Meier Clemens-Magnus, Furtwängler Rhoikos, von Schweinitz Dietrich, Stein Raimund, Welter Nils, Wagenpfeil Stefan, Kager Leo, Schenk Jens-Peter, Vokuhl Christian, Melchior Patrick, Fuchs Jörg, Graf Norbert
Department of General Surgery, Visceral, Vascular and Pediatric Surgery, Saarland University Medical Center, 66421 Homburg, Germany.
Department of Pediatric Oncology and Hematology, Saarland University Medical Center, 66421 Homburg, Germany.
Cancers (Basel). 2022 Aug 14;14(16):3924. doi: 10.3390/cancers14163924.
(1) Background: Vena cava thrombus (VCT) is rare in Wilms tumor (WT) (4−10%). The aim of this study is to identify factors for an outcome to improve treatment for better survival. (2) Methods: 148/3015 patients with WT (aged < 18 years) and VCT, prospectively enrolled over a period of 32 years (1989−2020) by the German Society for Pediatric Oncology and Hematology (SIOP-9/GPOH, SIOP-93-01/GPOH and SIOP-2001/GPOH), are retrospectively analyzed to describe clinical features, response to preoperative chemotherapy (PC) (142 patients) and surgical interventions and to evaluate risk factors for overall survival (OS). (3) Results: 14 VCT regressed completely with PC and another 12 in parts. The thrombus was completely removed in 111 (85.4%), incompletely in 16 (12.3%), and not removed in 3 (2.3%). The type of removal is unknown in four patients. Patients without VCT have a significantly (p < 0.001) better OS (97.8%) than those with VCT (90.1%). OS after complete resection is (89.9%), after incomplete (93.8%) and with no resection (100%). Patients with anaplasia or stage IV without complete remission (CR) after PC had a significantly worse OS compared to the remaining patients with VCT (77.1% vs. 94.4%; p = 0.002). (4) Conclusions: As a result of our study, two risk factors for poor outcomes in WT patients with VCT emerge: diffuse anaplasia and metastatic disease, especially those with non-CR after PC.
（1）背景：腔静脉血栓（VCT）在肾母细胞瘤（WT）中较为罕见（4%-10%）。本研究的目的是确定影响预后的因素，以改善治疗方法，提高生存率。（2）方法：回顾性分析德国儿科肿瘤学和血液学协会（SIOP-9/GPOH、SIOP-93-01/GPOH和SIOP-2001/GPOH）在32年（1989-2020年）期间前瞻性纳入的148例年龄＜18岁的WT合并VCT患者，描述其临床特征、术前化疗（PC）反应（142例患者）和手术干预情况，并评估总生存（OS）的危险因素。（3）结果：14例VCT经PC后完全消退，另外12例部分消退。111例（85.4%）血栓被完全清除，16例（12.3%）清除不完全，3例（2.3%）未清除。4例患者的清除类型未知。无VCT的患者OS（97.8%）显著优于有VCT的患者（90.1%）（p＜0.001）。完全切除后的OS为（89.9%），不完全切除后为（93.8%），未切除为（100%）。与其余有VCT的患者相比，PC后未完全缓解（CR）的间变或IV期患者OS显著更差（77.1%对94.4%；p = 0.002）。（4）结论：我们的研究结果显示，WT合并VCT患者预后不良的两个危险因素为弥漫性间变和转移性疾病，尤其是PC后未CR的患者。
