Surgical resection of huge hepatic hepatoblastoma involving second, third hepatic hilum: a study of 59 cases

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Abstract Background A considerable proportion of pediatric giant hepatoblastoma cases involve the second and third hepatic portals (GHSTP), which were previously considered unresectable. Surgical management of such GHSTP-involved cases is associated with significant technical challenges. This retrospective study was designed to assess the feasibility and surgical approaches for pediatric giant hepatoblastoma with involvement of the second and third hepatic portals. Methods From January 2006 to December 2024, 59 pediatric patients with giant hepatoblastoma involving the second and third hepatic portals (GHSTP) underwent partial hepatectomy. The therapeutic outcomes of these cases were retrospectively analyzed. Results All 59 patients underwent successful liver tumor resection. The operative duration ranged from 160 to 350 minutes, with a mean of 270 minutes. First hepatic portal clamping was performed in all cases. The resection approaches were as follows: 13 cases underwent resection of segments V-VIII; 12 cases, resection of segments IV-VIII; 6 cases, resection of segments I-IV; 10 cases, resection of segments I, IV, and VIII; 8 cases, resection of segments I, IV, V, and VIII; 4 cases, resection of segment I plus segments V-VIII; and 6 cases, resection of segment I plus segments IV-VIII. There was no mortality, and postoperative complications occurred in 8 cases. Conclusion Resection of giant hepatoblastoma involving the second and third hepatic portals remains technically challenging; however, with meticulous and accurate preoperative assessment coupled with a thorough understanding of hepatic anatomy, such tumor resection is feasible and can be performed with acceptable safety. Moreover, complete resection of this subset of tumors may effectively improve the long-term survival outcomes of patients with hepatoblastoma.
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Surgical management of such GHSTP-involved cases is associated with significant technical challenges. This retrospective study was designed to assess the feasibility and surgical approaches for pediatric giant hepatoblastoma with involvement of the second and third hepatic portals. Methods From January 2006 to December 2024, 59 pediatric patients with giant hepatoblastoma involving the second and third hepatic portals (GHSTP) underwent partial hepatectomy. The therapeutic outcomes of these cases were retrospectively analyzed. Results All 59 patients underwent successful liver tumor resection. The operative duration ranged from 160 to 350 minutes, with a mean of 270 minutes. First hepatic portal clamping was performed in all cases. The resection approaches were as follows: 13 cases underwent resection of segments V-VIII; 12 cases, resection of segments IV-VIII; 6 cases, resection of segments I-IV; 10 cases, resection of segments I, IV, and VIII; 8 cases, resection of segments I, IV, V, and VIII; 4 cases, resection of segment I plus segments V-VIII; and 6 cases, resection of segment I plus segments IV-VIII. There was no mortality, and postoperative complications occurred in 8 cases. Conclusion Resection of giant hepatoblastoma involving the second and third hepatic portals remains technically challenging; however, with meticulous and accurate preoperative assessment coupled with a thorough understanding of hepatic anatomy, such tumor resection is feasible and can be performed with acceptable safety. Moreover, complete resection of this subset of tumors may effectively improve the long-term survival outcomes of patients with hepatoblastoma. Surgical resection huge hepatic hepatoblastoma hepatic hilum Figures Figure 1 Figure 2 Introduction Hepatoblastoma is the most common malignant liver tumor in children, accounting for 1.3% of malignant tumors in children(Clinical effectiveness of multimodality treatment on advanced pediatric hepatoblastom). Pediatric hepatoblastoma doesn’t have clear symptoms at early stage, and usually won’t be identified until it causes clear abdominal mass or compression of surrounding organs(1, 2). Quite a number of pediatric giant hepatoblastoma cases involve the second and third hepatic portals (GHSTP), which had been thought to be unresectable because uncontrollable massive hemorrhage is likely to occur during operation. With the development of pediatric liver surgery and the improvement of perioperative treatment levels, the giant hepatoblastoma involving the second and third hepatic portals is transiting from unresectable to resectable. However, there remains high operative risk and mortality rate. This operation is technically demanding, need to have a good understanding of intrahepatic anatomy. This study explored the operative feasibility and methods of this type of tumors expecting to expand the surgical indications and improve the efficacy of pediatric hepatoblastoma treatment. METHODS Clinical data From January 2006 to December 2024, a total of 59 pediatric patients diagnosed with hepatoblastoma and undergoing partial hepatectomy for giant hepatoblastoma with synchronous portal vein tumor thrombosis (GHSTP) were retrospectively enrolled in our hospital (Figure 1). The age of these patients ranged from 3 months to 15 years, with a mean age of 4.1 years. The diagnosis of hepatoblastoma was histopathologically confirmed in all cases. All 59 patients received preoperative chemotherapy for 1 to 6 courses, with treatment continued until no further reduction in tumor size was observed. The mean maximum diameter of the tumors was 15.2 cm, ranging from 8.5 cm to 22.6 cm (Table 1). Table 1 Demographic data of 59 children Parameter Number Proportion(%) Clinical characteristics Gender boy girl 22 37 37.2 62.7 Age at operation 1year 10 25 24 16.9 42.3 40.7 Cycles of postoperative chemotherapy 0 1-3 >3 0 36 23 61.0 39.0 Pre-operative laboratory examination Red blood cells(10 12 /L) 3.58±0.61 white blood cell(10 9 /L) 5.04±2.93 Platelet (10 9 /L) 376.80±2131 AFP at diagnosis(ng/ml) 715.96±429.95 Histology (main component) Epithelial Mixed Others 35 20 4 59.3 33.8 6.9 Diameter (cm) 15.2(8.5-22.6) Vascular invasion No Yes 37 22 62.7 37.2 Metastases No Yes 52 7 88.1 11.9 Surgical and perioperative treatment method All pediatric patients with GHSTP underwent routine B-mode ultrasound, contrast-enhanced computed tomography (CT), or magnetic resonance imaging (MRI) to evaluate tumor location, size, and relationship with major hepatic vasculature. A subcostal incision was performed to achieve adequate release of adhesions and ligaments surrounding the liver. Preoperative vascular control was established by placing occluders at the portal vein, as well as the suprahepatic and infrahepatic segments of the inferior vena cava, respectively. After dissecting and freeing the ligaments, the short hepatic veins were transected, and the liver was carefully separated from the inferior vena cava. Resection margins were preoperatively planned based on tumor extent. The superficial hepatic parenchyma was incised using electrocoagulation, followed by gentle fragmentation of liver tissue with right-angled forceps. Unresected residual vascular or biliary structures within the liver parenchyma were identified as part of the hepatic hilar system and meticulously ligated. During tumor resection, intermittent clamping of the hepatic portal triad was performed. If an inferior vena cava defect was identified after tumor removal, primary repair was conducted under vascular occlusion. Follow up methods and endpoints All patients were subjected to regular postoperative follow-up. During the first 2 years after surgery, serum alpha-fetoprotein (AFP) levels and B-mode ultrasonography were assessed monthly, and computed tomography (CT) was performed every 3 months. Thereafter, follow-up intervals were extended to 3–6 months. The diagnostic criteria for intrahepatic recurrence were