| Evidence-based clinical practice guidelines for pediatric hepatoblastoma: a systematic review and expert recommendations from Korea |
Jin Kyung Suh1 
, Jisun Hwang2
, Hee Mang Yoon2
, Kyong Ihn3
, Jung-Man Namgoong4
, Jeong A. Park5
, Jung-Sun Kim6
, Hyoung Soo Choi1
, Sung Hye Park7
, Dong Ah Park8
, Kyung-Nam Koh9
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1Department of Pediatrics, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seongnam, Korea
2Department of Radiology and Research Institute of Radiology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
3Division of Pediatric Surgery, Severance Children's Hospital, Department of Surgery, Yonsei University College of Medicine, Seoul, Korea
4Department of Pediatric Surgery, Asan Medical Center Children’s Hospital, University of Ulsan College of Medicine, Seoul, Korea
5Department of Pediatrics, Inha University Hospital, Incheon, Korea
6Department of Pathology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
7Department of Pathology, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea
8Division of Healthcare Research, National Evidence-based Healthcare Collaborating Agency (NECA), Seoul, Korea
9Division of Pediatric Hematology/Oncology, Department of Pediatrics, Asan Medical Center Children’s Hospital, University of Ulsan College of Medicine, Seoul, Korea |
Correspondence:
Kyung-Nam Koh, Email: pedkkn@amc.seoul.kr
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Received: 23 August 2026 • Revised: 22 September 2026 • Accepted: 29 September 2026 |
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| Abstract |
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Hepatoblastoma (HB) is the most common primary liver malignancy in children, predominantly affecting those younger than 3 years of age. Although the overall survival rate has improved significantly over the past few decades, standardized clinical practice guidelines for HB in Korea have been lacking. This clinical practice guideline was developed to provide evidence-based recommendations for the diagnosis and treatment of HB in children. A multidisciplinary working group comprising pediatric hematologists/oncologists, radiologists, surgeons, pathologists, and methodologists systematically reviewed the literature published up to May 2023. For pretreatment imaging, both abdominal computed tomography and magnetic resonance imaging are useful for PRE-Treatment EXTent of disease (PRETEXT) staging, with each modality offering distinct advantages. PRETEXT staging at diagnosis is recommended as it provides valuable prognostic information. For standard-risk HB (PRETEXT I–III without metastasis and alpha-fetoprotein ≥100 ng/mL), cisplatinbased chemotherapy without doxorubicin is recommended over cisplatin-doxorubicin combination therapy, as it demonstrates equivalent survival outcomes with fewer adverse effects. In carefully selected patients who are eligible for complete resection at diagnosis, upfront surgery without neoadjuvant chemotherapy should be considered to spare unnecessary chemotherapy and its toxicities. For patients with residual pulmonary metastases after chemotherapy, pulmonary metastasectomy should be considered. Sodium thiosulfate administration following cisplatin should be considered to reduce ototoxicity, although the supporting evidence is derived predominantly from standard-risk patients and its availability in Korea remains limited. Regarding histological subtypes, well-differentiated fetal HB is associated with favorable outcomes and may be treated with surgery alone, whereas the small cell undifferentiated pattern is no longer classified as a HB subtype and requires immunohistochemical and molecular testing to distinguish it from malignant rhabdoid tumor and other small round cell tumors. This guideline represents the first evidence-based clinical practice guideline for pediatric HB in Korea. It aims to standardize the diagnostic and therapeutic approaches, ultimately improving outcomes for Korean children with HB. Given the rarity of this tumor, international collaboration and prospective data collection remain essential for future guideline updates. |
| Key Words:
Hepatoblastoma, Child, Practice guideline, Drug therapy, Prognosis |
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