Primary Smooth Muscle Tumors of Venous Origin

Vein tumors are rare, difficult to diagnose, and usually malignant. We have encountered three: a leiomyoma of the jugular vein and leiomyosarcomas of the saphenous vein and inferior vena cava (IVC). The leiomyoma was lost to follow-up, the saphenous vein leiomyosarcoma survived nine years, and the leiomyosarcoma of the IVC is six months without recurrence. Half of venous leiomyosarcomas arise in the IVC, predominately in women over 50 years of age. Surgical excision is the treatment of choice since malignant or benign status cannot be determined operatively. Resection should include a segment of the original vessel. This poses problems in the IVC when the renal veins require sacrifice. Right renal vein interruption mandates nephrectomy. Edema following IVC resection is evaluated. The incidence is lower than anticipated when resection is for tumor if there is no history of phlebitis. The IVC was reconstructed with a composite autograft but this is not now recommended. Despite significant local recurrences or distal metastases, cure or long-term palliation can often be achieved. Radiation and chemotherapy do not improve survival or prevent recurrence.