemotherapeutic treatment of this unusual tumor.