---
title: "Modified SIOPEL-4 Shows Superior Outcomes in High-Risk Pediatric Hepatoblastoma"
id: "pubmed-42595556"
canonical_url: "https://medichelpline.com/clinical-feed/pubmed-42595556"
content_type: "clinical_feed_article"
specialty: "Oncology"
source_name: "PubMed / NCBI"
source_url: "https://pubmed.ncbi.nlm.nih.gov/42595556/"
doi: "10.3760/cma.j.cn112152-20251121-00580"
published_at: "2026-08-23T00:00:00.000Z"
evidence_level: "Journal Article"
license: "CC-BY-NC-4.0 / Informational Use"
---
# Modified SIOPEL-4 Shows Superior Outcomes in High-Risk Pediatric Hepatoblastoma
## Provenance & Clinical Metadata
- **Canonical URL:** https://medichelpline.com/clinical-feed/pubmed-42595556
- **Specialty:** [Oncology](https://medichelpline.com/clinical-feed/oncology.md)
- **Primary Source:** PubMed / NCBI
- **Source URL:** [Original Journal Publication](https://pubmed.ncbi.nlm.nih.gov/42595556/)
- **DOI:** [10.3760/cma.j.cn112152-20251121-00580](https://doi.org/10.3760%2Fcma.j.cn112152-20251121-00580)
- **Published At:** 2026-08-23T00:00:00.000Z
- **Evidence Rating:** Journal Article
## Executive GIST (TL;DR)
- Study type: retrospective cohort of 99 children (<18 years) with **high-risk hepatoblastoma** treated at Sun Yat-sen University Cancer Center from December 2004 to July 2024. - Three chemotherapy regimens compared: **PLADO** (cisplatin + doxorubicin; n=27), **C5VD/IIV** (alternating cisplatin/5‑FU/vincristine/doxorubicin with irinotecan/ifosfamide/vincristine; n=29), and a **modified SIOPEL‑4** regimen (SIOPEL‑4 with omission of high‑dose cisplatin on day 15; n=43). - Primary comparisons included short‑term objective response rate (ORR), disease control rate (DCR), adverse effects, 3‑year progression‑free survival (PFS) and 3‑year overall survival (OS). - ORRs: PLADO 73.9% (17/23), C5VD/IIV 70.8% (17/24), modified SIOPEL‑4 88.6% (31/35); differences not statistically significant (P = 0.190). - DCRs: PLADO 82.6% (19/23), C5VD/IIV 83.3% (20/24), modified SIOPEL‑4 100% (35/35); modified SIOPEL‑4 superior (P < 0.05). - 3‑year PFS: PLADO 19.2%, C5VD/IIV 37.0%, modified SIOPEL‑4 56.7%; modified SIOPEL‑4 had significantly better PFS (P < 0.05). - 3‑year OS: PLADO 69.2%, C5VD/IIV 55.6%, modified SIOPEL‑4 79.0%; after combining PLADO and C5VD/IIV groups, modified SIOPEL‑4 showed superior PFS and OS (P < 0.05). - Authors conclude modified SIOPEL‑4 offers superior efficacy for high‑risk HB but acknowledge room for further improvement; recommend intensified supportive care and aggressive local therapy, including consideration of upfront liver transplantation for unresectable primary tumors. - Conflict of interest: all authors declared no conflicts. Details on toxicity profiles and specific adverse event rates were summarized in the source but full granular data and certain methodological specifics (e.g., selection criteria for each regimen, statistical methods) were not reported in the abstract.
