Abstract
Vascular sarcomas are very rare tumours arising from the mesenchyme of blood vessels. They are among the most malignant tumours.
The incidence of leiomyosarcoma in veins is five times higher than in arteries. Diagnosis is usually made in the advanced stages and these tumours have a poor prognosis despite the multidisciplinary approaches taken by doctors.
The five-year survival rate is only 25%. The main treatment involves early surgical resection.
These tumours grow rapidly, often becoming inoperable within a few months. In order to cure them, radical oncological resection is required - resection with a margin of healthy tissue.
Radiotherapy is an important adjuvant for local control when the surgical margins are close or positive. The role of chemotherapy in systemic management remains unclear.