Suchergebnisse

Germ cell tumors are relatively rare compared with other malignancies, and compilations of knowledge that encompass the entire spectrum of the disease are lacking. This textbook, written by the foremost authorities in the field, rectifies the situation by discussing in depth a broad range of topics, including biology, epidemiology, pathology, treatment, and late effects. Bearing in mind that germ cell tumors are most prevalent in the adolescent and young adult age group, causes of disease and treatment approaches in pediatric and adult patients are compared and contrasted. By spanning the entire life course, from prenatal origins of disease through to treatment in adults and late effects of treatment, the editors have produced a book that will be of interest to both pediatric and adult oncologists

Despite multimodal treatment, long term outcome for patients with Ewing sarcoma is still poor. The second "European interdisciplinary Ewing sarcoma research summit" assembled a large group of scientific experts in the field to discuss their latest unpublished findings on the way to the identification of novel therapeutic targets and strategies. Ewing sarcoma is characterized by a quiet genome with presence of an EWSR1-ETS gene rearrangement as the only and defining genetic aberration. RNA-sequencing of recently described Ewing-like sarcomas with variant translocations identified them as biologically distinct diseases. Various presentations adressed mechanisms of EWS-ETS fusion protein activities with a focus on EWS-FLI1. Data were presented shedding light on the molecular underpinnings of genetic permissiveness to this disease uncovering interaction of EWS-FLI1 with recently discovered susceptibility loci. Epigenetic context as a consequence of the interaction between the oncoprotein, cell type, developmental stage, and tissue microenvironment emerged as dominant theme in the discussion of the molecular pathogenesis and inter- and intra-tumor heterogeneity of Ewing sarcoma, and the difficulty to generate animal models faithfully recapitulating the human disease. The problem of preclinical development of biologically targeted therapeutics was discussed and promising perspectives were offered from the study of novel in vitro models. Finally, it was concluded that in order to facilitate rapid pre-clinical and clinical development of novel therapies in Ewing sarcoma, the community needs a platform to maintain knowledge of unpublished results, systems and models used in drug testing and to continue the open dialogue initiated at the first two Ewing sarcoma summits. ; We thank Anirah Amber and Nuno Andrade for excellent organization of the meeting. The conference was supported by the European Union's Seventh Framework Programme grants 261743(ENCCA) and 259348 (ASSET). ; Sí