defined as the presence of newly identified lesions on ultrasonography or CT combined with an elevation in serum AFP levels. However, intrahepatic recurrence was suspected in cases where CT revealed atypical lesions, or when serum AFP levels were elevated without corresponding new lesions detected by ultrasonography or CT; in such instances, further evaluation with magnetic resonance imaging (MRI) or hepatic angiography was required. The primary endpoint of this study was tumor recurrence. Disease-free survival (DFS) was defined as the time interval from the date of surgery to the date of confirmed tumor recurrence or death (whichever occurred first). The follow-up duration ranged from 16 to 220 months, with a median follow-up time of 136.7 months. Statistics Disease-free survival (DFS) was evaluated using the Kaplan–Meier method, with analysis restricted to patients who survived the postoperative period (excluding those who died postoperatively). A two-tailed P value < 0.05 was considered statistically significant. Ethics, consent and permissions This study was designed as a retrospective analysis utilizing de-identified registry data. In accordance with the guidelines of the local institutional review board (IRB), this study was exempt from formal ethical approval due to its retrospective nature and the use of anonymized data. Written informed consent for the collection, storage, and research use of clinical data was obtained from all patients or their legal guardians during the initial hospitalization period. Result Surgical results and postoperative complications Liver tumor resection was successfully performed in all 59 pediatric patients, with all achieving clear (R0) resection margins. Intraoperative hemodynamic parameters remained stable in all patients, with no occurrences of shock, liver failure, or perioperative mortality observed. The operative duration ranged from 160 to 350 minutes, with a mean of 270 minutes. Intraoperative blood loss was 40–500 mL, and the mean perioperative red blood cell (RBC) transfusion volume was 1.3 units (range, 1 to 4 units). RBC transfusion was required in 81.6% of patients (n=40). Intermittent hepatic portal triad clamping was performed in all pediatric patients, with a total clamping duration ranging from 15 to 35 minutes (mean, 22 minutes). The clamping protocol was as follows: 15 patients underwent a single clamping episode, 26 patients required two episodes, and 18 patients needed more than two episodes. Each individual clamping interval did not exceed 20 minutes. The resection approaches adopted were as follows: 13 cases underwent resection of segments V to VIII; 12 cases underwent resection of segments IV to VIII; 6 cases underwent resection of segments I to IV; 10 cases underwent resection of segments I, IV, and VIII; 8 cases underwent resection of segments I, IV, V, and VIII; 4 cases underwent resection of segment I plus segments V to VIII; and 6 cases underwent resection of segment I plus segments IV to VIII. All pediatric patients achieved an uneventful recovery after the operation. There was no mortality, and postoperative complications occurred in 8 cases, specifically: 2 cases of abdominal hemorrhage, 1 case of intra-abdominal abscess, 1 case of bile leakage, 2 cases of intestinal obstruction, and 2 cases of transient respiratory insufficiency. All patients exhibited liver function impairment, which was primarily characterized by elevated alanine transaminase (ALT) levels; decreased albumin levels were observed in some patients. Generally, liver function returned to normal within 2–4 weeks postoperatively. The median length of hospital stay ranged from 8 to 16 days, with a mean of 12.5 days (Figures 1 and 2). Postoperative survival status All children were regularly followed up, and the final follow-up time was October 2024. In all children, the one-year non-neoplasm survival rate was 91.5%, 3-year non-neoplasm survival rate was 71.2%, and 5-year non-neoplasm survival rate was 32.5%. The longest non-neoplasm survival rate was 109 months (this patient is still living in a non-neoplasm healthy state). 59 patients received standard postoperative chemotherapy and did regular outpatient check-ups after chemotherapy, recurrence was found in 30 (51.0%) of them and chemotherapy was continued after finding of recurrence. In the six patients with pulmonary metastases, 3 patients showed absence of metastases after surgery and chemotherapy, and 3 patients died within one year after surgery. Discussion Hepatoblastoma is the most common malignant liver tumor in children, accounting for 0.8%–2% of all pediatric malignant tumors [3, 4]. Over the past two decades, with advancements in hepatic surgical techniques, improvements in anesthetic management, and the introduction of novel chemotherapeutic agents, the overall survival rate of patients with hepatoblastoma has significantly improved. The 3-year overall survival rate is approximately 70% [3, 4–6]. However, hepatoblastoma frequently invades major blood vessels, primarily due to its presentation at an advanced stage upon diagnosis and its rapid growth rate. This poses challenges in defining appropriate surgical indications and renders the surgical procedures themselves highly demanding. Furthermore, the occurrence of multiple postoperative complications and a high mortality rate have hindered the widespread implementation of surgical intervention. The aim of this study was to analyze and summarize 59 cases of giant hepatoblastoma in pediatric patients involving the second and third hepatic portals, with the purpose of discussing the key points and challenges in surgical techniques for this specific group of infant patients and improving the overall survival rate. Preoperative liver assessment Preoperative evaluation of each patient is essential, encompassing assessments of the tumor characteristics, surgical feasibility, liver function, residual liver volume, and the patient's general condition, among other factors. Imaging modalities such as B-mode ultrasound, computed tomography (CT), and magnetic resonance imaging (MRI) facilitate a comprehensive understanding of the hepatic vascular anatomy, effectively enhance the precision of surgery, and are crucial for the selection and formulation of surgical plans as well as specific intraoperative management. Three-dimensional computed tomography (3D CT) imaging enables clear visualization of major blood vessels, including the portal venous system, inferior vena cava, and hepatic veins. Avoiding injury to these vessels is critical in surgeries involving the second and third hepatic portals. Of particular importance is the relationship between the left, middle, and right hepatic veins at the second hepatic portal and the tumor, as well as whether these vessels have been invaded by the tumor. Thus, preoperative imaging evaluation is of great significance. All patients in this cohort underwent preoperative three-dimensional computed tomography (3D CT) reconstruction. The high-quality imaging enabled clearer visualization of the tumor boundaries. In particular, based on the hepatic vascular imaging, we could delineate the relationship between the tumors and the portal veins as well as the hepatic veins, and more accurately assess the feasibility of successful resection prior to surgery. Management of hepatic hilum during operation Giant hepatic tumors, particularly those located in the central liver segments (IV, V, VIII) and the caudate lobe (I), often involve the retro hepatic inferior vena cava, hepatic veins, and short hepatic veins. Avoiding injury to these vessels and effectively controlling bleeding in case of iatrogenic damage are critical to the success of the operation. First, adequate exposure of the second hepatic portal and the tumor is essential to enable resection under direct visualization. Second, during hepatic resection, it is preferable to manage the main hepatic veins and short hepatic veins outside the liver parenchyma, as this facilitates