Multicentric pheochromocytoma and involvement of the inferior vena cava.

CONTEXT

Extension of pheochromocytomas to the inferior vena cava is rare. Multicentric tumors are rare as well, being present in up to 10% of cases. Surgery is the treatment of choice because of the long-term survival free of disease.

DESIGN

Case report.

CASE REPORT

We report on a case of right adrenal pheochromocytoma with extension to the supra-diaphragmatic vena cava, which underwent surgical excision through thoracophrenic laparotomy without the need for cardiopulmonary bypass. In a 6-year follow-up, another pheochromocytoma was found in the infra-renal Zuckerkandl's organ. Complete surgical excision of the tumor was performed by a median laparotomy and complete retroperitoneal dissection. In both cases, the total removal of the pheochromocytoma has been guaranteed by having margins free of tumor and a normal post-operative level of catecholamines. The pathological study revealed a malignant pheochromocytoma with margins free of neoplasia in both specimens.