Desmoplastic small round cell tumor (DSRCT) is an aggressive sarcoma occurring in the young, teenager, and adult populations. The aim of this study is to compare initial tumor presentation, therapeutic management and scalability between pediatric and adult DSRCT patients and investigate the possibility of specific therapeutic approaches. A multicenter retrospective study of 81 Franco-Belgian medical files with DSRCT harboring Ewing sarcoma-Wilm tumor transcript was made. Median age was 17 years (3 to 58) with 42 children (13.5 y [3;17]) and 39 adults (28 y [18;58]). No significant differences were found between the 2 groups regarding initial symptoms and metastasis at diagnosis. The therapeutic approaches were similar for both groups: use of neoadjuvant chemotherapy (78.6% vs. 79.5%, P=1), primary surgery (71.4% vs. 69.2%, P=0.73), adjuvant chemotherapy (54.8% vs. 61.5%, P=0.99), radiotherapy (23.8% and 10.3%, P=0.11) and intraperitoneal chemotherapy (14.3% vs. 2.6%; P=0.11). Median time to recurrence was 12 versus 18 months (P=0.13). Overall survival at 2 years and recurrence free were 46.4% versus 60.1% (P=0.83) and 14.3% versus 16%, respectively (P=0.16). Clinical presentation, initial therapeutics and outcome of DSRCT are equivalent suggesting that similar management should be considered for children and adults with DSRCT.

中文翻译：

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具有 EWS-WT1 转录物表达的促结缔组织增生性小圆细胞肿瘤：我们应该区别对待儿童和成人患者吗？

促纤维增生性小圆细胞瘤 (DSRCT) 是一种侵袭性肉瘤，发生在年轻人、青少年和成人人群中。本研究的目的是比较儿科和成人 DSRCT 患者的初始肿瘤表现、治疗管理和可扩展性，并研究特定治疗方法的可能性。对 81 份法国-比利时医疗文件进行了一项多中心回顾性研究，其中 DSRCT 包含尤文肉瘤-Wilm 肿瘤转录本。中位年龄为 17 岁（3 至 58 岁），其中有 42 名儿童（13.5 岁 [3;17]）和 39 名成人（28 岁 [18;58]）。两组之间在诊断时的初始症状和转移方面没有显着差异。两组的治疗方法相似：使用新辅助化疗（78.6% 对 79.5%，P=1），初次手术（71.4% 对 69.2%，P=0.73），辅助化疗（54.8% vs. 61.5%，P=0.99）、放疗（23.8%和10.3%，P=0.11）和腹腔化疗（14.3% vs. 2.6%；P=0.11）。中位复发时间分别为 12 个月和 18 个月 (P=0.13)。2 年总生存率和无复发率分别为 46.4% 和 60.1% (P=0.83) 和 14.3% 和 16% (P=0.16)。DSRCT 的临床表现、初始治疗和结果是相同的，这表明对于患有 DSRCT 的儿童和成人应该考虑类似的管理。