Background: Leiomyosarcomas of the inferior vena cava represent a rare form of soft-tissue sarcomas. Management strategies necessarily vary because of limited experience. Questions about necessity of multimodality therapy and IVC reconstruction remain.
Study design: Six patients were referred to our institution during a period of 6 years for leiomyosarcomas of the IVC. Demographic data, imaging results, pathology reports, preoperative radiation regimen, and postoperative outcomes were reviewed. Outcomes were compared with those of other published institutional experiences.
Results: After preoperative external-beam radiation (4,500 to 5,000 cGy), all patients underwent en bloc resection of the primary malignancy. Four patients (66%) had an R0 resection. All tumors were high grade. No reconstruction of the IVC was undertaken. Lower-extremity edema developed in 3 patients (50%), but this was well tolerated and did not lead to any long-term sequelae. Pulmonary metastasis developed postoperatively in 1 patient (17%) and was successfully treated with chemotherapy and metastectomy. Acute renal failure developed in 3 patients, but all recovered full function. Chylous leak developed in 2 patients (34%).
Conclusions: Leiomyosarcoma of the IVC is a treatable malignancy. Preoperative external-beam radiation facilitates marginally negative resection, although our study is too small to demonstrate a survival benefit. Reconstruction of the IVC is not necessary for resection of tumors below the level of the hepatic veins in most if not all cases. Lower-extremity edema after ligation of the IVC is well tolerated. Acute renal failure can be a common, albeit transient, early postoperative complication. Extensive periaortic dissection can be associated with chylous leak, which can be managed with internal or external drainage.