REALITY OF TREATMENT IN PATIENTS WITH NEPHROBLASTOMA WHO IMMEDIATE TUMOR AND KIDNEY RESECTION BEFORE CHEMOTHERAPY AT THE VIETNAM NATIONAL CHILDREN’S HOSPITAL REALITY OF TREATMENT IN PATIENTS WITH NEPHROBLASTOMA WHO IMMEDIATE TUMOR AND KIDNEY RESECTION BEFORE CHEMOTHERAPY AT THE VIETNAM NATIONAL CHILDREN’S HOSPITAL

Thinh Linh Trinh1, Ngoc Lan Bui2, Minh Dien Tran1,2
1 University of Medicine and Pharmacy, Vietnam National University, Hanoi
2 Vietnam National Children’s Hospital

##plugins.themes.vojs.article.main##

Abstract

Objective: To analyze the clinical characteristics, investigation fndings and evaluate the treatment outcomes of patients treated under the NWTS-5 protocol from 2017 to 2023, as


well as propose solutions to improve nephroblastoma treatment outcomes.


Methods: A retrospective case series study.


Results: 30 patients underwent immediate surgery followed by postoperative chemotherapy under the NWTS-5 protocol, accounting for 22.3% among patients with nephroblastoma for seven years. The median age at diagnosis was 16 months (ranging from 18 days to 50 months). The male-to-female ratio was 2.3:1. The most common reasons for hospital admission were palpable abdominal tumors, abdominal distension, and hematuria. The median preoperative tumor size based on imaging was 256.2 cm³, while the median tumor volume at surgery was 297 cm³. The most common histological type was the mixed type (66.7%). Stage I accounted for 63.3%, stage II for 23.3% and stage III for 13.3%, with all cases classifed as intermediate risk. The median time from diagnosis to surgery was 9.5 days. Ten patients (33.3%) had a statistically signifcant increase in tumor size at the time of surgery with a mean surgical waiting time of 10.4 days, including 2 patients with stage III disease. The mean surgical waiting time of 4 patients with stage III disease was 13 days (11-19 days). The 5-year overall survival (OS) rate was 95.5%, and the 5-year event-free survival (EFS) rate was 92.5%. There was one case of recurrence (3.3%), in which the patient continued treatment and survived. Another patient abandoned treatment after surgery and subsequently died.


Conclusion: Among nephroblastoma patients, 22.3% were treated under the NTWS-5 protocol (immediate surgical). Theses patients had a younger age at disease, small tumor sizes, and high overall and event-free survival rates. To improve the treatment results of nephroblastoma at the National Children’s Hospital, it is necessary to perform additional tumor genetic tests and apply a preoperative chemotherapy regimen (SIOP Umbrella).

##plugins.themes.vojs.article.details##

References

1. Howlader N, Noone AM, Krapcho M et al 2020. SEER Cancer Statistics Review (CSR) 1975-2016. Updated April 9, 2020.
2. Szychot E, Apps J, Pritchard-Jones K (2014). Wilms’ tumor: biology, diagnosis and treatment. Transl Pediatr;3(1):12-24. https://doi.org/10.3978/j.issn.2224-4336.2014.01.09
3. Dome JS, Graf N, Geller JI et al. Advances in Wilms tumor treatment and biology: Progress through international collaboration. J Clin Oncol 2015;33(27):332999-3007.https://doi.org/10.1200/jco.2015.62.1888
4. Servaes SE, Hoffer FA, Smith EA et al.Imaging of Wilms tumor: an update. Pediatric radiology 2019;49(11):1441-1452. https://doi.org/10.1007/s00247-019-04423-3
5. Pritchard-Jones K, Bergeron C, de Camargo B et al. Omission of doxorubicin from the treatment of stage II-III, intermediate-risk Wilms’ tumour (SIOP WT 2001): An open-label, non-inferiority, randomised controlled trial. Lancet 2015;386(9999):1156-1164. https://doi.org/10.1016/s0140-6736(14)62395-3
6. SIOP Nephroblastoma Trial & Study Office. Interim statistical report of Nephroblastoma Clinical Trial and Study 2001, Amsterdam (Netherland) 2015.
7. Ngô Thụy Minh Nhi. Kết quả điều trị bướu Wilms theo phác đồ SIOP 2011, Đại học Y dược Thành Phố Hồ Chí Minh, Luận văn thạc sĩ Y học 2015.
8. Nguyễn Thị Quỳnh Như. Các yếu tố liên quan tới sống còn của bệnh nhi có bướu Wilms tại bệnh viện Nhi Đồng 2, Tạp chí Y Học TP. Hồ Chí Minh 2023:453 -455.
9. Lanzkowsky P, Lipton J M, Fish J D et al. Lanzkowsky’s Manual of Pediatric Hematology and Oncology (Sixth Edition), Academic Press, San Diego 2016: 491-504.
10. Trần Đức Hậu. Nghiên cứu kết quả điều trị u nguyên bào thận theo phác đồ SIOP 2001 tại Bệnh viện Nhi Trung ương, Luận án tiến sĩ y khoa, Đại học Y Hà Nội 2014.
11. Weirich A, Leuschner I, Harms D et al. Clinical impact of histologic subtypes in localized non-anaplastic nephroblastoma treated according to the trial and study SIOP-9/GPOH. Ann Oncol 2001;12(3):311-319.https://doi.org/10.1023/a:1011167924230
12. Pritchard-Jones K, Moroz V, Vujanic G. Treatment and outcome of Wilms tumour patients: an analysis of all cases registered in the UKW3 trial. Annals of Oncology 2012;23(9):2457–2463.https://doi.org/10.1093/annonc/mds025
13. Metzger ML, Dome JS. Current Therapy for Wilms’ Tumor. Oncologist 2005;10(10):815-826.https://doi.org/10.1634/theoncologist.10-10-815
14. Oue T, Fukuzawa M, Okita H et al. Outcome of pediatric renal tumor treated using the Japan Wilms Tumor Study-1 (JWiTS-1) protocol. A report from the JwiTS group. Pediatr Surg Int 2009;25(11):923-929. https://doi.org/10.1007/s00383-009-2449-0
15. Yao W, Xiao X, Gao J et al. Outcomes of Wilms’ tumor in eastern China: 10 years of experience at a single center. Invest Surg 2012;25(3):181-185. https://doi.org/10.3109/08941939.2011.615893
16. Sangkhathat S, Chotsampancharaen T, Kayasut K et al. Outcomes of pediatric nephroblastoma in southern Thailand. Asian Pac J Cancer 2008;9(4):643-647.
17. Dome JS, Fernandez CV, Mullen EA et al. Children’s Oncology Group 2013 Blueprint for Research: Renal tumors. Pediatr Blood Cancer 2013;60(6):994-1000.https://doi.org/10.1002/pbc.24419
18. Nghiêm Ngọc Linh. Đánh giá hiệu quả điều trị phác đồ u nguyên bào thận tại Bệnh viện Nhi Trung ương, Luận văn chuyên khoa cấp 2, Đại học Y Hà Nội 2024.