Liver Tumors In Children Research Articles

Comparison of ultrasonographic features between two types of hepatic echinococcosis and hepatoblastoma in children

IntroductionThe prevalence of hepatic alveolar echinococcosis (AE) and cystic echinococcosis (CE) is notably high among children in western China, with the mortality rate for untreated children potentially reaching up to 90%. Meanwhile, hepatoblastoma is the most common malignant liver tumor in children, yet its treatment differs significantly from that of hepatic echinococcosis. This study aimed to compare and analyze the ultrasonographic and contrast-enhanced ultrasound (CEUS) characteristics of hepatic CE, AE, and hepatoblastoma in children, providing more imaging evidence for accurate clinical diagnosis.MethodsA retrospective analysis was conducted on preoperative data from children with pathologically confirmed hepatic echinococcosis in our hospital between 2012 and 2024. Furthermore, children consecutively diagnosed with hepatoblastoma between 2020 and 2024, confirmed by histopathological examination, were selected as the control group. Clinical data, conventional ultrasound images, and CEUS characteristics of hepatic echinococcosis and hepatoblastoma were analyzed and compared.ResultsThe mean ages of 22 children with hepatic CE and nine children with hepatic AE were 11.6 ± 2.8 years and 11.8 ± 3.6 years, respectively. The mean age of 36 children with hepatoblastoma was 2.9 ± 3.0 years. Significant differences were observed in age and history of residence or travel to endemic areas among children with hepatic echinococcosis and hepatoblastoma (P < 0.001). Hepatic CE and hepatoblastoma showed a higher proportion of cystic degeneration [≥50% (54.5%, 12/22), and between 0% and <50% (47.2%, 17/36, respectively)], while hepatic AE predominantly showed no cystic degeneration (88.9%, 8/9). Clear boundaries were most commonly seen in hepatic CE lesions (95.5%, 21/22), while unclear boundaries were more frequent in hepatic AE lesions (88.9%, 8/9) (P < 0.05). Calcification was more prevalent in hepatic AE compared to hepatic CE and hepatoblastoma (P < 0.05). Hepatoblastoma exhibited richer color Doppler signals (94.4%, 34/36) compared to hepatic CE and AE (P < 0.05). CEUS was performed on two hepatic echinococcosis and nine hepatoblastoma lesions. On CEUS, one hepatic AE lesion showed peripheral hyperenhancement in the arterial phase, while one hepatic CE lesion showed no significant enhancement. In hepatoblastoma, nine lesions demonstrated hyperenhancement in the arterial phase and hypoenhancement in the late phase.ConclusionThis study demonstrates the value of ultrasound in differentiating hepatic echinococcosis from hepatoblastoma in children. Hepatic CE typically manifests as a well-defined cystic or cystic-solid mass, while hepatic AE often presents as an ill-defined cystic-solid or solid mass with diffuse calcifications. Conversely, hepatoblastoma appears as a partially well-defined cystic-solid or solid mass with abundant color Doppler signals within and around the lesion.

Asynchronous Transitions from Hepatoblastoma to Carcinoma in High-Risk Pediatric Tumors.

Most malignant hepatocellular tumors in children are classified as either hepatoblastoma (HB) or hepatocellular carcinoma (HCC), but some tumors demonstrate features of both HB and HCC1-3. These tumors have been recognized under a provisional diagnostic category by the World Health Organization and are distinguished from HB and HCC by a combination of histological, immunohistochemical, and molecular features4-6. Their outcomes and cellular composition remain an open question7-9. The heterogeneous histological and molecular profiles of hepatoblastomas with carcinoma features (HBCs)4 may result from cells with combined HB and HCC characteristics (HBC cells) or from mixtures of cells displaying either HB or HCC signatures. We used multiomics profiling to show that HBCs are mixtures of HB, HBC, and HCC cell types. HBC cells are more chemoresistant than HB cells, and their chemoresistance-a driver of poor outcomes10-12-is determined by their cell types, genetic alterations, and embryonic differentiation stages. We showed that the prognosis of HBCs is significantly worse than that of HBs. We also showed that HBC cells are derived from HB cells at early hepatoblast differentiation stages, that aberrant activation of WNT-signaling initiates HBC transformation, and that WNT inhibition promotes differentiation and increases sensitivity to chemotherapy. Furthermore, our analysis revealed that each HBC is the product of multiple HB-to-HBC and HBC-to-HCC transitions. Thus, multiomics profiling of HBCs provided key insights into their biology and resolved major questions regarding the etiology of these childhood liver tumors.

