Case report:

we present the case of a 64-year-old man with suspected inferior vena cava leiomyosarcoma (IVC) treated with neoadjuvant radiotherapy, tumor and IVC overall surgical resection with a ringshaped PTFE graft reconstruction, followed by chemotherapy.

Discussion:

IVC leiomyosarcoma is a very infrequent malignant disease, its prognosis is dark and requires multidisciplinary management. They represent the most frequent location of venous leiomyosarcomas, have a predilection for the female sex and a mean age of presentation between 50 and 60 years. The most frequent symptom is abdominal pain, although many are asymptomatic. The diagnostic test of choice is MRI and treatment is based on surgical resection, the role of radio and chemotherapy is not clearly defined yet.

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