Primary sarcoma of the inferior vena cava: review of diagnosis, treatment, and outcomes in a case series

Leiomyosarcoma of the inferior vena cava is a rare mesenchymal tumor. The diagnostic approach, based on general guidelines of oncologic surgery, seems to be relatively routine; specific aspects of treatment, including vascular reconstruction, depend on tumor stage, grade, and location. In this report, the management of this disease in 5 patients is summarized and the literature is reviewed. A thorough diagnostic assessment includes sonography, computed tomography, angiography or duplex ultrasonography, perioperative pathohistologic examination, and appropriate differential diagnosis. Radical resection is associated with the best outcome and long-term survival. In this series, 4 of 5 patients underwent tumor resection. In 2 patients, the disease was classified as R0. Another patient had R1 status found at resection and underwent postoperative radiation after the tumor bed was marked intraoperatively. She has remained stable since treatment. One patient died of pulmonary metastases 32 months after primary R1 tumor resection. The 5th patient has been stable since diagnosis; resection was not possible because of severe accompanying diseases and because consent for surgical intervention could not be obtained from the patient. There is reasonable hope that leiomyosarcoma of the inferior vena cava can be treated successfully, even in advanced stages, with novel antineoplastic drugs and radiotherapeutic protocols. However, general treatment recommendations have not yet been compiled.