背景:尤文肉瘤(EWS)是骨和软组织的高度恶性肿瘤,偶尔起源于内脏。鉴于内脏EWS(V-EWS)的器官分布各异,并且通常是后期表现,因此难以管理。我们的目标是描述我们在V-EWS的机构经验,专注于其手术管理,并将V-EWS结果与骨性(O-EWS)和软组织EWS(ST-EWS)比较。
方法:对2000年至2022年期间在单一机构就诊的所有≤21岁的EWS患者进行回顾性回顾。比较了患者和疾病的特异性特征。使用KaplanMeier方法和对数秩检验估计总体和无复发生存率。
结果:确定了156例EWS患者:117个O-EWS,20ST-EWS,和19个V-EWS。V-EWS出现在肾脏(n=5),肺(n=5),肠(n=2),食管(n=1),肝脏(n=1),胰腺(n=1),肾上腺(n=1),阴道(n=1),大脑(n=1),和脊髓(n=1)。在EWS组之间没有检测到显著的人口统计学差异。V-EWS在出现时更频繁地转移(63.2%;p=0.005),然而,EWS组之间没有显著的总体或无复发生存差异,具有相似的随访间隔。虽然V-EWS需要多种独特的操作策略来获得主要控制,组间治疗策略无显著差异.仅手术的主要控制与所有组的总体和无复发生存率的改善相关。
结论:V-EWS在儿童和青少年中提出了独特的管理挑战,因为其来源的不同部位。这个大型队列是第一个描述V-EWS的手术管理和结果的队列,表现出更频繁的转移表现,同时实现各组相似的生存。
方法:第2级-队列研究。
BACKGROUND: Ewing sarcoma (EWS) is a highly malignant tumor of bone and soft tissue that occasionally arises from viscera. Visceral EWS (V-EWS) is challenging to manage given its varied organ distribution and often late-stage presentation. We aimed to characterize our institutional experience with V-EWS, focusing on its surgical management, and to compare V-EWS outcomes against those with osseous (O-EWS) and soft tissue EWS (ST-EWS).
METHODS: Retrospective review of all EWS patients ≤21 years presenting to a single institution between 2000 and 2022. Patient- and disease-specific characteristics were compared. Overall and relapse-free survival were estimated using Kaplan Meier methods and log-rank test.
RESULTS: 156 EWS patients were identified: 117 O-EWS, 20 ST-EWS, and 19 V-EWS. V-EWS arose in the kidney (n = 5), lung (n = 5), intestine (n = 2), esophagus (n = 1), liver (n = 1), pancreas (n = 1), adrenal gland (n = 1), vagina (n = 1), brain (n = 1), and spinal cord (n = 1). No significant demographic differences were detected between EWS groups. V-EWS was more frequently metastatic at presentation (63.2%; p = 0.005), yet no significant overall or relapse-free survival differences emerged between EWS groups, with similar follow-up intervals. While V-EWS required multiple unique operative strategies to gain primary control, no significant difference in treatment strategies appeared between groups. Surgery-only primary control was associated with improved overall and relapse-free survival in all groups.
CONCLUSIONS: V-EWS presents unique management challenges in children and adolescents given its variable sites of origin. This large cohort is the first to describe the surgical management and outcomes of V-EWS, demonstrating more frequent metastatic presentation, while achieving similar survival across groups.
METHODS: Level 2 - Cohort Study.