Worldwide burden of liver cancer across childhood and adolescence, 2000–2021: a systematic analysis of the Global Burden of Disease Study 2021

Liver cancer is a significant contributor to the global disease burden, of which hepatoblastomas are the most common liver tumors in children, with 90% of cases occurring within the first 5 years of life. It is important for pediatricians and subspecialists in pediatric gastroenterology and hepatology to have knowledge of the epidemiology and incidence trends of pediatric hepatic cancer, despite its rarity. In the present study, we first provide estimates of the incidence and mortality burden of hepatoblastoma and liver cancer from 2000 to 2021 in the childhood and adolescence. Liver cancer burden and its attributable risk factors were estimated using data from the Global Burden of Disease Study (GBD) 2021. Percentage change was estimated to show the trend of liver cancer estimates from 2000 to 2021. The age-standardized rate (ASR) and estimated annual percentage change (EAPC) were utilized for measuring hepatoblastomas incidence and deaths rate trends. In accordance with the GBD framework, 95% uncertainty intervals (UIs) for all estimates by averaging the data from 1000 draws, with the lower and upper bounds of the 95% UIs. Globally, from 2000 to 2021 in the age 5-19 years group, the incidence cases and deaths cases due to liver cancer decreased from 2449.2 (95% UI: 2235.9-2689.8) to 1692.9 (95% UI: 1482.0-1992.5) and 2248.5 (95% UI: 2053.7-2474.9) to 1516.6 (95% UI: 1322.1-1797.9), respectively. Meanwhile, from 2000 to 2021 in the age 20-24 years group, the incidence cases and deaths cases due to liver cancer decreased from 1453.5 (95% UI: 1327.8-1609.4) to 1285.1 (95% UI: 1159.2-1447.2) and 1432.3 (95% UI: 1307.6-1585.7) to 1195.5 (95% UI: 1066.1-1355.2), respectively. In addition, the prevalence of liver cancer decreased from 41.9% (95% UI: 18.7%-64.7%) to 26.4% (95% UI: 14.2%-39.1%) in the age 5-19 years group, and 46.6% (95% UI: 42.8%-51.5%) to 36.5% (95% UI: 33.1%-40.9%) in the age 20-24 years. From 2000 to 2021, in the age group of 5-19 years, the proportion of liver cancer incidence due to hepatitis B has decreased from 42.2% to 37.9%, while the proportion due to hepatitis C has increased from 1.1% to 1.6%. Additionally, there has been an increase in the proportion of NASH-induced liver cancer incidence from 5.2% to 9.4%, and alcohol use induced liver cancer incidence has also increased from 0.5% to 0.7% over the same period. Globally, from 2000 to 2021, the incidence cases and deaths cases due to hepatoblastoma decreased from 6131.8 (95%UI: 5234.8-6961.9) to 4045.6 (95% UI: 3250-4995.8) and 4059.2 (95% UI: 3494.5-4621.2) to 2416 (95% UI: 1940.2-3022.5), respectively. There was some variation in age-related sex-specific patterns, the highest number of hepatoblastoma incidence cases occurred in children between 2 and 4 years old and females in the age range of 12 months to 9 years had a higher number of new cases. Importantly, the incidence of hepatoblastoma was started to increase sharply after the age of 1 month. The results of the present study are significant for liver health policy and practice in childhood and adolescence. Differentiated intervention and outreach strategies based on age and gender would be necessary to reduce the impact of liver cancer. Early screening and interventions for hepatoblastoma is important especially in the population of under 9 years old. This study was supported by the National Key R&D Program of China (grant numbers 2023YFC2307000), National Natural Science Foundation of China [grant numbers 82170571 and 81974068], China Postdoctoral Science Foundation (grant numbers 2023M741283).

