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Volume 2016, Article ID 5043640, 11 pages
Research Article

Ewing’s Sarcoma as a Second Malignancy in Long-Term Survivors of Childhood Hematologic Malignancies

1Department of Neurology, University Hospital Zurich, Frauenklinikstrasse 26, 8091 Zurich, Switzerland
2Department of Medical Oncology, University Hospital Zurich, Rämistrasse 100, 8091 Zurich, Switzerland
3Department of Pediatric Oncology, University Children’s Hospital, Zurich, Steinwiesstrasse 75, 8032 Zurich, Switzerland
4Institute of Surgical Pathology, University Hospital Zurich, Schmelzbergstrasse 12, 8091 Zurich, Switzerland
5Department of Neuropathology, University Hospital Zurich, Schmelzbergstrasse 12, 8091 Zurich, Switzerland

Received 3 April 2016; Accepted 19 June 2016

Academic Editor: Natia Esiashvili

Copyright © 2016 Fabian Wolpert et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


Modern multimodal treatment has significantly increased survival for patients affected by hematologic malignancies, especially in childhood. Following remission, however, the risk of developing a further malignancy is an important issue. The long-term estimated risk of developing a sarcoma as a secondary malignancy is increased severalfold in comparison to the general population. Ewing’s sarcoma family encompasses a group of highly aggressive, undifferentiated, intra- and extraosseous, mesenchymal tumors, caused by several types of translocations usually involving the EWSR1 gene. Translocation associated sarcomas, such as Ewing sarcoma, are only rarely encountered as therapy associated secondary tumors. We describe the clinical course and management of three patients from a single institution with Ewing’s sarcoma that followed successfully treated lymphoblastic T-cell leukemia or non-Hodgkin lymphoma. The literature on secondary Ewing’s sarcoma is summarized and possible pathogenic mechanisms are critically discussed.