A CASE OF EXTRA-ADRENAL MALIGNANT PHEOCHROMOCYTOMA WITH INFILTRATION INTO THE INFERIOR VENA CAVA
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A 53-year-old man, whose operation under a diagnosis of a retroperitoneal tumor was sustained because his blood pressure increased at an intraoperative biopsy of the tumor at another hospital, was referred to the hospital with a diagnosis of pheochromocytoma. The diagnosis was made based on abnormally high levels of blood and urinary catecholamines and histopathological findings. On admission to the hospita, an abdominal ultrasonography, CT, and magnetic resonance imaging scans showed a tumor dosal to the liver, and infiltration into the inferior vena cava was suspected. No abnormalities were found in the bilateral adrenals. A removal of the extra-adrenal pheochromocytoma with a partial excision of the inferior vena cava was performed. The excised tumor was 6×6×5 cm in diameter and 66 g in weight, with multinodular surface and segmented section. Histopathologically, tumor cells showed honeycomb proliferation, and infiltration into the surrounding fatty tissues and venous invasion were also seen, which were indicative of malignancy. From these findings, extra-adrenal malignant pheochromocytoma was definitely diagnosed. After the operation, blood and urinary levels of catecholamines immediately decreased into normal ranges. The patient's postoperative course was uneventful and he was discharged from the hospital on 19th postoperative day.Extra-adrenal malignant pheochromocytoma reportedly accounts for about 4% of all pheochromocytomas, but its criteria for diagnosis are not necessarily clear. We report this rare case of extraadrenal pheochromocytoma, together with some discussion in terms of differentiation between benign and malignant lesions.
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