Accuracy of contrast-enhanced CT in liver neoplasms in children under 2 years age

BackgroundMultiple differentials exist for pediatric liver tumors under 2 years. Accurate imaging diagnosis may obviate the need for tissue sampling in most cases.ObjectiveTo evaluate the imaging features and diagnostic accuracy of computed tomography (CT) in liver tumors in children under 2 years.MethodsEighty-eight children under 2 years with treatment naive liver neoplasms and baseline contrast-enhanced CT were included in this institutional review board approved retrospective study. Two blinded onco-radiologists assessed these tumors in consensus. Findings assessed included enhancement pattern, lobulated appearance, cystic change, calcifications, central scar-like appearance, and metastases. The radiologists classified the lesion as hepatoblastoma, infantile hemangioma, mesenchymal hamartoma, rhabdoid tumor, or indeterminate, first based purely on imaging and then after alpha-fetoprotein (AFP) correlation. Multivariate analysis and methods of comparing means and frequencies were used for statistical analysis wherever applicable. Diagnostic accuracy, sensitivity, and positive predictive values were analyzed.ResultsThe mean age of the sample was 11.4 months (95% CI, 10.9–11.8) with 50/88 (57%) boys. The study included 72 hepatoblastomas, 6 hemangiomas, 4 mesenchymal hamartomas, and 6 rhabdoid tumors. Presence of calcifications, multilobular pattern of arterial enhancement, lobulated morphology, and central scar-like appearance was significantly associated with hepatoblastomas (P-value < 0.05). Fourteen out of eighty-eight lesions were called indeterminate based on imaging alone; six lesions remained indeterminate after AFP correlation. Pure radiology-based diagnostic accuracy was 81.8% (95% CI, 72.2–89.2%), which increased to 92.1% (95% CI, 84.3–96.7%) (P-value > 0.05) after AFP correlation, with one hepatoblastoma misdiagnosed as a rhabdoid tumor. If indeterminate lesions were excluded for biopsy, the accuracy would be 98.8% (95% CI, 93.4–99.9%).ConclusionCT had high accuracy for diagnosing liver neoplasms in the under 2-year age population after AFP correlation. Certain imaging features were significantly associated with the diagnosis of hepatoblastoma. A policy of biopsying only indeterminate lesions after CT and AFP correlation would avoid sampling in the majority of patients.Graphical

FOXM1 and CHD4 expression is associated with chemoresistance in hepatoblastoma

Hepatoblastoma (HB) is the most common malignant liver tumor in childhood. Although pre-operative cisplatin (CDDP)-based chemotherapy is often used in cases of HB, about 20％ of HB patients exhibit resistance to CDDP. Forkhead box protein M1 (FOXM1) and chromo-domain-helicase-DNA-binding protein 4 (CHD4) have been associated with CDDP resistance in various tumors. We here analyzed the immunohistochemical expression of FOXM1 and CHD4 in HB specimens of 33 patients (mean age: 20 months) post-chemotherapy. The differentiation of specimens was assessed using the digital pathology software QuPath®, and then the relation between the FOXM1 or CHD4 expression and the differentiation and various other clinicopathological parameters was investigated. The histological type was epithelial in 19 cases (57.6%) and mixed epithelial and mesenchymal in 14 cases (42.4%). Nine cases had only a fetal component, 1 case had only an embryonal component, 22 cases had both fetal and embryonal components, and 1 case had no viable tumor. Both the FOXM1 and CHD4 immunoexpressions were found significantly more frequently in the embryonal than fetal components (p<0.0001 and p<0.0001, respectively). Regarding chemotherapy efficacy, the alpha-fetoprotein (AFP) level after chemotherapy was correlated with both the imaging shrinkage rate (R=-0.52) and histological residual rate (the percentage of the viable tumors of HB after chemotherapy)(R=0.62). High FOXM1 score was correlated with a high-postoperative AFP value (p<0.01) and a low AFP attenuation rate (p<0.05), but the FOXM1 score was not correlated with the imaging shrinkage rate (p=0.4418) or histological residual rate (p=0.4418). High CHD4 score showed a nonsignificant trend toward correlation with high postoperative AFP value (p=0.0849) and was not significantly correlated with the other parameters. Collectively, our results showed that FOXM1 expression may be useful in evaluating the response to CDDP-based chemotherapeutic regimens. Accurate measurement of FOXM1 expression by our scoring system using QuPath® is important in cases with mixed HB components of various differentiation levels.

