Desmoplastic Small Round Cell Tumor/Ewings Sarcoma Model

促结缔组织增生性小圆细胞肿瘤(DSRCT)是一种罕见但极具侵袭性的肿瘤，发生于腹部的青少年。在迄今检查的所有DSRCT病例中，涉及尤文斯肉瘤基因(EWS)和Wilms肿瘤抑制基因WT1的染色体易位导致了一种新的转录因子的产生。另一种令人感兴趣的儿科肿瘤是尤文？S肉瘤，其中EWS基因与Fl1基因融合。我们的实验室专注于了解EWS/WT1和EWS/FLI1基因产物的功能以及它们如何在各自的癌症发生中起作用。 为了达到这一目标，我们目前正在开发深低温诱导肿瘤和尤因？S肉瘤的小鼠模型，以更好地在整个生物体的背景下了解这些肿瘤的病因和发展。如果成功，我们的动物模型将对开发新的和现有的治疗DSRCT和尤因？S肉瘤的方法具有非常重要的价值。

Desmoplastic small round cell tumor (DSRCT) is a rare but extremely aggressive cancer arising in young adolescents in the abdominal area. In all cases of DSRCT examined to date, a chromosomal translocation involving the Ewings Sarcoma gene (EWS) and the Wilms tumor suppressor gene, WT1, results in genesis of a novel transcription factor. Another pediatric tumor of interest is the Ewing?s sarcoma, where EWS gene is fused to the Fli1 gene. Our laboratory is focused on understanding the functions of EWS/WT1 and EWS/Fli1 gene products and how they contribute to tumorigenesis in their respective cancers. Towards this goal, we are currently developing mouse models of DSRCT and Ewing?s sarcoma to better understand the etiology and the development of these tumors in the context of whole organism. If successful, our animal models will be invaluable for the development of new and existing therapeutics against DSRCT and Ewing?s sarcoma.