Management and outcome of pediatric Wilms tumor with malignant inferior Vena cava thrombus: largest cohort of single-center experience.,International Journal of Clinical Oncology

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Management and outcome of pediatric Wilms tumor with malignant inferior Vena cava thrombus: largest cohort of single-center experience.
International Journal of Clinical Oncology ( IF 3.3 ) Pub Date : 2020-04-05 , DOI: 10.1007/s10147-020-01667-0
Moatasem Elayadi _{1,

2} , Mahmoud Hammad _{1,

2} , Kareem Sallam ₃ , Gehad Ahmed _{3,

4} , Soha Ahmed _{5,

6} , Ahmed Ibrahim ₇ , Amal Refaat _{8,

9} , Naglaa Elkinaai _{10,

11} , Alaa Younes _{4,

12} , Norbert Graf ₁₃ , Wael Zekri _{1,

2}

Affiliation

Department of Pediatric Oncology, National Cancer Institute, Cairo University, Fom El-khalig, Square Kasr El-Aini St., Cairo, 11796, Egypt.
Department of Pediatric Oncology, Children Cancer Hospital Egypt-57357, Cairo, Egypt.
Department of Surgery, Faculty of Medicine, Helwan University, Cairo, Egypt.
Department of Surgical Oncology, Children Cancer Hospital Egypt-57357, Cairo, Egypt.
Department of Radiation Oncology, Children Cancer Hospital Egypt-57357, Cairo, Egypt.
Department of Clinical Oncology, Aswan University, Aswan, Egypt.
Department of Clinical Research, Children Cancer Hospital Egypt-57357, Cairo, Egypt.
Department of Radio-Diagnosis, National Cancer Institute, Cairo University, Cairo, Egypt.
Department of Radio-Diagnosis, Children Cancer Hospital Egypt-57357, Cairo, Egypt.
Department of Pathology, National Cancer Institute, Cairo University, Cairo, Egypt.
Department of Pathology, Children Cancer Hospital Egypt-57357, Cairo, Egypt.
Department of Surgical Oncology, National Cancer Institute, Cairo University, Cairo, Egypt.
Department of Pediatric, Saarland University, Campus Homburg, Homburg, Germany.

Background

Wilms tumor (WT) with an inferior Vena cava (IVC) malignant thrombus comprises 4–10% of all WT cases.

Methods

This retrospective analysis included 51 pediatric patients presenting at Children Cancer Hospital Egypt-57357 from July 2007 to December 2016 with the diagnosis of WT with malignant IVC thrombus.

Results

Median age at presentation = 4.4 years and 28 cases (55%) were females. Twenty-five patients (49%) were metastatic and 4 patients (7.8%) had bilateral disease. Forty-seven cases (92.2%) had favorable histology with no evidence of anaplasia. Level of thrombus extension at presentation was classified as infra-hepatic, retro-hepatic, supra-hepatic and intra-cardiac in 33, 9, 6 and 3 patients, respectively. Fifty patients started neoadjuvant chemotherapy (CTH) with 16 patients showing complete resolution of thrombus after 6 weeks of CTH. None of the patients developed thrombus progression after neoadjuvant CTH; one patient had stationary intra-cardiac thrombus, while remaining patients showed partial regression of their thrombus and had nephrectomy with en-bloc thrombectomy. The mean cranio-caudal dimension of IVC thrombi at initial presentation was 6.5 cm, and 3.6 cm post 6th week of CTH. The 5-year OS and EFS were 75.9% and 71.1%, respectively. There was no significant correlation of initial levels of thrombus extension with survival.

Conclusion

Neoadjuvant chemotherapy followed by radical nephrectomy with en-bloc thrombectomy and radiotherapy seems a successful approach for management of patients with WT and IVC tumor thrombus. Measurement of the cranio-caudal dimension of thrombus and its response to treatment should be considered in the surgical planning.

中文翻译：

恶性下腔静脉血栓形成的小儿Wilms肿瘤的治疗和结局：单中心经验最大的队列。

背景

下腔静脉（IVC）恶性血栓的Wilms肿瘤（WT）占所有WT病例的4-10％。

方法

这项回顾性分析纳入了2007年7月至2016年12月在埃及儿童癌症医院（England-57357）诊治的WT合并恶性IVC血栓的51例儿科患者。

结果

出现时的中位年龄为4.4岁，其中28例（55％）为女性。二十五例（49％）转移，四例（7.8％）患有双侧疾病。47例（92.2％）的组织学检查良好，没有发育不良的迹象。出现时的血栓扩展水平分别分为33例，9例，6例和3例为肝内，肝后，肝上和心脏内。50名患者开始新辅助化疗（CTH），其中16名患者在CTH治疗6周后血栓完全消失。新辅助CTH后无患者发生血栓进展。1例患者发生了心脏内固定血栓，其余患者表现出部分血栓消退，并进行了全血栓切除术。初次出现时，IVC血栓的平均颅尾尺寸为6。CTH第6周后5厘米和3.6厘米。五年OS和EFS分别为75.9％和71.1％。血栓延伸的初始水平与生存率无显着相关性。