Moatasem Elayadi1,2, Mahmoud Hammad3,4, Kareem Sallam5, Gehad Ahmed5,6, Soha Ahmed7,8, Ahmed Ibrahim9, Amal Refaat10,11, Naglaa Elkinaai12,13, Alaa Younes6,14, Norbert Graf15, Wael Zekri3,4. 1. Department of Pediatric Oncology, National Cancer Institute, Cairo University, Fom El-khalig, Square Kasr El-Aini St., Cairo, 11796, Egypt. moatasem.elayadi@nci.cu.edu.eg. 2. Department of Pediatric Oncology, Children Cancer Hospital Egypt-57357, Cairo, Egypt. moatasem.elayadi@nci.cu.edu.eg. 3. Department of Pediatric Oncology, National Cancer Institute, Cairo University, Fom El-khalig, Square Kasr El-Aini St., Cairo, 11796, Egypt. 4. Department of Pediatric Oncology, Children Cancer Hospital Egypt-57357, Cairo, Egypt. 5. Department of Surgery, Faculty of Medicine, Helwan University, Cairo, Egypt. 6. Department of Surgical Oncology, Children Cancer Hospital Egypt-57357, Cairo, Egypt. 7. Department of Radiation Oncology, Children Cancer Hospital Egypt-57357, Cairo, Egypt. 8. Department of Clinical Oncology, Aswan University, Aswan, Egypt. 9. Department of Clinical Research, Children Cancer Hospital Egypt-57357, Cairo, Egypt. 10. Department of Radio-Diagnosis, National Cancer Institute, Cairo University, Cairo, Egypt. 11. Department of Radio-Diagnosis, Children Cancer Hospital Egypt-57357, Cairo, Egypt. 12. Department of Pathology, National Cancer Institute, Cairo University, Cairo, Egypt. 13. Department of Pathology, Children Cancer Hospital Egypt-57357, Cairo, Egypt. 14. Department of Surgical Oncology, National Cancer Institute, Cairo University, Cairo, Egypt. 15. Department of Pediatric, Saarland University, Campus Homburg, Homburg, Germany.
Abstract
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.
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 ChildrenCancer 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.
Authors: Clemens-Magnus Meier; Rhoikos Furtwängler; Dietrich von Schweinitz; Raimund Stein; Nils Welter; Stefan Wagenpfeil; Leo Kager; Jens-Peter Schenk; Christian Vokuhl; Patrick Melchior; Jörg Fuchs; Norbert Graf Journal: Cancers (Basel) Date: 2022-08-14 Impact factor: 6.575
Authors: Mayara Caroline Amorim Fanelli; José Cícero Stocco Guilhen; Alexandre Alberto Barros Duarte; Fernanda Kelly Marques de Souza; Monica Dos Santos Cypriano; Eliana Maria Monteiro Caran; Henrique Manoel Lederman; Maria Teresa de Seixas Alves; Simone de Campos Vieira Abib Journal: Front Pediatr Date: 2022-01-04 Impact factor: 3.418