clear dissection and prevents iatrogenic injury and bleeding. For resection of giant hepatoblastoma in the right liver, the right hepatic lobe should be mobilized to the right wall of the inferior vena cava, followed by resection of several short hepatic veins and the retrohepatic inferior vena cava in a bottom-up sequence. The space between the root of the right hepatic vein and the inferior vena cava should be carefully dissected to allow placement of a clamp in the suprahepatic vena cava. The caudate lobe is located in a deep anatomical position, adjacent to the inferior vena cava, hepatic veins, and the first hepatic portal. In this cohort of giant hepatoblastoma cases, 34 involved the caudate lobe: 8 underwent complete caudate lobe resection combined with resection of other liver segments, and 26 underwent partial caudate lobe resection with resection of other segments. Intraoperatively, 5–7 thick short hepatic veins are typically encountered, which drain into the orifices of the three main hepatic veins; care must be taken to avoid injury to the right and middle hepatic veins. Resection of the caudate lobe and segments VI or VII is performed under occlusion of the first hepatic portal. To ensure surgical success, hepatic portal occlusion is often required, and strict control of the duration and frequency of occlusion is essential for operative safety. The key technical points for surgeries involving the second and third hepatic portals can be summarized as follows:(1) Adequately mobilize the liver to expose the extent of the tumor and major blood vessels in the second and third hepatic portals;(2) Preset occluders at the first and second hepatic portals, and perform intermittent occlusion when necessary; (3) Protect the hepatic venous return vessels and ensure a sufficient residual liver volume. Precautions are as follows: (1) Adequately ligate the vascular branches to prevent wound bleeding and oozing after tumor resection; (2) The duration of portal occlusion should not be excessively long, generally within 20 minutes; (3) Carefully inspect the inflow and outflow vessels of the residual liver postoperatively to ensure adequate hepatic perfusion and venous return. Two-stage operation If preoperative evaluation indicates that the tumor has invaded multiple major vessels, or that the tumor volume exceeds 80% of the total liver volume with insufficient residual liver function postoperatively, a two-stage surgical approach may be considered for such pediatric patients. International researchers have reported that portal vein branch embolization can be performed when preoperative assessment reveals insufficient residual liver volume, followed by hepatic tumor resection once the contralateral normal liver parenchyma has hypertrophied [7–9]. Liver tumor resection following phase I transcatheter arterial chemoembolization (TACE) is also regarded as an effective therapeutic strategy for the management of large unresectable hepatic tumors [10-12]. We recently admitted a pediatric patient with a giant right hepatic lobe hepatoblastoma. This patient had undergone 5 courses of standard chemotherapy at an external hospital; however, despite the treatment, the tumor showed minimal shrinkage. Given the massive size of the tumor, computational analysis indicated that the estimated residual liver volume after resection would be less than 20%, which would be insufficient to sustain the patient’s hepatic functional requirements. Thus, in the first-stage procedure, both the hepatic artery and right portal vein branch were ligated. A follow-up CT scan at 2 weeks postoperatively revealed compensatory hypertrophy of the patient’s left hepatic lobe, with no significant tumor progression. Subsequent resection of the giant right hepatic tumor was performed successfully; pathological examination confirmed the diagnosis of fetal-type hepatoblastoma, and the patient achieved an uneventful recovery postoperatively. In summary, resection of giant hepatoblastoma involving the second and third hepatic portals is technically challenging. However, with detailed and accurate preoperative evaluation and a thorough understanding of hepatic anatomy, such tumor resection is feasible and can be performed safely. Complete resection of this type of tumor may effectively improve the long-term survival rate of patients with hepatoblastoma. Declarations Compliance with Ethical Standards: Funding source: There was no funding source for this study. The corresponding author had full access to all the data in the study and had final responsibility for the decision to submit for publication. Conflict of interest statement: None declared Ethical approval: All procedures performed in studies were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. Informed consent: All patients signed an infprmed consent approved by the institutional Review Board. Author Contribution Chen Zhen and Qiao Guoliang designed the study, collected and analyzed data, and drafted the manuscript.Ge Juntao contributed to data interpretation, and revised the manuscript critically for important intellectual content.Chen Xinghai supervised the study and approved the final version of the manuscript.Li Long participated in study design, conducted literature review, and edited the manuscript.All authors reviewed the manuscript. Acknowledgments: We thank those patients who supported our study and authors who provided us with the full-text. References Cai M, Liu Y, Wang Y, et al. Three-dimensional reconstruction combined with virtual surgery for pediatric giant hepatoblastoma involving hepatic hilum: a single-center experience. J Pediatr Surg. 2023;58(6):1118-1124. DOI: 10.1016/j.jpedsurg.2023.01.023 Vasireddi AK, Leo ME, Squires JH. Magnetic resonance imaging of pediatric liver tumors. Pediatr Radiol. 2022 Feb;52(2):177-188. doi: 10.1007/s00247-021-05058-z. Epub 2021 Apr 14. PMID: 33852026. Srinivasan S, Prasad M, Parambil BC, Shrimal A, Gollamudi VRM, Subramani V, Ramadwar M, Khanna N, Baheti AD, Gala K, Patil V, Laskar S, Qureshi S, Chinnaswamy G. Treatment outcomes and prognostic factors in children with hepatoblastoma using a risk-stratified approach. Pediatr Blood Cancer. 2023 Jul;70(7):e30302. doi: 10.1002/pbc.30302. Epub 2023 Apr 12. PMID: 37046413. Meyers RL, Maibach R, Hiyama E, Häberle B, Krailo M, Rangaswami A, Aronson DC, Malogolowkin MH, Perilongo G, von Schweinitz D, Ansari M, Lopez-Terrada D, Tanaka Y, Alaggio R, Leuschner I, Hishiki T, Schmid I, Watanabe K, Yoshimura K, Feng Y, Rinaldi E, Saraceno D, Derosa M, Czauderna P. Risk-stratified staging in paediatric hepatoblastoma: a unified analysis from the Children's Hepatic tumors International Collaboration. Lancet Oncol. 2017 Jan;18(1):122-131. doi: 10.1016/S1470-2045(16)30598-8. Epub 2016 Nov 22. PMID: 27884679; PMCID: PMC5650231. Kremer N, Walther AE, Tiao GM. Management of hepatoblastoma: an update. CURR OPIN PEDIATR. 2014 2014-06-01;26(3):362-9. Ren X, Li H, Diao M, Xu H, Li L. Impact of microscopically margin-positive resection on survival in children with hepatoblastoma after hepatectomy: a retrospective cohort study. Int J Clin Oncol. 2020 Apr;25(4):765-773. doi: 10.1007/s10147-019-01573-0. Epub 2019 Nov 7. PMID: 31701290. Elias D, Debaere T, Roche A, Bonvallot S, Lasser P. Preoperative selective portal vein embolizations are an effective means of extending the indications of major hepatectomy in the normal and injured liver. HEPATO-GASTROENTEROL. 1998;45(19):170-7 Greget M, Pfleger N, Briggs P, Blonde E, Veillon F, Jaeck D. Interest of portal vein embolization combined with two-stage hepatectomy in treatment of colorectal liver metastases. 2015. Giraudo G, Greget M, Oussoultzoglou E. Preoperative contralateral portal vein embolization before major hepatic resection is a safe and efficient procedure: A large single institution experience. SURGERY. 2008;143(4):476-82. Sullivan KL. Hepatic artery chemoembolization *. SEMIN ONCOL. 