1、19130 1Introduction rule of 10s for pheochromocytoma (PCC) 10% bilateral 10% extra-adrenal 10% extra-abdomen 10% malignant 10% familial 10% children 10% normal blood pressure19130 2Introduction The most frequent site of metastases is the skeleton Additional sites are liver, retroperitoneum with lymp
2、h nodes, CNS, pleura, and kidney19130 3Malignant vs. Benign Currently, there is no effective cure for malignant pheochromocytoma. There are also no reliable histopathological methods for distinguishing benign from malignant tumors. Malignancy requires evidence of metastases at non-chromaffin sites d
3、istant from that of the primary tumor.19130 4 Metastatic disease in pheochromocytoma may be present at the time of initial diagnosis or may only became evident after surgical removal of the primary tumor, usually within 5 years, but sometimes 16 or more years later.19130 5 Due to the rarity of the t
4、umor, clinical studies about pheochromocytoma suffer from a fragmented nature and usually involve too small a number of cases to reach conclusive results.19130 6 Because there is currently no effective cure for malignant pheochromocytoma, most treatment are palliative, but in some cases may reduce t
5、umor burden and prolong survival. Without treatment, the 5-year survival is generally less than 50%. The course, however, can be highly variable with occasional patients living more than 20 years after diagnosis.19130 7 Once malignancy is diagnosed, therapy is generally directed at controlling blood
6、 pressure, but may also include tumor debulking.19130 8Alternative of Current Therapy Surgery Radiopharmaceuticals Combined Chemotherapy Arterial Embolization19130 9Alternative of Current Therapy Surgery Radiopharmaceuticals Combined Chemotherapy Arterial Embolization19130 10 Primary surgical resection is the treatment of choice whenever possible Limited disease: curative intention Extended disease: still to be considered in the first place for debulking and as palliative treatment(Mundschenk et al. 1998)