Interventional Radiology Management of Pediatric Liver Tumors

AbstractPediatric liver tumors are not only rare, they are also different from the adult liver tumors. Hepatoblastomas and hepatocellular carcinomas (HCCs) are the most common primary malignant tumors seen in children. Fibrolamellar HCC, sarcomas, cholangiocarcinoma, germ cell tumor, and rhabdoid tumor and metastatic tumors comprise the remainder of liver malignancies. Hepatic infantile hemangiomas, mesenchymal hamartoma, regenerative nodules, adenomas, and focal nodular hyperplasia are the common benign liver tumors of childhood. The Pediatric Hepatic International Tumour Trial (PHITT) is an international trial with the goal of evaluating new and existing treatments for pediatric liver tumors. Chemotherapy, surgery, and liver transplantation are well-established in the management of pediatric liver tumors. In the background of increasing incidence of pediatric tumors, and availability of newer, less invasive modalities to treat tumors, it is vital to understand the role of interventional radiology in the management of pediatric liver tumors.

Risk for Recurrence in Long-Term Follow-Up of Children after Liver Transplantation for Hepatoblastoma or Hepatocellular Carcinoma.

The aim of this study was to assess the long-term results of liver transplantation (LT) in pediatric patients with unresectable hepatoblastoma (HB) or hepatocellular carcinoma (HCC) with special reference to the risk of tumor recurrence. We retrospectively analyzed data from 46 HB and 26 HCC patients who underwent LT between 1990 and 2022. In HCC patients, we compared outcomes depending on donor type. We evaluated the impact of a number of risk factors on recurrence-free survival after LT. Estimated patient survival after 5, 10, and 15 years was 82%, 73%, and 73% in the HB group and 79%, 75%, and 75% in the HCC group, respectively (p = 0.76). In the HCC group, living donor LT (LDLT) and deceased donor LT (DDLT) provided similar patient survival (p = 0.09). Estimated recurrence-free survival in patients who had three or fewer risk factors was significantly better than in patients with more than three risk factors (p = 0.0001). Adequate patient selection is necessary when considering LT for primary liver tumors in children. The presence of more than three risk factors is associated with a very high risk of recurrence and indicates poor prognosis, whereas extrahepatic disease may be considered a contraindication for transplantation.

Primary non-hepatoblastoma liver tumors in children- defining the clinical profile and outcomes of a single center cohort

Primary non-hepatoblastoma liver tumors in children- defining the clinical profile and outcomes of a single center cohort

Anaesthetic Challenges in Paediatric Hepatoblastoma Resection: A Case Report

The most common primary liver tumour in children is hepatoblastoma, which is often diagnosed in the first three years of life. While the majority of hepatoblastomas are sporadic, some associations are reported with genetic disorders like BeckwithWiedemann syndrome and familial adenomatous polyposis. The annual incidence of hepatoblastoma in children has gradually increased during the past three decades. Babies born prematurely, weighing &lt;1 kg, have been found to be significantly more likely to acquire hepatoblastoma. The most common symptoms with which children present are abdominal discomfort, easy fatigability, loss of appetite, and anaemia. The choice of treatment is surgical resection of the mass or surgical resection coupled with chemotherapy. Present case is of a seven-month-old, 7 kg infant who presented with a gradually increasing abdominal mass diagnosed as hepatoblastoma. Left hepatectomy was done under general anaesthesia and epidural analgesia. Liver resection is one of the major surgical procedures that carries a risk of blood loss and pulmonary embolism, but improved expertise in paediatric anaesthesia and surgical skills have reduced operative risk. The challenges in this case were securing vascular access, maintaining blood volume with considerable fluid and blood loss, and assuring haemodynamic stability. Maintenance of normothermia, glucose levels, and effective pain management were all crucial, necessitating careful planning and a multidisciplinary approach to maximise results and guarantee patient safety

Update on the Pathology of Pediatric Liver Tumors: A Pictorial Review

Liver tumors in children are uncommon and show remarkable morphologic heterogeneity. Pediatric tumors may arise from either the epithelial or mesenchymal component of the liver and rarely may also show both lines of differentiation. Both benign and malignant liver tumors have been reported in children. The most common pediatric liver tumors by age are benign hepatic infantile hemangiomas in neonates and infants, malignant hepatoblastoma in infants and toddlers, and malignant hepatocellular carcinoma in teenagers. Here, we provide an up-to-date review of pediatric liver tumors. We discuss the clinical presentation, imaging findings, pathology, and relevant molecular features that can help in the correct identification of these tumors, which is important in managing these children.

Feeding artery characteristics and enhancement patterns of hepatoblastoma patients treated with transarterial chemoembolization (TACE): Digital subtraction angiography evaluation.