2002;29(2):145-51. Pelletier G, Roche A, Ink O, Anciaux ML, Derhy S, Rougier P, et al. A randomized trial of hepatic arterial chemoembolization in patients with unresectable hepatocellular carcinoma. J HEPATOL. 1990;11(2):181. Sanjay GMD, Marcella MJMS, Ravi MMD, Kamran AMD, Michael JWMD, David CMMD, et al. Hepatic arterial embolization and chemoembolization for the treatment of patients with metastatic neuroendocrine tumors. CANCER-AM CANCER SOC. 2005;104(8):1590-602. Additional Declarations No competing interests reported. Cite Share Download PDF Status: Published Journal Publication published 15 Nov, 2025 Read the published version in Pediatric Surgery International → Version 1 posted Editorial decision: Revision requested 21 Sep, 2025 Reviews received at journal 15 Sep, 2025 Reviewers agreed at journal 06 Sep, 2025 Reviewers invited by journal 06 Sep, 2025 Editor assigned by journal 14 Aug, 2025 Submission checks completed at journal 13 Aug, 2025 First submitted to journal 12 Aug, 2025 You are reading this latest preprint version Research Square lets you share your work early, gain feedback from the community, and start making changes to your manuscript prior to peer review in a journal. As a division of Research Square Company, we’re committed to making research communication faster, fairer, and more useful. We do this by developing innovative software and high quality services for the global research community. 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Also discoverable on Platform About Our Team In Review Editorial Policies Advisory Board Help Center Resources Author Services Accessibility API Access RSS feed Manage Cookie Preferences © Research Square 2026 | ISSN 2693-5015 (online) Privacy Policy Terms of Service Do Not Sell My Personal Information {"props":{"pageProps":{"initialData":{"identity":"rs-7357968","acceptedTermsAndConditions":true,"allowDirectSubmit":false,"archivedVersions":[],"articleType":"Research Article","associatedPublications":[],"authors":[{"id":511225838,"identity":"1d251e31-080c-4772-bbc4-c1bd9d8bca28","order_by":0,"name":"Chen Zhen","email":"","orcid":"","institution":"Capital Institute of Pediatrics","correspondingAuthor":false,"prefix":"","firstName":"Chen","middleName":"","lastName":"Zhen","suffix":""},{"id":511225839,"identity":"fe7cc27d-021e-4b75-a928-27c821a7dfbf","order_by":1,"name":"Qiao Guoliang","email":"","orcid":"","institution":"Capital Institute of Pediatrics","correspondingAuthor":false,"prefix":"","firstName":"Qiao","middleName":"","lastName":"Guoliang","suffix":""},{"id":511225840,"identity":"04738ca9-b0b2-4541-84dd-7a1a5dd1955b","order_by":2,"name":"Ge Juntao","email":"","orcid":"","institution":"The First Affiliated Hospital of Shandong First Medical University \u0026 Shandong Provincial Qianfoshan Hospital","correspondingAuthor":false,"prefix":"","firstName":"Ge","middleName":"","lastName":"Juntao","suffix":""},{"id":511225841,"identity":"e1e733e2-c18a-47aa-962c-aa7f3ba39d25","order_by":3,"name":"Chen Xinghai","email":"","orcid":"","institution":"Capital Institute of Pediatrics","correspondingAuthor":false,"prefix":"","firstName":"Chen","middleName":"","lastName":"Xinghai","suffix":""},{"id":511225842,"identity":"ffb3d911-e879-4722-8351-e021eab8bd54","order_by":4,"name":"Li Long","email":"data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAAZAAAAAyAQMAAABI0h/eAAAABlBMVEX///8AAABVwtN+AAAACXBIWXMAAA7EAAAOxAGVKw4bAAAA5ElEQVRIie3QPQrCMBTA8RcC7RLbteKgR3hS8ANCz1IoZOrgDawUnKqueguPUBusiwdwcCs4OxbpoGZyMh0F819C4P3IB4DJ9IM5lCavhc83/epU35EHWmJR8iaC7BIRdrczEekJKFKQfRIPeuxeqO13YpNFNXvkFOEMPsecgi2Pe83FUn+3vlpjssqjGK8OMCEuGrLsdbIbm6ZOKGO8UfDYqA2RHpZsmE5QkqQVYbVEPDOfQkuS+h31wZYgGYrI0r3FdeWhYg0PXY+WUDc8cG1ZfiUqsvw8VzuuatqNmUwm05/2BELpRn2LeMwiAAAAAElFTkSuQmCC","orcid":"","institution":"Capital Institute of Pediatrics","correspondingAuthor":true,"prefix":"","firstName":"Li","middleName":"","lastName":"Long","suffix":""}],"badges":[],"createdAt":"2025-08-12 16:53:04","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-7357968/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-7357968/v1","draftVersion":[],"editorialEvents":[{"content":"https://doi.org/10.1007/s00383-025-06225-1","type":"published","date":"2025-11-15T15:58:48+00:00"}],"editorialNote":"","failedWorkflow":false,"files":[{"id":91193455,"identity":"548cbdde-0af1-4fb3-91ec-9d80c7684ddd","added_by":"auto","created_at":"2025-09-12 14:45:12","extension":"png","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":643791,"visible":true,"origin":"","legend":"\u003cp\u003ePreoperative CT showed that the huge tumor\u003c/p\u003e","description":"","filename":"1.png","url":"https://assets-eu.researchsquare.com/files/rs-7357968/v1/4ccb01991792807b7a5290d5.png"},{"id":91193460,"identity":"3932689b-d4ae-4421-b8b8-68020d5b13e1","added_by":"auto","created_at":"2025-09-12 14:45:12","extension":"png","order_by":2,"title":"Figure 2","display":"","copyAsset":false,"role":"figure","size":989549,"visible":true,"origin":"","legend":"\u003cp\u003eCT showed that the remnant liver increased and no tumor recurrence 6 months after operation\u003c/p\u003e","description":"","filename":"2.png","url":"https://assets-eu.researchsquare.com/files/rs-7357968/v1/3bfcb65a28bf3bfc14197a80.png"},{"id":96105149,"identity":"3ef0d733-59bc-4dfc-a99d-686e6f03d57a","added_by":"auto","created_at":"2025-11-17 16:09:21","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":3067713,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-7357968/v1/4e47a0b4-5968-4b9e-9419-59b3f172ba34.pdf"}],"financialInterests":"No competing interests reported.","formattedTitle":"Surgical resection of huge hepatic hepatoblastoma involving second, third hepatic hilum: a study of 59 cases","fulltext":[{"header":"Introduction","content":"\u003cp\u003eHepatoblastoma is the most common malignant liver tumor in children, accounting for 1.3% of malignant tumors in children(Clinical effectiveness of multimodality treatment on advanced pediatric hepatoblastom). Pediatric hepatoblastoma doesn\u0026rsquo;t have clear symptoms at early stage, and usually won\u0026rsquo;t be identified until it causes clear abdominal mass or compression of surrounding organs(1, 2). Quite a number of pediatric giant hepatoblastoma cases involve the second and third hepatic portals (GHSTP), which had been thought to be unresectable because uncontrollable massive hemorrhage is likely to occur during operation. With the development of pediatric liver surgery and the improvement of perioperative treatment levels, the giant hepatoblastoma involving the second and third hepatic portals is transiting from unresectable to resectable. However, there remains high operative risk and mortality rate. This operation is technically demanding, need to have a good understanding of intrahepatic anatomy. This study explored the operative feasibility and methods of this type of tumors expecting to expand the surgical indications and improve the efficacy of pediatric hepatoblastoma treatment.\u003c/p\u003e"},{"header":"METHODS","content":"\u003cp\u003e\u003cstrong\u003eClinical data\u0026nbsp;\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eFrom January 2006 to December 2024, a total of 59 pediatric patients diagnosed with hepatoblastoma and undergoing partial hepatectomy for giant hepatoblastoma with synchronous portal vein tumor thrombosis (GHSTP) were retrospectively enrolled in our hospital (Figure 1). The age of these patients ranged from 3 months to 15 years, with a mean age of 4.1 years. The diagnosis of hepatoblastoma was histopathologically confirmed in all cases. All 59 patients received preoperative chemotherapy for 1 to 6 courses, with treatment continued until no further reduction in tumor size was observed. The mean maximum diameter of the tumors was 15.2 cm, ranging from 8.5 cm to 22.6 cm (Table 1).