Title: The role of the minimally invasive surgery in the management of paediatric liver tumours

Source: eCancer 2025, 2017, Nov 13. [E–publication]  

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Date of publication: November 2025

Publication type: Article

Abstract: Minimally invasive surgical techniques are increasingly adopted for the management of hepatic masses in children. Laparoscopic liver biopsy can be used to obtain tissue diagnosis while avoiding the risks of open surgery and providing improved cosmesis. Laparoscopic or robotic liver resection has more gradually been adopted in children than in adults but can be utilized for appropriately located tumours as long as oncologic principles are maintained. Patient size is a factor when choosing whether to perform liver resection via a minimally invasive approach. Laparoscopic radiofrequency ablation offers an alternative strategy to surgery for paediatric patients with small masses or can serve as a bridge to transplant.

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Title: Benign and malignant pediatric liver masses: radiologic-pathologic update from the pediatric LI-RADS working group

Source: Radiographics 2025, 45 (11): e240246

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Date of publication: November 2025 

Publication type: Review article

Abstract: Primary pediatric liver tumors are uncommon but not rare, and they have clinical effects that range from self-limited to life-threatening. Although malignant liver tumors are more common than benign lesions in children, they comprise only 1%-2% of all pediatric malignancies. The combination of low incidence and variable outcomes can add uncertainty to interpretation. The authors review the most common pediatric liver lesions, both benign and malignant. Some lesions such as hemangioma, mesenchymal hamartoma, hepatoblastoma, and undifferentiated embryonal sarcoma of the liver are exclusively pediatric diseases. Others such as focal nodular hyperplasia, hepatocellular adenoma, hepatocellular carcinoma, and fibrolamellar carcinoma are well known in adult radiologic practices, but children may present and be treated differently. The patient’s age at tumor development can help to narrow the differential diagnosis. An understanding of the underlying abnormality and its correlation to imaging findings also improves diagnostic confidence. MRI with hepatobiliary contrast agents may help to diagnose focal nodular hyperplasia by demonstrating contrast material retention in its functioning hepatocytes, but certain adenomas, well-differentiated malignancies, and even hemangiomas may show some degree of contrast material retention as well. Contrast-enhanced US can help to distinguish liver tumors based on temporal patterns of enhancement, but continued work shows overlap and exceptions that should be kept in mind. This review is aimed to update and review the current understanding of the most common pediatric liver tumors.

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Title: Characteristics of transfusion and association with oncologic outcomes in hepatoblastoma resection

Source: Pediatric Blood Cancer 2025, Sep 3. [E–publication]

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Date of publication: September 2025

Publication type: Multicentre retrospective observational study 

Abstract: Introduction: Children with hepatoblastoma (HB) and other solid tumors frequently undergo intraoperative blood transfusion (IBT) with unknown impact on oncologic outcomes and scant data to guide transfusion in this population. This study tested the hypothesis that IBT is associated with poorer survival in children with HB.

Methods: A multicenter retrospective observational study of patients aged <18 years with HB who underwent primary tumor resection, including liver transplantation, from 2010 to 2019 was performed at 19 institutions. The association of IBT with risk of recurrence and death were determined using propensity score reweighted (PSR) Cox proportional hazards regression analyses.

Results: There were 338 patients identified who met inclusion criteria and had sufficient data for inclusion. Of those, 257 (76%) received IBT, including 253 (98%) who received packed red blood cells (pRBC), 84 (33%) who received plasma, and 28 (11%) who received platelets. IBT was associated with higher pretreatment extent of disease (p < 0.001), presence of annotation factors (+VPEFR: 50%, n = 129 vs. 37%, n = 30, p = 0.035), and complex resections (extended, meso-, or total hepatectomy: 54%, n = 139 vs. 27%, n = 22, p < 0.001); these differences were mitigated after applying propensity score weighting. Patients who received IBT had greater postoperative hemoglobin (g/dL) (median 10 (I8-11) vs. 9 (8-10), p = 0.013) and required more postoperative plasma and platelet transfusions (p < 0.05). Median follow-up was 4.4 (2.0-8.3) years. Compared with non-IBT patients, those with IBT had higher incidence of death (PSR HR 2.35, 95% CI 1.10-5.02). Recurrence did not significantly differ across groups (PSR HR = 0.82, 95% CI 0.45-1.48).

