Leiomyosarcoma of the inferior vena cava: a clinicopathologic review and report of four cases

AIM: This article describes four cases of leiomyosarcoma treated in the University Hospital of Turin and reviews current understanding of the biological behavior of the tumor, together with essential diagnostic procedures and established approaches to treatment. METHODS: Between February 2004 and December 2005, four patients (two men and two women; mean age 57.5 years) with leiomyosarcoma of the interior vena cava (IVC) were treated at the Vascular Surgery Unit. Resection and excision of the tumor were carried out (4/4 patients), with resection of the IVC above and below the mass (2/4) and reconstruction of the vein with a Dacron prosthesis with termino-lateral reimplantation of the renal vein in one and placement of a polytetrafluorethylene graft in the other. RESULTS: The postoperative course was unremarkable; the patients were discharged between postoperative days VIII and XI. Oral anticoagulant therapy with dicumarol (3/4) was given to maintain venous or prosthesis patency during the follow-up period. The mean length of follow-up was 23 months (range, 16-28). All patients recovered without local recurrence of disease. CONCLUSION: This malignant slow-growing tumor produces late clinical manifestations, making the study of its natural history clinically important. Currently, surgical excision is the only therapy that can alter disease progression and improve survival. The utility of adjuvant chemo- and radiotherapy remains controversial