\u003c/p\u003e\n\u003cp\u003eTable 1 Demographic data of 59 children\u003c/p\u003e\n\u003ctable border=\"1\" cellspacing=\"0\" cellpadding=\"0\" width=\"100%\"\u003e\n \u003ctbody\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 350px;\"\u003e\n \u003cp\u003eParameter\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 170px;\"\u003e\n \u003cp\u003eNumber\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 151px;\"\u003e\n \u003cp\u003eProportion(%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 350px;\"\u003e\n \u003cp\u003e\u003cstrong\u003eClinical characteristics\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 170px;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 151px;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 350px;\"\u003e\n \u003cp\u003e\u003cstrong\u003eGender\u003c/strong\u003e\u003c/p\u003e\n \u003cp\u003e\u0026nbsp;boy\u003c/p\u003e\n \u003cp\u003e\u0026nbsp;girl\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 170px;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003cp\u003e22\u003c/p\u003e\n \u003cp\u003e37\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 151px;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003cp\u003e37.2\u003c/p\u003e\n \u003cp\u003e62.7\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 350px;\"\u003e\n \u003cp\u003e\u003cstrong\u003eAge at operation\u003c/strong\u003e\u003c/p\u003e\n \u003cp\u003e\u0026lt;6month\u003c/p\u003e\n \u003cp\u003e6momth-1year\u003c/p\u003e\n \u003cp\u003e\u0026gt;1year\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 170px;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003cp\u003e10\u003c/p\u003e\n \u003cp\u003e25\u003c/p\u003e\n \u003cp\u003e24\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 151px;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003cp\u003e16.9\u003c/p\u003e\n \u003cp\u003e42.3\u003c/p\u003e\n \u003cp\u003e40.7\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 350px;\"\u003e\n \u003cp\u003e\u003cstrong\u003eCycles of postoperative chemotherapy \u0026nbsp; \u0026nbsp;\u003c/strong\u003e\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; 0\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp; \u0026nbsp; 1-3\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp; \u0026nbsp; \u0026gt;3\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 170px;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003cp\u003e0\u003c/p\u003e\n \u003cp\u003e36\u003c/p\u003e\n \u003cp\u003e23\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 151px;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003cp\u003e61.0\u003c/p\u003e\n \u003cp\u003e39.0\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 350px;\"\u003e\n \u003cp\u003e\u003cstrong\u003ePre-operative laboratory examination\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 170px;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 151px;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 350px;\"\u003e\n \u003cp\u003eRed blood cells(10\u003csup\u003e12\u003c/sup\u003e/L)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 170px;\"\u003e\n \u003cp\u003e3.58\u0026plusmn;0.61\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 151px;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 350px;\"\u003e\n \u003cp\u003ewhite blood cell(10\u003csup\u003e9\u003c/sup\u003e/L)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 170px;\"\u003e\n \u003cp\u003e5.04\u0026plusmn;2.93\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 151px;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 350px;\"\u003e\n \u003cp\u003ePlatelet\u0026nbsp;(10\u003csup\u003e9\u003c/sup\u003e/L)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 170px;\"\u003e\n \u003cp\u003e376.80\u0026plusmn;2131\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 151px;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 350px;\"\u003e\n \u003cp\u003e\u003cstrong\u003eAFP at diagnosis(ng/ml)\u0026nbsp;\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 170px;\"\u003e\n \u003cp\u003e715.96\u0026plusmn;429.95\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 151px;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 350px;\"\u003e\n \u003cp\u003e\u003cstrong\u003eHistology (main component)\u003c/strong\u003e\u003c/p\u003e\n \u003cp\u003e\u003cstrong\u003e\u0026nbsp;\u003c/strong\u003e Epithelial\u003c/p\u003e\n \u003cp\u003eMixed\u003c/p\u003e\n \u003cp\u003e\u0026nbsp; Others\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 170px;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003cp\u003e35\u003c/p\u003e\n \u003cp\u003e20\u003c/p\u003e\n \u003cp\u003e4\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 151px;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003cp\u003e59.3\u003c/p\u003e\n \u003cp\u003e33.8\u003c/p\u003e\n \u003cp\u003e6.9\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 350px;\"\u003e\n \u003cp\u003e\u003cstrong\u003eDiameter (cm)\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 170px;\"\u003e\n \u003cp\u003e15.2(8.5-22.6)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 151px;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 350px;\"\u003e\n \u003cp\u003e\u003cstrong\u003eVascular invasion\u003c/strong\u003e\u003c/p\u003e\n \u003cp\u003e\u003cstrong\u003e\u0026nbsp; No\u003c/strong\u003e\u003c/p\u003e\n \u003cp\u003e\u003cstrong\u003e\u0026nbsp; Yes\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 170px;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003cp\u003e37\u003c/p\u003e\n \u003cp\u003e22\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 151px;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003cp\u003e62.7\u003c/p\u003e\n \u003cp\u003e37.2\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 350px;\"\u003e\n \u003cp\u003e\u003cstrong\u003eMetastases\u003c/strong\u003e\u003c/p\u003e\n \u003cp\u003e\u003cstrong\u003e\u0026nbsp;No\u003c/strong\u003e\u003c/p\u003e\n \u003cp\u003e\u003cstrong\u003e\u0026nbsp;Yes\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 170px;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003cp\u003e52\u003c/p\u003e\n \u003cp\u003e7\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 151px;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003cp\u003e88.1\u003c/p\u003e\n \u003cp\u003e11.9\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003c/tbody\u003e\n\u003c/table\u003e\n\u003cp\u003e\u003cstrong\u003e\u0026nbsp;Surgical and perioperative treatment method\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eAll pediatric patients with GHSTP underwent routine B-mode ultrasound, contrast-enhanced computed tomography (CT), or magnetic resonance imaging (MRI) to evaluate tumor location, size, and relationship with major hepatic vasculature. A subcostal incision was performed to achieve adequate release of adhesions and ligaments surrounding the liver. Preoperative vascular control was established by placing occluders at the portal vein, as well as the suprahepatic and infrahepatic segments of the inferior vena cava, respectively. After dissecting and freeing the ligaments, the short hepatic veins were transected, and the liver was carefully separated from the inferior vena cava. Resection margins were preoperatively planned based on tumor extent. The superficial hepatic parenchyma was incised using electrocoagulation, followed by gentle fragmentation of liver tissue with right-angled forceps. Unresected residual vascular or biliary structures within the liver parenchyma were identified as part of the hepatic hilar system and meticulously ligated. During tumor resection, intermittent clamping of the hepatic portal triad was performed. If an inferior vena cava defect was identified after tumor removal, primary repair was conducted under vascular occlusion.