Treatment of intimal sarcoma of peripheral veins

Abstract

Introduction: Intimal sarcoma is an extremely rare group of undifferentiated pleomorphic sarcoma arising from the intimal layer of vessels accounting for only 1% of all sarcomas, intimal sarcoma of large veins are even less common.

Cases presentation: We present two cases of intima sarcoma, one originated form the basilar vein and the other from the cephalic vein, the first one was treated with surgery and postoperative chemotherapy followed by Radiotherapy (RT), the second case was treated with isolated limb perfusion followed by marginal resection and RT. Both patients progressed to the lungs in a short time, the first case was treated with metastasectomy of the lung and is without evidence of disease 7months after surgery; the second case treated with isolated limb perfusion has stable disease.

Discussion: Intimal sarcoma are very aggressive tumors, with a high metastatic potential, the two patients progressed to lung in a short time (2months) after local treatment. Both cases exhibit good response to chemotherapy and metastasectomy with a disease - free period of 7months.

Conclusion: We propose that given the aggressive behavior of these tumors, they should be treated with chemoradiotherapy postoperative, either by systemic chemotherapy or isolated limb perfusion for the limp sparing surgery in this histology.

Keywords: Chemoradiotherapy; Intimal sarcoma; Intimal sarcoma of peripheral veins; Limp sparing surgery; Metastasectomy.