Leiomyosarcoma of the innominate vein is a rare but usually lethal disease. We report the
case of a 50-year-old woman, undergoing a curative resection of the tumor. She is alive and
free of disease at 88-month follow-up. Surgical excision remains the current optimal
treatment able to provide a chance of cure. KEY WORDS: Late survival, Venous
leiomyosarcoma.

Primary leiomyosarcoma of the innominate vein is a rare disease whose prognosis is
usually dismal. Nonetheless, two isolated cases of long-term survival after excision of the
neoplasm have been reported so far 1, 2. We now report a third case of late survival after
curative resection of this infrequent condition.