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eFollow up methods and endpoints\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eAll patients were subjected to regular postoperative follow-up. During the first 2 years after surgery, serum alpha-fetoprotein (AFP) levels and B-mode ultrasonography were assessed monthly, and computed tomography (CT) was performed every 3 months. Thereafter, follow-up intervals were extended to 3\u0026ndash;6 months. The diagnostic criteria for intrahepatic recurrence were defined as the presence of newly identified lesions on ultrasonography or CT combined with an elevation in serum AFP levels. However, intrahepatic recurrence was suspected in cases where CT revealed atypical lesions, or when serum AFP levels were elevated without corresponding new lesions detected by ultrasonography or CT; in such instances, further evaluation with magnetic resonance imaging (MRI) or hepatic angiography was required. The primary endpoint of this study was tumor recurrence. Disease-free survival (DFS) was defined as the time interval from the date of surgery to the date of confirmed tumor recurrence or death (whichever occurred first). The follow-up duration ranged from 16 to 220 months, with a median follow-up time of 136.7 months.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eStatistics\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eDisease-free survival (DFS) was evaluated using the Kaplan\u0026ndash;Meier method, with analysis restricted to patients who survived the postoperative period (excluding those who died postoperatively). A two-tailed P value \u0026lt; 0.05 was considered statistically significant.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eEthics, consent and permissions\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThis study was designed as a retrospective analysis utilizing de-identified registry data. In accordance with the guidelines of the local institutional review board (IRB), this study was exempt from formal ethical approval due to its retrospective nature and the use of anonymized data. Written informed consent for the collection, storage, and research use of clinical data was obtained from all patients or their legal guardians during the initial hospitalization period.\u003c/p\u003e"},{"header":"Result","content":"\u003cp\u003e\u003cstrong\u003eSurgical results and postoperative complications\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eLiver tumor resection was successfully performed in all 59 pediatric patients, with all achieving clear (R0) resection margins. Intraoperative hemodynamic parameters remained stable in all patients, with no occurrences of shock, liver failure, or perioperative mortality observed. The operative duration ranged from 160 to 350 minutes, with a mean of 270 minutes. Intraoperative blood loss was 40\u0026ndash;500 mL, and the mean perioperative red blood cell (RBC) transfusion volume was 1.3 units (range, 1 to 4 units). RBC transfusion was required in 81.6% of patients (n=40). Intermittent hepatic portal triad clamping was performed in all pediatric patients, with a total clamping duration ranging from 15 to 35 minutes (mean, 22 minutes). The clamping protocol was as follows: 15 patients underwent a single clamping episode, 26 patients required two episodes, and 18 patients needed more than two episodes. Each individual clamping interval did not exceed 20 minutes. The resection approaches adopted were as follows: 13 cases underwent resection of segments V to VIII; 12 cases underwent resection of segments IV to VIII; 6 cases underwent resection of segments I to IV; 10 cases underwent resection of segments I, IV, and VIII; 8 cases underwent resection of segments I, IV, V, and VIII; 4 cases underwent resection of segment I plus segments V to VIII; and 6 cases underwent resection of segment I plus segments IV to VIII. All pediatric patients achieved an uneventful recovery after the operation. There was no mortality, and postoperative complications occurred in 8 cases, specifically: 2 cases of abdominal hemorrhage, 1 case of intra-abdominal abscess, 1 case of bile leakage, 2 cases of intestinal obstruction, and 2 cases of transient respiratory insufficiency. All patients exhibited liver function impairment, which was primarily characterized by elevated alanine transaminase (ALT) levels; decreased albumin levels were observed in some patients. Generally, liver function returned to normal within 2\u0026ndash;4 weeks postoperatively. The median length of hospital stay ranged from 8 to 16 days, with a mean of 12.5 days (Figures 1 and 2).\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003ePostoperative survival status\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eAll children were regularly followed up, and the final follow-up time was October 2024. In all children, the one-year non-neoplasm survival rate was 91.5%, 3-year non-neoplasm survival rate was 71.2%, and 5-year non-neoplasm survival rate was 32.5%. The longest non-neoplasm survival rate was 109 months (this patient is still living in a non-neoplasm healthy state). 59 patients received standard postoperative chemotherapy and did regular outpatient check-ups after chemotherapy, recurrence was found in 30 (51.0%) of them and chemotherapy was continued after finding of recurrence. In the six patients with pulmonary metastases, 3 patients showed absence of metastases after surgery and chemotherapy, and 3 patients died within one year after surgery.\u003c/p\u003e"},{"header":"Discussion","content":"\u003cp\u003eHepatoblastoma is the most common malignant liver tumor in children, accounting for 0.8%\u0026ndash;2% of all pediatric malignant tumors [3, 4]. Over the past two decades, with advancements in hepatic surgical techniques, improvements in anesthetic management, and the introduction of novel chemotherapeutic agents, the overall survival rate of patients with hepatoblastoma has significantly improved. The 3-year overall survival rate is approximately 70% [3, 4\u0026ndash;6]. However, hepatoblastoma frequently invades major blood vessels, primarily due to its presentation at an advanced stage upon diagnosis and its rapid growth rate. This poses challenges in defining appropriate surgical indications and renders the surgical procedures themselves highly demanding.\u0026nbsp;Furthermore, the occurrence of multiple postoperative complications and a high mortality rate have hindered the widespread implementation of surgical intervention. The aim of this study was to analyze and summarize 59 cases of giant hepatoblastoma in pediatric patients involving the second and third hepatic portals, with the purpose of discussing the key points and challenges in surgical techniques for this specific group of infant patients and improving the overall survival rate.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003ePreoperative liver assessment\u0026nbsp;\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003ePreoperative evaluation of each patient is essential, encompassing assessments of the tumor characteristics, surgical feasibility, liver function, residual liver volume, and the patient\u0026apos;s general condition, among other factors. Imaging modalities such as B-mode ultrasound, computed tomography (CT), and magnetic resonance imaging (MRI) facilitate a comprehensive understanding of the hepatic vascular anatomy, effectively enhance the precision of surgery, and are crucial for the selection and formulation of surgical plans as well as specific intraoperative management. Three-dimensional computed tomography (3D CT) imaging enables clear visualization of major blood vessels, including the portal venous system, inferior vena cava, and hepatic veins. Avoiding injury to these vessels is critical in surgeries involving the second and third hepatic portals. Of particular importance is the relationship between the left, middle, and right hepatic veins at the second hepatic portal and the tumor, as well as whether these vessels have been invaded by the tumor. Thus, preoperative imaging evaluation is of great significance.\u0026nbsp;All patients in this cohort underwent preoperative three-dimensional computed tomography (3D CT) reconstruction. The high-quality imaging enabled clearer visualization of the tumor boundaries. In particular, based on the hepatic vascular imaging, we could delineate the relationship between the tumors and the portal veins as well as the hepatic veins, and more accurately assess the feasibility of successful resection prior to surgery.