恶性嗜铬细胞瘤的治疗.ppt

Therapy of Malignant Pheochromocytoma恶性嗜铬细胞瘤的治疗
Literature Report
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Introduction
rule of 10s for pheochromocytoma (PCC)
10% bilateral
10% extra-adrenal
10% extra-abdomen
10% malignant
10% familial
10% children
10% normal blood pressure
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Introduction
The most frequent site of metastases is the skeleton
Additional sites are liver, retroperitoneum with lymph nodes, CNS, pleura, and kidney
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Malignant 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 distant from that of the primary tumor.
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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.
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Due to the rarity of the tumor, clinical studies about pheochromocytoma suffer from a fragmented nature and usually involve too small a number of cases to reach conclusive results.
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Because there is currently no effective cure for malignant pheochromocytoma, most treatment are palliative, but in some cases may reduce tumor 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.
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Once malignancy is diagnosed, therapy is generally directed at controlling blood pressure, but may also include tumor debulking.
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Alternative of Current Therapy
Surgery
bined Chemotherapy
Arterial Embolization
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Alternative of Current Therapy
Surgery
bined Chemotherapy
Arterial Embolization
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