エピネフリンが異常高値を示した副腎外褐色細胞腫の一例

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  • A Case of Extra-adrenal Pheochromocytoma Producing Epinephrine

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We reported a case of a 72-year-old man with an extra-adrenal pheochromocytoma secreting excessive epinephrine (E). He had been treated for diabetes mellitus and essential hypertension. His diabetes was controlled poorly, but his blood pressure was controlled well. He was admitted to our hospital for weight loss evaluation. An upper gastrointestinal series and computed tomography of the abdomen revealed an ovoid mass (diameter, approximately 5×6 cm) overlying the abdominal aorta and the inferior vena cava between the second and the third lumbar vertebra. Aortography showed a hypervascular mass. Analysis of catecholamine (CA) and its metabolites showed elevations in plasma norepinephrine (NE, 3850 pg/ml) and E (560 pg/ml) levels as well as the urinary NE (1200-1400μg/day), E (310-350μg/day) and vanillylmandelic acid (26.8 mg/day) levels. Vena caval CA sampling indicated that the peak levels for both NE and E were at the third lumbar vertebra. The tumor (104 g) was removed in April 1991. Histological analysis revealed a typical pheochromocytoma with paraganglia adjacent to the tumor. The tumor cells were immunoreacted to phenylethanolamine-N-methyltransferase (PNMT). NE and E granules appeared on electron microscopy, and the urinary NE and E were normalized by the surgery. These findings suggest that our case may be an E-secreting extra-adrenal pheochromocytoma.<BR>The conversion of NE to E is catalyzed by PNMT found primarily in the adrenal medulla and the organ of Zuckerkandl. An E-secreting pheochromocytoma is generally located at either of these two sites. The classic location of the tumor to the Zuckerkandl organ is in the retroperitoneal tissue lateral to or overlying the lower portion of the abdominal aorta in the vicinity of the inferior mesenteric artery. Our case was at the typical location and had a microscopic pattern of paraganglia adjacent to the tumor. In our case, the pheochromocytoma appears to have originated in the Zuckerkandl.

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