Primary malignancy of the inferior vena cava, a review of surgical treatments and outcomes.

Introduction: Primary malignancies of the inferior vena cava comprise a heterogeneous group of histologic types that generally have a poor prognosis. Their rarity limits the data available in literature. There is no doubt that surgery is the mainstay treatment, but several questions about the best surgical strategy and multidisciplinary approach remain. The present review covers the surgical technique including the various reconstructive modalities and the available evidence about treatments and outcomes.

Evidence acquisition: From literature research, we identified 15 case series of patients with primary inferior vena cava tumors, who underwent surgery from 2000 onwards. Details on resection and reconstruction, focusing on both oncologic and surgical issues, were analyzed and summarized.

Evidence synthesis: Inferior vena cava malignancies can be either primary or secondary. The main primary tumor is leiomyosarcoma, while retroperitoneal liposarcomas and renal carcinomas are the main causes of secondary invasion. The outcomes of primary inferior vena cava leiomyosarcoma are linked to its metastatic risk. However, long-term survivors do exist. The factors that determine the surgical strategy include extent of the disease, which segment is involved, additional organ resection needed, presence of collateral venous circulation. After a partial resection, the wall defect can be repaired primarily or by patch interposition. After a circumferential resection, the first decision is whether to reconstruct the vascular continuity. There are several options, including prosthetic (polytetrafluoroethylene and Dacron) or biological materials (from autologous venous grafts to cryopreserved aortic grafts).

Conclusions: Tumors involving inferior vena cava are rare and challenging. A specific expertise is required to select the most appropriate surgical resection and reconstruction for the single patients in order to maximize the chance of cure alongside the quality of life.