Background. Tumors of the inferior vena cava (IVG) are rare tumors. Although often locally confined, juxtaposed vital structures usually limit the extent of resection. However, complete surgical resection has been shown to be the most important positive prognostic factor. Methods. Four patients had resection of primary vena caval tumors. In two patients with locally extensive vena caval tumors the limits of conventional resection were extended by means of complete resection of the involved infrahepatic IVC, aorta, and both kidneys. The IVC and aorta were reconstructed with synthetic grafts, and the uninvolved kidney was autotransplanted for both patients. Results. Of the two patients treated with more extensive resection, one patient had no evidence of disease 26 months after operation, and the second patient died of recurrent disease 23 months after operation. Conclusions. Primary tumors of the IVC may extend locally without distant metastasis. Radical surgical excision as the primary mode of treatment provides the best chance for prolonged survival in appropriately selected patients with tumors of the IVC. After surgical excision the patient with the leiomyosarcoma was treated with radiation therapy and the patient with rhabdomyosarcoma by chemotherapy. Although leiomyosarcomas of the IVC are rare tumors, the first patient is only the third reported case of the even rarer rhabdomyosarcoma of the IVC.
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