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eManagement of hepatic hilum during operation\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eGiant hepatic tumors, particularly those located in the central liver segments (IV, V, VIII) and the caudate lobe (I), often involve the retro hepatic inferior vena cava, hepatic veins, and short hepatic veins. Avoiding injury to these vessels and effectively controlling bleeding in case of iatrogenic damage are critical to the success of the operation. First, adequate exposure of the second hepatic portal and the tumor is essential to enable resection under direct visualization. Second, during hepatic resection, it is preferable to manage the main hepatic veins and short hepatic veins outside the liver parenchyma, as this facilitates clear dissection and prevents iatrogenic injury and bleeding. For resection of giant hepatoblastoma in the right liver, the right hepatic lobe should be mobilized to the right wall of the inferior vena cava, followed by resection of several short hepatic veins and the retrohepatic inferior vena cava in a bottom-up sequence. The space between the root of the right hepatic vein and the inferior vena cava should be carefully dissected to allow placement of a clamp in the suprahepatic vena cava. \u0026nbsp; \u0026nbsp;\u003c/p\u003e\n\u003cp\u003eThe caudate lobe is located in a deep anatomical position, adjacent to the inferior vena cava, hepatic veins, and the first hepatic portal. In this cohort of giant hepatoblastoma cases, 34 involved the caudate lobe: 8 underwent complete caudate lobe resection combined with resection of other liver segments, and 26 underwent partial caudate lobe resection with resection of other segments. Intraoperatively, 5\u0026ndash;7 thick short hepatic veins are typically encountered, which drain into the orifices of the three main hepatic veins; care must be taken to avoid injury to the right and middle hepatic veins. Resection of the caudate lobe and segments VI or VII is performed under occlusion of the first hepatic portal. To ensure surgical success, hepatic portal occlusion is often required, and strict control of the duration and frequency of occlusion is essential for operative safety. The key technical points for surgeries involving the second and third hepatic portals can be summarized as follows:(1) Adequately mobilize the liver to expose the extent of the tumor and major blood vessels in the second and third hepatic portals;(2) Preset occluders at the first and second hepatic portals, and perform intermittent occlusion when necessary; (3) Protect the hepatic venous return vessels and ensure a sufficient residual liver volume. Precautions are as follows: (1) Adequately ligate the vascular branches to prevent wound bleeding and oozing after tumor resection; (2) The duration of portal occlusion should not be excessively long, generally within 20 minutes; (3) Carefully inspect the inflow and outflow vessels of the residual liver postoperatively to ensure adequate hepatic perfusion and venous return.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eTwo-stage operation\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eIf preoperative evaluation indicates that the tumor has invaded multiple major vessels, or that the tumor volume exceeds 80% of the total liver volume with insufficient residual liver function postoperatively, a two-stage surgical approach may be considered for such pediatric patients. International researchers have reported that portal vein branch embolization can be performed when preoperative assessment reveals insufficient residual liver volume, followed by hepatic tumor resection once the contralateral normal liver parenchyma has hypertrophied [7\u0026ndash;9]. Liver tumor resection following phase I transcatheter arterial chemoembolization (TACE) is also regarded as an effective therapeutic strategy for the management of large unresectable hepatic tumors [10-12]. We recently admitted a pediatric patient with a giant right hepatic lobe hepatoblastoma. This patient had undergone 5 courses of standard chemotherapy at an external hospital; however, despite the treatment, the tumor showed minimal shrinkage. Given the massive size of the tumor, computational analysis indicated that the estimated residual liver volume after resection would be less than 20%, which would be insufficient to sustain the patient\u0026rsquo;s hepatic functional requirements. Thus, in the first-stage procedure, both the hepatic artery and right portal vein branch were ligated. A follow-up CT scan at 2 weeks postoperatively revealed compensatory hypertrophy of the patient\u0026rsquo;s left hepatic lobe, with no significant tumor progression. Subsequent resection of the giant right hepatic tumor was performed successfully; pathological examination confirmed the diagnosis of fetal-type hepatoblastoma, and the patient achieved an uneventful recovery postoperatively.\u003c/p\u003e\n\u003cp\u003eIn summary, resection of giant hepatoblastoma involving the second and third hepatic portals is technically challenging. However, with detailed and accurate preoperative evaluation and a thorough understanding of hepatic anatomy, such tumor resection is feasible and can be performed safely. Complete resection of this type of tumor may effectively improve the long-term survival rate of patients with hepatoblastoma.\u003c/p\u003e"},{"header":"Declarations","content":"\u003ch2\u003eCompliance with Ethical Standards:\u003c/h2\u003e\n\u003ch2\u003eFunding source:\u003c/h2\u003e\n\u003cp\u003eThere was no funding source for this study. The corresponding author had full access to all the data in the study and had final responsibility for the decision to submit for publication.\u003c/p\u003e\n\u003ch2\u003eConflict of interest statement:\u003c/h2\u003e\n\u003cp\u003eNone declared\u003c/p\u003e\n\u003ch2\u003eEthical approval:\u003c/h2\u003e\n\u003cp\u003eAll procedures performed in studies were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.\u003c/p\u003e\n\u003ch2\u003eInformed consent:\u003c/h2\u003e\n\u003cp\u003eAll patients signed an infprmed consent approved by the institutional Review Board.\u003c/p\u003e\n\u003ch2\u003eAuthor Contribution\u003c/h2\u003e\n\u003cp\u003eChen Zhen and Qiao Guoliang designed the study, collected and analyzed data, and drafted the manuscript.Ge Juntao contributed to data interpretation, and revised the manuscript critically for important intellectual content.Chen Xinghai supervised the study and approved the final version of the manuscript.Li Long participated in study design, conducted literature review, and edited the manuscript.All authors reviewed the manuscript.\u003c/p\u003e\n\u003ch2\u003eAcknowledgments:\u003c/h2\u003e\n\u003cp\u003eWe thank those patients who supported our study and authors who provided us with the full-text.\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\n\u003cli\u003eCai M, Liu Y, Wang Y, et al. Three-dimensional reconstruction combined with virtual surgery for pediatric giant hepatoblastoma involving hepatic hilum: a single-center experience. J Pediatr Surg. 2023;58(6):1118-1124. DOI: 10.1016/j.jpedsurg.2023.01.023\u003c/li\u003e\n\u003cli\u003eVasireddi AK, Leo ME, Squires JH. Magnetic resonance imaging of pediatric liver tumors. Pediatr Radiol. 2022 Feb;52(2):177-188. doi: 10.1007/s00247-021-05058-z. Epub 2021 Apr 14. PMID: 33852026.\u003c/li\u003e\n\u003cli\u003eSrinivasan S, Prasad M, Parambil BC, Shrimal A, Gollamudi VRM, Subramani V, Ramadwar M, Khanna N, Baheti AD, Gala K, Patil V, Laskar S, Qureshi S, Chinnaswamy G. Treatment outcomes and prognostic factors in children with hepatoblastoma using a risk-stratified approach. Pediatr Blood Cancer. 2023 Jul;70(7):e30302. doi: 10.1002/pbc.30302. Epub 2023 Apr 12. PMID: 37046413.