Pharmacological management of Ewing sarcoma family of tumours

Abstract

The Ewing sarcoma family of tumours comprises of a group of well-characterised neoplasms with aggressive behaviour. Despite significant progress with the use of intensive multiagent chemotherapy and local control measures, a significant proportion of patients die because of disease progression. Most treatment regimens are based on the intensification of alkylating agents and topoisomerase-II inhibitors. Using this approach, the expected survival rate is between 70 and 80% in patients with localised disease. An increasingly important complication among survivors is the development of treatment-related haematological malignancies. The outcome for patients with metastatic disease is very poor and many studies have explored the use of high-dose chemotherapy with haematopoietic stem cell transplantation (HSCT). The benefit of this approach is unclear and HSCT must therefore be considered investigational. New strategies, such as the use of immunotherapy, biological modifiers and novel classes of chemotherapeutic agents must be explored.