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more resources Comparative Study Zhonghua Zhong Liu Za Zhi Actions * [ Search in PubMed ](https://pubmed.ncbi.nlm.nih.gov/?term=%22Zhonghua+Zhong+Liu+Za+Zhi%22%5Bjour%5D&sort=date&sort_order=desc) * [ Search in NLM Catalog ](https://www.ncbi.nlm.nih.gov/nlmcatalog?term=%22Zhonghua+Zhong+Liu+Za+Zhi%22%5BTitle+Abbreviation%5D) * [ Add to Search ](https://pubmed.ncbi.nlm.nih.gov/42595556/) . 2026 Aug 23;48(8):1016-1022. doi: 10.3760/cma.j.cn112152-20251121-00580. # [Efficacy comparisons of chemotherapy regimens with different intensities in high-risk pediatric hepatoblastoma] [Article in Chinese] [Y Mao](https://pubmed.ncbi.nlm.nih.gov/?term=Mao+Y&cauthor_id=42595556)[ 1 ](https://pubmed.ncbi.nlm.nih.gov/42595556/#full-view-affiliation-1 "Department of Pediatric Oncology, State Key Laboratory of Oncology in South China, Guangdong Provincial Clinical Research Center for Cancer, Sun Yat-sen University Cancer Center, Guangzhou 510060, China."), [C G Zeng](https://pubmed.ncbi.nlm.nih.gov/?term=Zeng+CG&cauthor_id=42595556)[ 1 ](https://pubmed.ncbi.nlm.nih.gov/42595556/#full-view-affiliation-1 "Department of Pediatric Oncology, State Key Laboratory of Oncology in South China, Guangdong Provincial Clinical Research Center for Cancer, Sun Yat-sen University Cancer Center, Guangzhou 510060, China."), [Y Li](https://pubmed.ncbi.nlm.nih.gov/?term=Li+Y&cauthor_id=42595556)[ 1 ](https://pubmed.ncbi.nlm.nih.gov/42595556/#full-view-affiliation-1 "Department of Pediatric Oncology, State Key Laboratory of Oncology in South China, Guangdong Provincial Clinical Research Center for Cancer, Sun Yat-sen University Cancer Center, Guangzhou 510060, China."), [J T Huang](https://pubmed.ncbi.nlm.nih.gov/?term=Huang+JT&cauthor_id=42595556)[ 1 ](https://pubmed.ncbi.nlm.nih.gov/42595556/#full-view-affiliation-1 "Department of Pediatric Oncology, State Key Laboratory of Oncology in South China, Guangdong Provincial Clinical Research Center for Cancer, Sun Yat-sen University Cancer Center, Guangzhou 510060, China."), [F F Sun](https://pubmed.ncbi.nlm.nih.gov/?term=Sun+FF&cauthor_id=42595556)[ 1 ](https://pubmed.ncbi.nlm.nih.gov/42595556/#full-view-affiliation-1 "Department of Pediatric Oncology, State Key Laboratory of Oncology in South China, Guangdong Provincial Clinical Research Center for Cancer, Sun Yat-sen University Cancer Center, Guangzhou 510060, China."), [J Wang](https://pubmed.ncbi.nlm.nih.gov/?term=Wang+J&cauthor_id=42595556)[ 1 ](https://pubmed.ncbi.nlm.nih.gov/42595556/#full-view-affiliation-1 "Department of Pediatric Oncology, State Key Laboratory of Oncology in South China, Guangdong Provincial Clinical Research Center for Cancer, Sun Yat-sen University Cancer Center, Guangzhou 510060, China."), [J Zhu](https://pubmed.ncbi.nlm.nih.gov/?term=Zhu+J&cauthor_id=42595556)[ 1 ](https://pubmed.ncbi.nlm.nih.gov/42595556/#full-view-affiliation-1 "Department of Pediatric Oncology, State Key Laboratory of Oncology in South China, Guangdong Provincial Clinical Research Center