Malignant pheochromocytoma of the organ of Zuckerkandl requiring aortic and vena caval reconstruction.

OBJECTIVE

To describe a case of a malignant pheochromocytoma located in the organ of Zuckerkandl that required aortic and vena caval resection and reconstruction.

METHODS

We present a case report that includes clinical, laboratory, and radiographic data as well as photographs, results from pathology, and a brief review of the literature.

RESULTS

A 46-year-old man was referred for evaluation of a 1.4-cm left adrenal mass incidentally discovered on an abdominopelvic computed tomography (CT) scan. Subsequent laboratory evaluation revealed the following values: urine norepinephrine, 252 microg/24 h; urine normetanephrine, 1122 microg/24 h; urine metanephrine, 162 microg/24 h; urine epinephrine, 7 microg/24 h; urine vanillylmandelic acid, 8 mg/24 h; and plasma metanephrine, 98 pg/ mL. Imaging characteristics of the left adrenal mass were consistent with a benign adenoma, but CT also demonstrated a hypervascular para-aortic mass. 123I-metaiodo-benzylguanidine scanning with fusion CT imaging demonstrated increased radiopharmaceutical uptake within the para-aortic mass consistent with a paraganglioma in the organ of Zuckerkandl. Findings from CT angiography of the abdomen and pelvis suggested aortic involvement and vena caval thrombus. The mass was excised en bloc, including portions of the aorta, inferior vena cava, and right ureter. The aorta and vena cava were reconstructed using Dacron grafts. The remaining right ureter and kidney were removed to avoid the possibility of a urine leak from an ureteroureterostomy. Final pathologic and operative findings confirmed a malignant pheochromocytoma of the organ of Zuckerkandl with invasion into the wall of the inferior vena cava and tumor thrombus extending into the lumen.

CONCLUSION

Malignant pheochromocytoma of the organ of Zuckerkandl involving the aorta and inferior vena cava is exceedingly rare, and although surgical resection and reconstruction can be radical and aggressive, this treatment offers the only chance for cure.