Complete remission of relapsing high-grade angiosarcoma with single-agent metronomic trofosfamide

A limited repertoire of chemotherapeutics is available for the therapy of metastasizing angiosarcoma. Moreover, response rates are typically low and outcomes are unfavorable. Metronomically, dosed oral chemotherapy provides a convenient treatment option and surprisingly high response rates have been published for small patient groups. We report on a case from our clinic, in which a complete response with oral trofosfamide was achieved in a patient suffering from relapsed high-grade angiosarcoma metastasizing to the liver and lung. The patient experienced minimal side-effects from her trofosfamide treatment. Responses like this are encouraging and should make us rethink treatment approaches for metastasizing soft-tissue sarcoma. The mechanism of action of metronomic chemotherapy, although thought to be antiangiogenic in nature, is still unclear, as is the additive effect of angiogenic inhibitors like cyclooxygenase II inhibitors or peroxisome proliferator-activated receptor-gamma agonists. Prospective studies that include the examination of patient samples during treatment are ongoing in order to optimize further development of this novel therapeutic approach.