eComment: Sarcomas of the great vessels. Is there a role for chemotherapy?

We read with great interest the article by Tanaka et al. regarding a case of aortic intimal sarcoma [1]. We would like to highlight the role of adjuvant chemotherapy in a setting of multimodality treatment. Sarcomas of the great vessels previously diagnosed during surgery or autopsy are rare and highly lethal. This represents predominantely female patients between the age of 22 and 81. The prognosis is poor with a mean survival of 12 months after onset of symptoms and one and two years survival rates of 22% and 7%, respectively [2]. The mainstay of treatment for sarcoma is surgical resection as this remains the only potentially curative modality. Adjuvant chemotherapy and radiation can be considered following surgical excision although their role remains undefined. An ∼20% response rate can be expected with a combination chemotherapy regimen involving an anthracycline and an alkylating agent; however, the value of this regimen in the adjuvant setting for intimal aortic sarcoma is unclear [3]. According to the literature, there are two cases with satisfactory survival. The first patient underwent resection followed by adjuvant chemotherapy [4]. The second one was found inoperable during surgery and underwent a two-drug combination chemotherapy consisting of ifosfamide and epirubicin with long-term survival [5]. In our institute, we had a similar unresectable case of primary pulmonary artery sarcoma. The patient underwent the same chemotherapeutic scheme and is still alive after six months, with a marked regression of the tumor. In conclusion, we think that intensive chemotherapy is worth trying in unresectable patients.