Case SeriesTreatment of intimal sarcoma of peripheral veins

•Intimal sarcoma is an extremely rare group.•Intimal sarcoma of large veins even rarer information and case reports are limited.•Unique case in the medical literature and the best current evidence in the biological behavior and the response of the multiple options of treatment in this type of tumors.

IntroductionIntimal 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 presentationWe 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 7 months after surgery; the second case treated with isolated limb perfusion has stable disease.DiscussionIntimal sarcoma are very aggressive tumors, with a high metastatic potential, the two patients progressed to lung in a short time (2 months) after local treatment. Both cases exhibit good response to chemotherapy and metastasectomy with a disease - free period of 7 months.ConclusionWe 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.