Pediatric Hematology and Oncology Institute, Centre Léon Berard, Lyon.
SIREDO Oncology Center (Care, Innovation and Research for Children, Adolescents and Young Adults With Cancer), Institut Curie, PSL University, Paris.
J Pediatr Hematol Oncol. 2022 Apr 1;44(3):e637-e642. doi: 10.1097/MPH.0000000000002252.
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.
促纤维增生性小圆细胞肿瘤（DSRCT）是一种发生于年轻人、青少年和成年人群中的侵袭性肉瘤。本研究旨在比较儿科和成人 DSRCT 患者的初始肿瘤表现、治疗管理和可扩展性，并探讨特定治疗方法的可能性。进行了一项多中心回顾性研究，纳入了 81 例携带尤文肉瘤-Wilms 瘤转录本的法比荷医学文件。中位年龄为 17 岁（3-58 岁），其中 42 例为儿童（13.5 岁[3-17 岁]），39 例为成人（28 岁[18-58 岁]）。两组在初始症状和诊断时的转移方面无显著差异。两组的治疗方法相似：使用新辅助化疗（78.6%对 79.5%，P=1）、原发性手术（71.4%对 69.2%，P=0.73）、辅助化疗（54.8%对 61.5%，P=0.99）、放疗（23.8%和 10.3%，P=0.11）和腹腔内化疗（14.3%对 2.6%；P=0.11）。复发中位时间为 12 个月对 18 个月（P=0.13）。2 年总生存率和无复发生存率分别为 46.4%对 60.1%（P=0.83）和 14.3%对 16%（P=0.16）。DSRCT 的临床表现、初始治疗和结局相似，表明儿童和成人 DSRCT 应考虑采用类似的治疗方法。
