Comparison of tumor size for benign pheochromocytomas and malignant pheochromocytomas with local disease only
Size does not reliably predict malignancy in pheochromocytomas with local disease only
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Problem
When signs of regional involvement or distant disease are absent, there is currently no reliable preoperative diagnostic test that can differentiate between malignant and benign pheochromocytomas
Limited disease: curative intention
Extended disease: still to be considered in the first place for debulking and as palliative treatment
(Mundschenk et al. 2019)
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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.
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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Tumor size (mean ± SD) <2 cm 2.0-3.9 cm 4.0-5.9 cm 6.0-7.9 cm 8.0-9.9 cm ≥10 cm
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Байду номын сангаас
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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.
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.
Surgery Radiopharmaceuticals Combined Chemotherapy Arterial Embolization
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Primary surgical resection is the treatment of choice whenever possible
Introduction
The most frequent site of metastases is the skeleton
Additional sites are liver, retroperitoneum with lymph nodes, CNS, pleura, and kidney
Should pheochromocytoma size influence surgical approach?
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A comparison of 90 malignant and 60 benign pheochromocytomas
(Wen T. Shen et al.2019)
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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 Radiopharmaceuticals Combined Chemotherapy Arterial Embolization
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Alternative of Current Therapy
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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.