Conclusion: IBT was associated with greater hazard of death. Postoperative hemoglobin levels suggested that unnecessary transfusions occurred and a pRBC-focused approach to IBT led to coagulopathy. Development of optimal transfusion strategies for HB are needed to minimize unnecessary transfusions.

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Title: Does chemotherapy have an effect on the treatment success of children and adolescents with unresectable hepatocellular carcinoma? Findings from the German Liver Tumour Registry

Source: Cancers (Basel) 2025, 17 (15): 2444

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Date of publication: July 2025

Publication type: Article 

Abstract: Background: Paediatric hepatocellular carcinoma (HCC), including its fibrolamellar variant (FLC), is a rare malignancy with distinct biological behaviour and limited therapeutic options. While complete surgical resection is a key determinant of survival, many patients present with unresectable tumours at diagnosis. The role of neoadjuvant chemotherapy in improving resectability, particularly in histologically distinct subtypes, remains inconclusive.

Methods: We retrospectively analysed 43 patients (<18 years) with histologically confirmed conventional HCC (cHCC, n = 27) or FLC (n = 16) enrolled in the German Pediatric Liver Tumour Registry. We assessed clinical characteristics, treatment response, surgical outcomes, and survival. Special focus was placed on the impact of neoadjuvant chemotherapy in initially unresectable tumours.

Results: FLC and cHCC exhibited significant differences in clinical presentation, such as age of presentation, AFP elevation, or presence of underlying liver disease. Although overall survival did not significantly differ between groups, cHCC tumours showed a markedly higher response to chemotherapy (62.5% partial remission vs. 0% in FLC). Complete resection (R0) was achieved in 77% of all patients and was the strongest predictor of survival. Importantly, a subset of cHCC patients who initially had unresectable tumours became eligible for curative surgery following neoadjuvant chemotherapy. Notably, delayed resection after chemotherapy led to outcomes comparable to those with upfront surgery, whereas progression during chemotherapy was associated with a universally poor prognosis.

Conclusions: This study supports upfront resection as the preferred strategy in paediatric HCC and FLC whenever feasible. In cHCC, neoadjuvant chemotherapy demonstrated a favourable response profile and contributed to secondary resectability in a subset of initially unresectable cases, supporting a potential role within a multimodal treatment approach. In contrast, FLC showed limited responsiveness to current systemic therapies. These findings emphasise the importance of histology-specific strategies and highlight the ongoing need for more effective systemic options, particularly for unresectable FLC.

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Title: Surgical strategy for pediatric liver tumors involving the hepatic venous confluence and the inferior vena cava

Source: Annals of Surgical Oncology 2025, Mar 26. [E–publication]

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Date of publication: March 2025

Publication type: Article  

Abstract: Background: Pediatric liver tumors presenting as centrally located masses with contact to or even invasion of all three hepatic veins (HVs) and the inferior vena cava (IVC) present significant surgical challenges. While liver transplantation may be indicated in truly unresectable tumors, extended liver resection with vascular reconstruction can be an organ-preserving alternative.

Objective: This study aimed to present a reference center’s strategy for children with liver tumors involving the hepatic venous confluence or the retrohepatic IVC who underwent extended liver resection with vascular reconstruction.

Methods: All pediatric patients undergoing major hepatectomy with reconstruction of an HV or the IVC over a 10-year study period were included. Preoperative imaging, surgical techniques, and short- and long-term postoperative data were analyzed.

Results: From a total of 125 pediatric major hepatectomies, 17 children (15 hepatoblastoma, two undifferentiated embryonal sarcoma) underwent liver resection with vascular reconstruction of an HV or the IVC. In nine cases an HV was reconstructed, and in eight children, a partial resection of the IVC was performed. Total vascular exclusion of the liver was applied in 16/17 cases. No 90-day postoperative mortality, no major postoperative complication, and no local relapse occurred; 16/17 patients are alive without relapse at a median follow-up of 44 months (range 19-111).

Conclusion: This is the largest single-center series to report major hepatectomies with HV or IVC reconstruction in children. In specialized centers, these complex procedures are associated with excellent outcomes. Successful tumor resection can be achieved in selected cases even in locally advanced tumor stages.