Hepatoblastoma is one of the most common primary malignant liver tumors in children. The incidence of hepatoblastoma has been increasing, reaching 1.2 per million children now. The transarterial chemoembolization (TACE) procedure is one of the most practical and effective treatment options besides surgery. Digital subtraction angiography (DSA) is performed as the first step of the TACE procedure. The aim of this study was to provide information about the feeding arteries and enhancement pattern of the hepatoblastoma that was assessed by DSA before the TACE procedure. A retrospective study was conducted among hepatoblastoma cases that had undergone DSA on the TACE procedure to obtain information on the vascularity of the tumor. A total of 26 hepatoblastoma cases who had DSA examination as a part of their first TACE procedure were included, consisting of 15 boys and 11 girls, aged between 1-15 years. All cases were stage III and IV according to the Pre-Treatment Extent of Tumor (PRETEXT) staging classification. All hepatoblastoma cases had multiple feeding arteries, most of which were branches of the right hepatic artery. The largest diameter of the feeding artery was 1.82 mm, and the smallest was 0.63 mm. Most cases (84.62%) had strong contrast absorption, and spread evenly, either at the edges or in the center of the tumor. DSA is believed to be an accurate procedure to provide a detailed description of the feeding artery; enhancement patterns of hepatoblastoma were influenced by an adequate TACE.

Ferroptosis regulator NOS2 is closely associated with the prognosis and cell malignant behaviors of hepatoblastoma: a bioinformatic and in vitro study.

Hepatoblastoma (HB) is the most common liver tumor in children with easy metastasis. The emergence of ferroptosis as a novel form of cell death has gained increased attention in various human cancers. However, the roles of ferroptosis-related (FR) genes in HB remain elusive. The GSE133039, GSE131329, and GSE81928 datasets were utilized for screening core FR genes in HB. Through Lasso regression analysis and using the support vector machine recursive feature elimination (SVM-RFE) algorithm, three candidate FR genes were obtained for characterizing HB. Their expression patterns and their clinical associations were explored through the 'Limma' R package, and their diagnostic potential was evaluated using ROC curves. Nitric oxide synthase 2 (NOS2) emerged as a candidate for further analyses. The CIBERSORT algorithm and GSEA dataset were used to respectively investigate the immune and metabolism effects of NOS2; the former was validated through immunofluorescence. The GSDC database was employed to analyze the correlation between NOS2 expression and the therapeutic efficacy of multiple drugs. PCR, Western blotting, colony formation assays, and Transwell experiments, were used to determine biological functions of NOS2 in HB cells. Potential upstream transcription factors of NOS2 were predicted through the TRRUST, hTFtarget, GeneCards, and JASPAR databases. NQO1, SLC1A4, and NOS2 were identified as potential genes in HB and found to be significantly upregulated in tumor samples. Nevertheless, only NOS2 was closely associated with HB clinicopathological characteristics; high NOS2 expression indicated poor prognosis, metastatic tendency, and late clinical stage. Immune analyses indicated that high NOS2 expression was concomitant with decreased infiltration levels of CD8+ T cells but increased infiltration levels of macrophages. GSEA revealed that NOS2 failed to affect the enrichments of glycolysis, fatty acid metabolism, and cholesterol biosynthesis in HB. Moreover, NOS2 was positively correlated with the IC50 values of trametinib, lapatinib, and cisplatin. NOS2 overexpression promoted the proliferation, migration and invasion of HepG2 and HuH-6 cells. JUND was identified as a potential transcriptional regulator of NOS2 by binding to its promoter (5'-TTCTGACTCTTTT-3'). NOS2 plays a significant role in HB clinical assessments and holds promise as a novel therapeutic target.

Treatment Outcomes for Hepatoblastoma: 15-Years-Experience of a Single Institution – Sheikh Khalifa Medical City, Abu Dhabi, UAE