for Cancer, Sun Yat-sen University Cancer Center, Guangzhou 510060, China."), [S Y Lu](https://pubmed.ncbi.nlm.nih.gov/?term=Lu+SY&cauthor_id=42595556)[ 1 ](https://pubmed.ncbi.nlm.nih.gov/42595556/#full-view-affiliation-1 "Department of Pediatric Oncology, State Key Laboratory of Oncology in South China, Guangdong Provincial Clinical Research Center for Cancer, Sun Yat-sen University Cancer Center, Guangzhou 510060, China."), [Y Z Zhang](https://pubmed.ncbi.nlm.nih.gov/?term=Zhang+YZ&cauthor_id=42595556)[ 1 ](https://pubmed.ncbi.nlm.nih.gov/42595556/#full-view-affiliation-1 "Department of Pediatric Oncology, State Key Laboratory of Oncology in South China, Guangdong Provincial Clinical Research Center for Cancer, Sun Yat-sen University Cancer Center, Guangzhou 510060, China."), [Z Z Zhen](https://pubmed.ncbi.nlm.nih.gov/?term=Zhen+ZZ&cauthor_id=42595556)[ 1 ](https://pubmed.ncbi.nlm.nih.gov/42595556/#full-view-affiliation-1 "Department of Pediatric Oncology, State Key Laboratory of Oncology in South China, Guangdong Provincial Clinical Research Center for Cancer, Sun Yat-sen University Cancer Center, Guangzhou 510060, China.") Affiliations Expand ### Affiliation * 1 Department of Pediatric Oncology, State Key Laboratory of Oncology in South China, Guangdong Provincial Clinical Research Center for Cancer, Sun Yat-sen University Cancer Center, Guangzhou 510060, China. * PMID: **42595556** * DOI: [ 10.3760/cma.j.cn112152-20251121-00580 ](https://doi.org/10.3760/cma.j.cn112152-20251121-00580) Item in Clipboard Comparative Study # [Efficacy comparisons of chemotherapy regimens with different intensities in high-risk pediatric hepatoblastoma] [Article in Chinese] Y Mao et al. Zhonghua Zhong Liu Za Zhi. 2026. Show details Display options Display options Format Abstract PubMed PMID Zhonghua Zhong Liu Za Zhi Actions * [ Search in PubMed ](https://pubmed.ncbi.nlm.nih.gov/?term=%22Zhonghua+Zhong+Liu+Za+Zhi%22%5Bjour%5D&sort=date&sort_order=desc) * [ Search in NLM Catalog ](https://www.ncbi.nlm.nih.gov/nlmcatalog?term=%22Zhonghua+Zhong+Liu+Za+Zhi%22%5BTitle+Abbreviation%5D) * [ Add to Search ](https://pubmed.ncbi.nlm.nih.gov/42595556/) . 2026 Aug 23;48(8):1016-1022. doi: 10.3760/cma.j.cn112152-20251121-00580. ### Authors [Y Mao](https://pubmed.ncbi.nlm.nih.gov/?term=Mao+Y&cauthor_id=42595556)[ 1 ](https://pubmed.ncbi.nlm.nih.gov/42595556/#short-view-affiliation-1 "Department of Pediatric Oncology, State Key Laboratory of Oncology in South China, Guangdong Provincial Clinical Research Center for Cancer, Sun Yat-sen University Cancer Center, Guangzhou 510060, China."), [C G Zeng](https://pubmed.ncbi.nlm.nih.gov/?term=Zeng+CG&cauthor_id=42595556)[ 1 ](https://pubmed.ncbi.nlm.nih.gov/42595556/#short-view-affiliation-1 "Department of Pediatric Oncology, State Key Laboratory of Oncology in South China, Guangdong Provincial Clinical Research Center for Cancer, Sun Yat-sen University Cancer Center, Guangzhou 510060, China."), [Y Li](https://pubmed.ncbi.nlm.nih.gov/?term=Li+Y&cauthor_id=42595556)[ 1 ](https://pubmed.ncbi.nlm.nih.gov/42595556/#short-view-affiliation-1 "Department of Pediatric Oncology, State Key Laboratory of Oncology in South China, Guangdong Provincial Clinical Research Center for Cancer, Sun Yat-sen University Cancer Center, Guangzhou 510060, China."), [J T Huang](https://pubmed.ncbi.nlm.nih.gov/?term=Huang+JT&cauthor_id=42595556)[ 1 ](https://pubmed.ncbi.nlm.nih.gov/42595556/#short-view-affiliation-1 "Department of Pediatric Oncology, State Key Laboratory of Oncology in South China, Guangdong Provincial Clinical Research Center for Cancer, Sun Yat-sen University Cancer Center, Guangzhou 510060, China."), [F F Sun](https://pubmed.ncbi.nlm.nih.gov/?term=Sun+FF&cauthor_id=42595556)[ 1 ](https://pubmed.ncbi.nlm.nih.gov/42595556/#short-view-affiliation-1 "Department of Pediatric Oncology, State Key Laboratory of Oncology in South China, Guangdong Provincial Clinical Research Center for Cancer, Sun Yat-sen University Cancer Center, Guangzhou 510060, China."), [J Wang](https://pubmed.ncbi.nlm.nih.gov/?term=Wang+J&cauthor_id=42595556)[ 1 ](https://pubmed.ncbi.nlm.nih.gov/42595556/#short-view-affiliation-1 "Department of Pediatric Oncology, State Key Laboratory of Oncology in South China, Guangdong Provincial Clinical Research Center for Cancer, Sun Yat-sen University Cancer Center, Guangzhou 510060, China."), [J Zhu](https://pubmed.ncbi.nlm.nih.gov/?term=Zhu+J&cauthor_id=42595556)[ 1 ](https://pubmed.ncbi.nlm.nih.gov/42595556/#short-view-affiliation-1 "Department of Pediatric Oncology, State Key Laboratory of Oncology in South China, Guangdong Provincial Clinical Research Center for Cancer, Sun Yat-sen University Cancer Center, Guangzhou 510060, China."), [S Y Lu](https://pubmed.ncbi.nlm.nih.gov/?term=Lu+SY&cauthor_id=42595556)[ 1 ](https://pubmed.ncbi.nlm.nih.gov/42595556/#short-view-affiliation-1 "Department of Pediatric Oncology, State Key Laboratory of Oncology in South China, Guangdong Provincial Clinical Research Center for Cancer, Sun Yat-sen University Cancer Center, Guangzhou 510060, China."), [Y Z Zhang](https://pubmed.ncbi.nlm.nih.gov/?term=Zhang+YZ&cauthor_id=42595556)[ 1 ](https://pubmed.ncbi.nlm.nih.gov/42595556/#short-view-affiliation-1 "Department of Pediatric Oncology, State Key Laboratory of Oncology in South China, Guangdong Provincial Clinical Research Center for Cancer, Sun Yat-sen University Cancer Center, Guangzhou 510060, China."), [Z Z Zhen](https://pubmed.ncbi.nlm.nih.gov/?term=Zhen+ZZ&cauthor_id=42595556)[ 1 ](https://pubmed.ncbi.nlm.nih.gov/42595556/#short-view-affiliation-1 "Department of Pediatric Oncology, State Key Laboratory of Oncology in South China, Guangdong Provincial Clinical Research Center for Cancer, Sun Yat-sen University Cancer Center, Guangzhou 510060, China.") ### Affiliation * 1 Department of Pediatric Oncology, State Key Laboratory of Oncology in South China, Guangdong Provincial Clinical Research Center for Cancer, Sun Yat-sen University Cancer Center, Guangzhou 510060, China. * PMID: **42595556** * DOI: [ 10.3760/cma.j.cn112152-20251121-00580 ](https://doi.org/10.3760/cma.j.cn112152-20251121-00580) Item in Clipboard Full text links Cite Display options Display options Format Abstract PubMed PMID ## Abstract in [ English, ](https://pubmed.ncbi.nlm.nih.gov/42595556/#eng-abstract) [ Chinese ](https://pubmed.ncbi.nlm.nih.gov/42595556/#zho-abstract) **Objective:** To evaluate the efficacy of different chemotherapy regimens in children with high-risk hepatoblastoma (HB). **Methods:** A retrospective cohort study was conducted on 99 patients under 18 years old with high-risk HB treated at Sun Yat-sen University Cancer Center between December 2004 and July 2024. The patients were divided into three groups: the PLADO regimen (cisplatin in combination with doxorubicin, _n_ =27), the C5VD/IIV regimen (alternating cycles of cisplatin/5-flourouracil/vincristine/doxorubicin with irinotecan/ifosfamide/vincristine, _n_ =29), and the modified SIOPEL-4 regimen (the original SIOPEL-4 regimen with omitted high-dose cisplatin on day15, _n_ =43). Short-term efficacy, adverse effects, and long-term survival were compared among the three groups. **Results:** The objective response rates for the PLADO, C5VD/IIV, and modified SIOPEL-4 groups were 73.9% (17/23), 70.8% (17/24), and 88.6% (31/35), respectively, with no statistically significant difference among the three groups (_P_ =0.190). The disease control rates were 82.6% (19/23), 83.3% (20/24), and 100.0% (35/35), the 3-year progression-free survival (PFS) rates were 19.2%, 37.0%, and 56.7%, and the 3-year overall survival (OS) rates were 69.2%, 55.6%, and 79.0%, respectively. The modified SIOPEL-4 group showed significantly better disease control rate and PFS rate compared withthe other two groups (both _P_ ＜0.05). After merging the PLADO and C5VD/IIV groups, the modified SIOPEL-4 group demonstrated significantly superior PFS and OS rates (both _P_ ＜0.05). **Conclusions:** The efficacy of modified SIOPEL-4 regimen in treating high-risk HB is superior compared with those of PLADO and C5VD/IIV regimens, but there remains room for further improvement in efficacy. Intensifying supportive care and pursuing aggressive local therapies, especially upfront liver transplantation for locally inoperable tumors, may further improve outcomes. **目的：** 评价不同强度的化疗方案治疗儿童高危肝母细胞瘤（HB）的疗效。 **方法：** 采用回顾性队列研究方法，收集2004年12月至2024年7月在中山大学肿瘤防治中心诊治的18岁以下高危HB患者99例，其中采用PLADO方案（顺铂+阿霉素）27例，C5VD/IIV方案（顺铂+氟尿嘧啶+长春新碱+阿霉素与伊立替康+异环磷酰胺+长春新碱交替化疗）29例，改良SIOPEL-4方案（在欧洲国际儿童肝脏肿瘤协作组-4方案的基础上取消第15天的大剂量顺铂化疗）43例，比较3组的近期疗效、不良反应及患者的远期生存情况。 **结果：** PLADO方案组、C5VD/IIV方案组和改良SIOPEL-4方案组的化疗客观缓解率分别为73.9%（17/23）、70.8%（17/24）和88.6%（31/35），3组间比较差异无统计学意义（ _P_ =0.190）。疾病控制率分别为82.6%（19/23）、83.3%（20/24）和100.0%（35/35），3年无进展生存率分别为19.2%、37.0%和56.7%，3年总生存率分别为69.2%、55.6%和79.0%。3组比较，改良SIOPEL-4方案组的疾病控制率和无进展生存率优于其他两组（均 _P_ ＜0.05）。将PLADO方案组与C5VD/IIV方案组合并后与改良SIOPEL-4方案组比较，改良SIOPEL-4方案组的无进展生存率和总生存率均具有明显优势（均 _P_ ＜0.05）。 **结论：** 改良SIOPEL-4方案治疗高危HB疗效优于PLADO和C5VD/IIV方案，但疗效仍有进一步提升的空间。加强支持治疗和局部治疗，特别是对术前化疗后原发灶仍不可手术的患者尽可能采用肝移植，有助于进一步提高高危HB的疗效。. 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