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Title: Pediatric hepatocellular carcinoma: a review of predisposing conditions, molecular mechanisms, and clinical considerations  

Source: International Journal of Molecular Sciences 2025, 26 (3): 1252  

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Date of publication: January 2025  

Publication type: Review article  

Abstract: Pediatric hepatocellular carcinoma (HCC) is a rare malignant liver tumor affecting children and adolescents and occurring either sporadically or in the context of underlying liver disease. In this review, we detail the epidemiology of pediatric HCC with a focus on predisposing factors including hepatic or systemic disease, genetic disorders, and familial cancer syndromes. We summarize existing research on the pathophysiology of pediatric HCC, including molecular mechanisms of oncogenesis, highlighting unique disease features differentiating pediatric HCC from adult HCC. We then survey the landscape of therapeutic options for pediatric HCC, including novel therapeutics. Lastly, we discuss the pathologic spectrum upon which pediatric HCC is postulated to exist, ranging from hepatoblastoma to HCC and including the hybrid entity hepatocellular neoplasm not otherwise specifed (HCN-NOS). In summary, we highlight the key clinical and molecular features of pediatric HCC that may inform future research and novel approaches to the clinical care of these patients.

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Title: Impact of hepatoblastoma on infectious complications following pediatric liver transplantation

Source: Pediatric Transplantation 2025, 29 (1): e70035

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Date of publication: February 2025 

Publication type: Retrospective review  

Abstract: Background: Liver transplantation is the standard therapy for end-stage liver disease in pediatric patients with biliary atresia (BA), congenital and metabolic conditions, and for an unresectable malignant tumor like hepatoblastoma (HB). BA is the leading indication for pediatric liver transplantation, while HB is the most common childhood liver cancer. Despite improved outcomes through advanced surgical techniques and novel immunosuppression, pediatric liver transplantation (pLT) is complicated by post-transplant infections.

Methods: A retrospective review was performed of pLT recipients at Cincinnati Children’s Hospital Medical Center (CCHMC) and stratified patients by underlying disease to assess impact on post-transplant infectious events.

Results: BA patients were youngest at pLT (12.5 months; p < 0.001) compared to other disease cohorts (HB 30.8, other 43.7). All HB patients received organs from deceased donors. In the year following pLT, 93% of the patients experienced at least one infectious event (IE). HB patients had the highest mean number of IE across disease groups (5.5 IE/patient vs. BA 4.5, other 4.0; p = 0.055), with significantly more patients with fever and neutropenia (p < 0.001) and EBV infections (p = 0.012). HB patients were more likely to develop IE earlier after pLT than non-HB groups (p = 0.013), especially Clostridioides difficile (p < 0.01) and fever and neutropenia (p < 0.01). Despite having variable IE experiences, 1-and-5-year survival across disease groups were similar.

Conclusions: IE were frequently observed in HB patients after pLT, possibly related to pre-and-postoperative chemotherapy and associated neutropenia. Underlying disease may help inform targeted infection-related patient management following pLT.

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Title: Increased MASH-associated liver cancer in younger demographics

Source: Hepatology Communications 2025, 9 (1): e0629

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Date of publication: January 2025

Publication type: Article

Abstract: Background: The incidence of cancer and the prevalence of metabolic disease and metabolic dysfunction-associated steatotic liver disease is increasing in young adults. However, updated global data on metabolic dysfunction-associated steatohepatitis (MASH)-associated primary liver cancer (PLC) in young adults remains scarce.

Methods: This study analyzed data from the Global Burden of Disease study between 2000 and 2021 to assess the age-standardized incidence, mortality, and disability-adjusted life years rates from MASH-associated PLC in young adults (15-49 y).

Results: In 2021, there were 4300 incidence cases, 3550 deaths, and 179,340 disability-adjusted life years from MASH-associated PLC in young adults. Among various etiologies of PLC in young adults, only MASH-associated PLC had increased incidence rates (annual percent change: +0.26, 95% CI: 0.16%-0.35%), with the Eastern Mediterranean region having the largest observed increase (annual percent change: 1.46%, 95% CI: 1.40%-1.51%). In 2021, MASH-associated PLC in young adults made up 6% (+1% from 2000) incident cases, 6% (+2% from 2000) deaths, and 6% (+2% from 2000) disability-adjusted life years of all PLC in this age group. Over half of the countries exhibited an increase in age-standardized incidence rate from MASH-associated PLC in young adults from 2000 to 2021.