Background: Hepatoblastoma is the most common malignant liver tumor in children. Treatment protocols varies. In our center, we have adopted the Children’s Oncology Group (COG) AHEP0731 protocol, in addition to some experimental regimens for relapsed solid tumors. Methods: We aimed to investigate the outcome of the current hepatoblastoma treatment protocol at our center. 15 patients were included between January 2008 and June 2023. A retrospective review was carried to review the clinical presentation, serum α-fetoprotein (AFP) level at diagnosis, histological subtype, treatment, and outcomes. Results: 12 patients (80%) were symptomatic at time of diagnosis, with abdominal mass being the most common presenting complaint. Nine patients (60%) presented in stage 3 PRETEXT staging system. Epithelial histopathological subtype was predominant subtype. Thirteen patients have received preoperative chemotherapy, followed by surgical resection; only one patient underwent upfront surgical resection followed by chemotherapy. Preoperative chemotherapy consists of 2 to 4 cycles of Cisplatin, Fluorouracil, Vincristine and Doxorubicin (C5VD), followed by surgical resection. Four of them underwent neoadjuvent experimental chemotherapy utilizing agents such as Pazopanib, Pembrolizumab and Sorafenib. During followup, six patients died of progressive disease. The median survival time was 42 months (95% confidence interval: 18–42%). Five-year overall survival was 44.09% (95% confidence interval: 18–42%). Conclusions: The combination of surgery and chemotherapy for hepatoblastoma is an effective approach. Utilization of new-targeted therapies and relapsed solid tumors regimens may prolong life in patients who did not respond to standard therapy. Further studies are required to validate its usage on patients with advanced hepatoblastoma.

Differential diagnosis of benign and malignant liver tumors in children using the method of quantitative assessment of multiparametric magnetic resonance imaging data

Differential diagnosis of benign and malignant liver tumors in children using the method of quantitative assessment of multiparametric magnetic resonance imaging data

Reevaluation of sodium thiosulfate otoprotection using the consensus International Society of Paediatric Oncology Ototoxicity Scale: A report from the Children's Oncology Group study ACCL0431.

In two randomized trials (Children's Oncology Group ACCL0431 and International Childhood Liver Tumour Strategy Group SIOPEL-6), sodium thiosulfate (STS) demonstrated efficacy in preventing cisplatin-induced hearing loss (CIHL). However, the measures used in those trials have been superseded by the consensus International Society of Paediatric Oncology (SIOP) Ototoxicity Scale. To provide benchmark data for STS efficacy when using this contemporary scale, we reanalyzed ACCL0431 hearing outcomes with the SIOP scale and using multiple timepoints. Compared to the control arm, STS significantly reduced CIHL when assessed by the SIOP scale across these different approaches. These results provide critical data to inform treatment discussions and support future potential trial designs comparing otoprotectants.

Biliary complications post liver resection for pediatric liver tumors

ObjectiveTo review biliary complications following liver resection for liver tumors in children and their associated risk factors.MethodsRetrospectively, we reviewed children who underwent liver resection for liver tumors from 2010 to...

Mesenchymal Hamartoma of the Liver Associated with Discontinuity of the Vena Cava

Primary liver tumors in children are infrequent, accounting for 0.5-2 % of all pediatric neoplasms. About two-thirds are malignant. The most common pediatric malignant liver tumours are, in order of frequency, hepatoblastoma, hepatocellular carcinoma, and embryonic sarcoma of the liver. Among benign tumors, the most frequent are hemangioendothelioma and mesenchymal hamartoma of the liver, and more rarely focal nodular hyperplasia and hepatocellular adenoma [1,2]. We report the case of an infant who was entrusted to us for the management of a large liver tumor. Radiology (CT scan and MRI), in addition to the elements in favor of a mesenchymal hamartoma of the right liver revealed a congenital anomaly associated, in this case, with the absence of the inferior vena cava in its intraabdominal portion. A right hepatectomy enlarged to segment IV was performed. The right renal vein drained into the right suprahepatic vein was preserved. Note that this tumor represents 50% of the total weight of the patient, which explains the difficulties and constraints that hindered the development and quality of life of the young patient.

Epidemiological Study of Malignant Paediatric Liver Tumours in Denmark 1985–2020

Background: Malignant liver tumours in children are rare and national outcomes for this tumour entity are rarely published. This study mapped paediatric liver tumours in Denmark over 35 years and reported on the incidence, outcomes and long-term adverse events. Methods: We identified all liver tumours from the Danish Childhood Cancer Registry and reviewed the case records for patient and tumour characteristics, treatment and clinical outcome. Results: We included 79 patients in the analyses. Overall crude incidence was ~2.29 per 1 million children (&lt;15 yr) per year, with 61 hepatoblastomas (HB), 9 hepatocellular carcinomas and 9 other hepatic tumours. Overall 5-year survival was 84%, 78% and 44%, respectively. Nine patients had underlying liver disease or predisposition syndrome. Seventeen children underwent liver transplantation, with two late complications, biliary stenosis and liver fibrosis. For HB, age ≥ 8 years and diagnosis prior to 2000 were significant predictors of a poorer outcome. Adverse events included reduced renal function in 10%, reduced cardiac function in 6% and impaired hearing function in 60% (19% needed hearing aids). Behavioural conditions requiring additional support in school were registered in 10 children. Conclusions: In Denmark, incidences of malignant liver tumours during the last four decades have been increasing, as reported in the literature. HB survival has improved since the year 2000 and is comparable with international results. Reduced hearing is the major treatment-related side effect and affects approximately 60% of patients.