\u003c/li\u003e\n\u003cli\u003eMeyers RL, Maibach R, Hiyama E, H\u0026auml;berle B, Krailo M, Rangaswami A, Aronson DC, Malogolowkin MH, Perilongo G, von Schweinitz D, Ansari M, Lopez-Terrada D, Tanaka Y, Alaggio R, Leuschner I, Hishiki T, Schmid I, Watanabe K, Yoshimura K, Feng Y, Rinaldi E, Saraceno D, Derosa M, Czauderna P. Risk-stratified staging in paediatric hepatoblastoma: a unified analysis from the Children\u0026apos;s Hepatic tumors International Collaboration. Lancet Oncol. 2017 Jan;18(1):122-131. doi: 10.1016/S1470-2045(16)30598-8. Epub 2016 Nov 22. PMID: 27884679; PMCID: PMC5650231.\u003c/li\u003e\n\u003cli\u003eKremer N, Walther AE, Tiao GM. Management of hepatoblastoma: an update. CURR OPIN PEDIATR. 2014 2014-06-01;26(3):362-9.\u003c/li\u003e\n\u003cli\u003eRen X, Li H, Diao M, Xu H, Li L. Impact of microscopically margin-positive resection on survival in children with hepatoblastoma after hepatectomy: a retrospective cohort study. Int J Clin Oncol. 2020 Apr;25(4):765-773. doi: 10.1007/s10147-019-01573-0. Epub 2019 Nov 7. PMID: 31701290.\u003c/li\u003e\n\u003cli\u003eElias D, Debaere T, Roche A, Bonvallot S, Lasser P. Preoperative selective portal vein embolizations are an effective means of extending the indications of major hepatectomy in the normal and injured liver. HEPATO-GASTROENTEROL. 1998;45(19):170-7\u003c/li\u003e\n\u003cli\u003eGreget M, Pfleger N, Briggs P, Blonde E, Veillon F, Jaeck D. Interest of portal vein embolization combined with two-stage hepatectomy in treatment of colorectal liver metastases. 2015.\u003c/li\u003e\n\u003cli\u003eGiraudo G, Greget M, Oussoultzoglou E. Preoperative contralateral portal vein embolization before major hepatic resection is a safe and efficient procedure: A large single institution experience. SURGERY. 2008;143(4):476-82.\u003c/li\u003e\n\u003cli\u003eSullivan KL. Hepatic artery chemoembolization *. SEMIN ONCOL. 2002;29(2):145-51.\u003c/li\u003e\n\u003cli\u003ePelletier G, Roche A, Ink O, Anciaux ML, Derhy S, Rougier P, et al. A randomized trial of hepatic arterial chemoembolization in patients with unresectable hepatocellular carcinoma. J HEPATOL. 1990;11(2):181.\u003c/li\u003e\n\u003cli\u003eSanjay GMD, Marcella MJMS, Ravi MMD, Kamran AMD, Michael JWMD, David CMMD, et al. Hepatic arterial embolization and chemoembolization for the treatment of patients with metastatic neuroendocrine tumors. CANCER-AM CANCER SOC. 2005;104(8):1590-602.\u003c/li\u003e\n\u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":false,"hideJournal":false,"highlight":"","institution":"","isAcceptedByJournal":true,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"pediatric-surgery-international","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"pesi","sideBox":"Learn more about [Pediatric Surgery International](http://link.springer.com/journal/383)","snPcode":"383","submissionUrl":"https://submission.nature.com/new-submission/383/3","title":"Pediatric Surgery International","twitterHandle":"","acdcEnabled":true,"dfaEnabled":true,"editorialSystem":"em","reportingPortfolio":"Springer Hybrid","inReviewEnabled":true,"inReviewRevisionsEnabled":false},"keywords":"Surgical resection, huge hepatic hepatoblastoma, hepatic hilum","lastPublishedDoi":"10.21203/rs.3.rs-7357968/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-7357968/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003cp\u003e\u003cb\u003eBackground\u003c/b\u003e\u003c/p\u003e\u003cp\u003eA considerable proportion of pediatric giant hepatoblastoma cases involve the second and third hepatic portals (GHSTP), which were previously considered unresectable. Surgical management of such GHSTP-involved cases is associated with significant technical challenges. This retrospective study was designed to assess the feasibility and surgical approaches for pediatric giant hepatoblastoma with involvement of the second and third hepatic portals.\u003c/p\u003e\u003cp\u003e\u003cb\u003eMethods\u003c/b\u003e\u003c/p\u003e\u003cp\u003eFrom January 2006 to December 2024, 59 pediatric patients with giant hepatoblastoma involving the second and third hepatic portals (GHSTP) underwent partial hepatectomy. The therapeutic outcomes of these cases were retrospectively analyzed.\u003c/p\u003e\u003cp\u003e\u003cb\u003eResults\u003c/b\u003e\u003c/p\u003e\u003cp\u003eAll 59 patients underwent successful liver tumor resection. The operative duration ranged from 160 to 350 minutes, with a mean of 270 minutes. First hepatic portal clamping was performed in all cases. The resection approaches were as follows: 13 cases underwent resection of segments V-VIII; 12 cases, resection of segments IV-VIII; 6 cases, resection of segments I-IV; 10 cases, resection of segments I, IV, and VIII; 8 cases, resection of segments I, IV, V, and VIII; 4 cases, resection of segment I plus segments V-VIII; and 6 cases, resection of segment I plus segments IV-VIII. There was no mortality, and postoperative complications occurred in 8 cases.\u003c/p\u003e\u003cp\u003e\u003cb\u003eConclusion\u003c/b\u003e\u003c/p\u003e\u003cp\u003eResection of giant hepatoblastoma involving the second and third hepatic portals remains technically challenging; however, with meticulous and accurate preoperative assessment coupled with a thorough understanding of hepatic anatomy, such tumor resection is feasible and can be performed with acceptable safety. Moreover, complete resection of this subset of tumors may effectively improve the long-term survival outcomes of patients with hepatoblastoma.\u003c/p\u003e","manuscriptTitle":"Surgical resection of huge hepatic hepatoblastoma involving second, third hepatic hilum: a study of 59 cases","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2025-09-12 14:45:07","doi":"10.21203/rs.3.rs-7357968/v1","editorialEvents":[{"type":"communityComments","content":0},{"type":"decision","content":"Revision requested","date":"2025-09-21T19:47:19+00:00","index":"","fulltext":""},{"type":"editorInvitedReview","content":"","date":"2025-09-15T15:25:55+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"226658068707336676735108061940287318471","date":"2025-09-06T16:23:02+00:00","index":"hide","fulltext":""},{"type":"reviewersInvited","content":"","date":"2025-09-06T16:13:10+00:00","index":"","fulltext":""},{"type":"editorAssigned","content":"","date":"2025-08-14T19:53:31+00:00","index":"","fulltext":""},{"type":"checksComplete","content":"","date":"2025-08-13T07:41:26+00:00","index":"","fulltext":""},{"type":"submitted","content":"Pediatric Surgery International","date":"2025-08-12T16:37:02+00:00","index":"","fulltext":""}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"pediatric-surgery-international","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"pesi","sideBox":"Learn more about [Pediatric Surgery International](http://link.springer.com/journal/383)","snPcode":"383","submissionUrl":"https://submission.nature.com/new-submission/383/3","title":"Pediatric Surgery International","twitterHandle":"","acdcEnabled":true,"dfaEnabled":true,"editorialSystem":"em","reportingPortfolio":"Springer Hybrid","inReviewEnabled":true,"inReviewRevisionsEnabled":false}}],"origin":"","ownerIdentity":"c6df7028-de60-415c-ae5a-201487f47363","owner":[],"postedDate":"September 12th, 2025","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"published-in-journal","subjectAreas":[],"tags":[],"updatedAt":"2025-11-17T16:04:39+00:00","versionOfRecord":{"articleIdentity":"rs-7357968","link":"https://doi.org/10.1007/s00383-025-06225-1","journal":{"identity":"pediatric-surgery-international","isVorOnly":false,"title":"Pediatric Surgery International"},"publishedOn":"2025-11-15 15:58:48","publishedOnDateReadable":"November 15th, 2025"},"versionCreatedAt":"2025-09-12 14:45:07","video":"","vorDoi":"10.1007/s00383-025-06225-1","vorDoiUrl":"https://doi.org/10.1007/s00383-025-06225-1","workflowStages":[]},"version":"v1","identity":"rs-7357968","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-7357968","identity":"rs-7357968","version":["v1"]},"buildId":"8U1c8b4HqxoKbykW_rLl7","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}

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