Malignant pheochromocytoma

Abstract

Pheochromocytomas are rare but have significant morbidity and mortality. Malignant pheochromocytoma presents several difficulties in management, both in diagnosis, and in effective treatment. The diagnosis of malignant pheochromocytoma cannot be made reliably on histological, biochemical and immunochemical grounds. Malignant pheochromocytomas can only be accurately diagnosed if the tumors are found in areas that normally contain no chromaffin tissue. Extensive evaluations of tumor location are required to ascertain the presence of local or distant invasion or metastases. The main treatment for malignant tumor is resection and catecholamine blockade. If the tumor is inoperable or if there is residual disease after resection, MIBG or chemotherapy are the options. Life-long follow-up is recommended.