Conclusions: The incidence of MASH-associated PLC in young adults is significantly increasing, signaling likely future increases in PLC incidence among older adults as this cohort ages. This trend necessitates urgent strategies worldwide to mitigate the epidemics of MASH-associated PLC in young adults.

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Title: Outcomes of pediatric hepatocellular carcinoma: a single-center experience with resection versus transplantation

Source: Pediatric Transplantation 2024, 28 (8): e14882

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Date of publication: December 2024

Publication type: Article

Abstract: Background: Pediatric hepatocellular carcinoma (HCC) presents significant challenges due to its aggressive nature, with survival depending on complete resection. We aimed to assess outcomes between liver resection (LR) and liver transplantation (LT).

Methods: A total of 25 patients were retrieved, four of whom were classified as palliative at diagnosis. A subanalysis comparing cirrhotic liver (n = 14) versus de novo (n = 11) HCC was performed to identify confounding variables. Further evaluation focused on the 21 children with histologically confirmed HCC who underwent LR (n = 7) versus LT (n = 14). Kaplan-Meier survival curves were constructed.

Results: The mean age was 7.8 ± 6.1 years for patients with cirrhotic liver and 12.1 ± 3.5 years for de novo HCC. Our group observed overall total survival rates of 100%, 85%, and 77% at 12, 36, and 60 months, respectively. De novo tumors had a higher recurrence rate and a poorer prognosis (p = 0.039 and p = 0.045). The disease-free survival at 60 months in our cohort was significantly lower among the LR group compared to the LT group (14% vs. 82%; p = 0.0081). Recurrence after initial management (n = 8) showed location differences between LR and LT. Preoperative alpha-fetoprotein (AFP) was elevated in 71% of children, but did not correlate with recurrence or compromised survival. Elevated AFP 3 months post-operation affected the course negatively (p = 0.044). Tumor number and diameter exhibited a trend towards poorer outcomes.

Conclusions: These findings emphasize the need for comprehensive pediatric surgical guidelines for HCC. We recommend LT over LR in pediatric cases. Extrahepatic disease post-neoadjuvant chemotherapy remains the only absolute contraindication.

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Title: Too big to fail: volumetric analyses and incidence of posthepatectomy liver failure in 125 major hepatectomies in children

Source: Annals of Surgery 2024, Nov 25. [E–publication]

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Date of publication: November 2024

Publication type: Article

Abstract: Objective: To assess the incidence of posthepatectomy liver failure (PHLF) and the role of the future liver remnant (FLR) in children undergoing major hepatectomy.

Summary background data: Incidence and risk factors of PHLF in children are unclear, with no validated definition for this age group. Consequently, the role of the FLR in pediatric hepatectomy and evidence-based preoperative guidelines remain undefined.

Methods: All pediatric patients undergoing major hepatectomy at a tertiary care center over a 10-year study period were analyzed. Preoperative imaging was used for volumetry. The incidence of PHLF was assessed applying predefined definitions, and the prognostic impact of the FLR on PHLF and complications was evaluated.

Results: 125 children underwent major hepatectomy, including 35 trisectionectomies. There was a strong correlation between imaging-based measured total liver volume and calculated standard liver volume (r=0.728, P<0.001). The median total liver volume-to-body weight (BW) ratio was 3.4%, the median FLR/BW ratio was 1.5%. The median FLR-to-total liver volume ratio was 44% (range 18%-97%). No clinically relevant PHLF occurred. FLR/total liver volume and FLR/BW ratios had low predictive value for postoperative liver dysfunction and morbidity.

Conclusion: This is the largest reported single-center series of pediatric major hepatectomies. PHLF is exceedingly rare in children. The liver volume-to-body weight ratio is higher in children compared to adults, and the FLR is sufficient even in extreme resections with less than 20% of liver remnant. These findings strongly question the use of ALPPS, portal vein embolization, or transplantation based on suspected insufficient liver remnant in children.

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