Quality of Life Outcomes for Patients Who Underwent Conventional Resection and Liver Transplantation for Locally Advanced Hepatoblastoma.

Hepatoblastoma is the most common malignant liver tumor of childhood, with liver transplant and extended resection used as surgical treatments for locally advanced tumors. Although each approach has well-described post-operative complications, quality-of-life outcomes have not been described following the two interventions. Long-term pediatric survivors of hepatoblastoma who underwent conventional liver resection or liver transplantation at a single institution from January 2000-December 2013 were recruited to complete quality-of-life surveys. Survey responses for the Pediatric Quality of Life Generic Core 4.0 (PedsQL, n = 30 patient and n = 31 parent surveys) and Pediatric Quality of Life Cancer Module 3.0 (PedsQL-Cancer, n = 29 patient and n = 31 parent surveys) were collected from patients and parents. The mean total patient-reported PedsQL score was 73.7, and the parent-reported score was 73.9. There were no significant differences in scores on the PedsQL between patients who underwent resection compared to those who underwent transplantation (p > 0.05 for all comparisons). On the PedsQL-Cancer module, procedural anxiety scores were significantly lower for patients who underwent resection as compared to transplant (M = 33.47 points less, CI [-60.41, -6.53], p-value 0.017). This cross-sectional study demonstrates that quality of life outcomes are overall similar among patients receiving transplants and resections. Patients who received a resection reported worse procedural anxiety.

Clinical features and a prognostic nomogram based on the SEER database for hepatoblastoma, hepatocellular carcinoma, and embryonal sarcoma among children and adolescents.

Hepatoblastoma (HB), hepatocellular carcinoma (HCC), and embryonal sarcoma (ES) are the three main types of liver tumors in children and adolescents. At present, epidemiological knowledge and predictors of these three liver tumor types in multi-ethnic populations are limited. This study aimed to outline the clinical features and construct a prognostic nomogram for these tumors, which can contribute to the prediction of dynamic overall survival probability during the follow-up period. A total of 1,122 patients liver tumor patients between 2000 to 2019 in Surveillance, Epidemiology, and End Results (SEER) database were enrolled for the current study, and separated into 824 HB, 219 HCC, and 79 ES according to the type of pathology. Independent prognostic factors were screened by univariate and multivariate Cox regression analysis, and a prognostic nomogram was constructed for overall survival. The accuracy and discriminative abilities of the nomogram were evaluated by concordance index as well as time-dependent receiver operating characteristic curves and calibration curves. Race (P=0.0016), surgery [hazard ratio (HR): 0.1021, P<0.001], and chemotherapy (HR: 0.27, P=0.00018) are independent prognostic factors for hepatoblastoma. Pathological tissue grading (P=0.00043), tumor node metastasis (TNM) staging (P=0.00061), and surgery are independent prognostic factors for hepatocellular carcinoma. Household income and surgery (HR: 0.1906, P<0.001) are independent prognostic factors for embryonal sarcoma. All of these prognostic factors are significantly associated with prognosis. A nomogram consisting of these variables was established, which showed a good concordance index (0.747, 0.775, and 0.828 in hepatoblastoma, hepatocellular carcinoma, and embryonal sarcoma, respectively). Also, the 5-year area under curve (AUC) of the nomogram were 0.738, 0.812, and 0.839 in hepatoblastoma, hepatocellular carcinoma, and embryonal sarcoma, respectively. In the calibration diagram, an optimal agreement between the nomogram-predicted and actual observed survival was evident. We developed an effective prognostic nomogram for overall survival prediction in hepatoblastoma, hepatocellular carcinoma, and embryonal sarcoma in children and adolescent patients, which will further benefit the assessment of long-term outcomes.