| Popis: |
Malignant pheochromocytomas and paragangliomas are rare. Resection of the primary tumor and metastatic lesions, when feasible, is recommended. Goals of resection include improvement of clinical symptoms, reduction of catecholamine excess, local disease control, improved efficacy of subsequent non-operative therapies, and the possibility of improved survival. A R0 or R1 resection clearly provides more robust biochemical improvement when compared to a R2 resection or ‘surgical debulking.’ Additional non-surgical therapies can be used in conjunction with surgery or as the primary treatment modality in some cases. Non-surgical local therapies include external beam radiation, percutaneous tumor ablation, and directed transarterial chemoembolization. In addition, systemic therapies include radioactive iodine meta-iodobenzylguanidine (131I-MIBG), cytotoxic chemotherapy, and molecular targeted therapy. Care should be taken to provide patients with the appropriate pharmacologic adrenergic blockade prior to the initiation of most therapies. Patients benefit from a multidisciplinary approach at a center familiar with managing malignant pheochromocytoma patients. |
