Abstract Hepatoblastoma (HB) is a rare liver tumour, and its congenital counterpart (CHB) is even less frequent. CHB has a clinically challenging management and a generally perceived worse outcome. This study aims to review the literature on CHB to better define presentation, diagnosis, available treatments and management options. The analysis of outcomes suggests that a significant portion of mortality is unrelated to the malignant nature of the tumour. Key factors influencing overall outcomes were identified: mortality linked to the ‘mass effect’ during both the prenatal (22%) and perinatal (32%) stages, as well as ‘oncological’ mortality encompassing tumour and/or treatment‐related factors (46%). Overall, after birth, CHB does not seem to confer a worse oncological prognosis per se, and should be managed similarly to older children, if patients are stable enough to undergo proper staging and treatment. A deeper knowledge and better outcomes would come from a large, homogeneous, collection of data possibly allowing a global protocol, focusing on a comprehensive management of CHB. 

Congenital hepatoblastoma: Expanding knowledge, improving outcomes

Liver transplantation (LT) has been recommended for hepatoblastoma (HB) involving all four liver sectors at diagnosis (PRETEXT IV). However, recent studies question the universal indication for primary LT in PRETEXT IV HB. We reviewed 32 PRETEXT IV HB patients treated at our center from February 2007 to July 2024. A multidisciplinary team reassessed surgical resectability to evaluate treatment strategies for PRETEXT IV HB. Of 32 patients, 28 (87.5%) underwent LT, while 4 (12.5%) underwent liver resection (LR). Median follow-up was 4.6 years (IQR 2.3-8.5). The tumor, which was deemed radiologically resectable before surgery, was found pathologically unresectable in two LT patients: viable tumor microembolism at sites where the tumor was present at diagnosis in one patient and severe tumor ossification close to the major vascular system in one patient. Four patients with tumor thrombus, unresectable radiologically, were pathologically resectable using LR with extracorporeal circulation. Among the four patients undergoing LR, two patients eventually received LT. One had a recurrence at the initial tumor site, and the other developed cirrhosis post-LR. Surgical complications occurred in three LT patients and one LR patient. No complications related to long-term immunosuppression have been observed in LT patients. However, one LT patient developed portal hypertension due to chronic rejection and is awaiting a deceased donor LT. One LT patient died due to recurrence. Despite uncertainties about long-term LT outcomes, LT remains the preferred strategy for safely achieving complete resection in PRETEXT IV HB patients. 

Surgical Strategies for Hepatoblastoma Involving Four Liver Sectors, Look Back to Look Forward: Lessons From a Single‐Center Experience

Abstract Two percent of pediatric malignancies arise primarily in the liver; roughly 60% of these cancers are hepatoblastoma (HB). Despite the rarity of these cases, international collaborative efforts have led to the consistent histological classification and staging systems, which facilitate ongoing clinical trials. Other primary liver malignancies seen in children include hepatocellular carcinoma (HCC) with or without underlying liver disease, fibrolamellar carcinoma (FLC), undifferentiated embryonal sarcoma of the liver (UESL), and hepatocellular neoplasm not otherwise specified (HCN‐NOS). This review describes principles of surgical management of malignant pediatric primary liver tumors, within the context of comprehensive multidisciplinary care. 

Surgical management of liver tumors

Abstract Background There is ongoing debate regarding liver transplantation (LT) versus liver resection (LR) for locally advanced hepatoblastoma. However, comparative studies are lacking. Consequently, a significant evidence gap persists, hindering the establishment of consensus guidelines. This study aimed to compare LT and LR for locally advanced hepatoblastoma, using predefined inclusion criteria to ensure comparable intervention groups. Methods According to current Children's Oncology Group (COG) and SIOPEL (European Childhood Liver Tumour Study Group) recommendations, hepatoblastoma that requires LT evaluation was defined as either PRETEXT (PRE‐Treatment EXTent of tumor) IV F+, POST‐TEXT (POST‐Treatment EXTent of tumor) IV, POST‐TEXT P+, and/or POST‐TEXT V+. A systematic literature search (Medline/Web‐of‐Science/Embase) was performed. Only patients who met the aforementioned criteria were included. Patient data were extracted individually and pooled. Results A total of 189 patients with locally advanced hepatoblastoma from 55 studies met the specified criteria, with 111 undergoing LT and 78 LR. There were no significant differences between the two groups in age, alpha‐fetoprotein (AFP), and PRETEXT stages. Local recurrence was more common after LR (14% vs. 3% in LT, p = .008), while distant recurrence was more often observed after LT (16% vs. 5% in LR, p = .035). Overall survival (OS) and event‐free survival (EFS) did not differ significantly between LT and LR (5‐year OS: LT = 75.3% [95% confidence interval: 66.5–85.2], LR = 87.6% [80.4–95.6], p = .140; 5‐year EFS: LT = 68.5% [59.3–79.1], LR = 71.1% [60.7–83.3], p = .700). Conclusion Real‐life data revealed that a considerable number of patients with locally advanced hepatoblastoma underwent LR. This analysis suggests that outcomes are similar and favorable for both approaches. LR can therefore be considered an effective alternative to LT in selected cases even in locally advanced hepatoblastoma. 

Reappraisal of liver resection as an alternative to transplantation in locally advanced hepatoblastoma: A systematic review and analysis of pooled individual patient data

Case of Advanced Hepatocellular Carcinoma Treated With Transarterial Radioembolization and Subsequent Liver Transplantation in a 22-Month-old.

Surgical Management and Outcomes of Patients with Multifocal Hepatoblastoma.

Popular Tasks used by Researchers

How can I help with your research?

Get more enhanced response by uploading the PDFs you want me to reference.

No relevant PDFs in your library

SciSpace is the AI research assistant for academics. Run systematic literature reviews on 280M+ papers, and write papers with cited sources. Trusted by 1M+ students, PhDs & researchers.

SciSpace | AI for Research

We encountered an error while loading the panel. Please try refreshing the page.

Something went wrong

Analyze PDFs

Code & Manuscripts

Funding & Grants

Literature & Patents

Medical & Clinical Data

Systematic Review

Visualize & Present

Web & Data

Build a Google Scholar-like website for your research.

Build a website

Create charts and images for your research

Create a Chart

Write a paper for submission to a journal

Draft a manuscript

Patent Search

Design eye-catching scientific posters in minutes.

Scientific Poster Generation

Systematic Literature Review

Anyone with the link can view this chat. You can switch it back to private anytime.

One task is running at the moment. Your messages will be shown right after.

Drag and drop or click here to browse

Loved by <highlight>1 million+</highlight> researchers

Extract a list of specific topics and their sources from unstructured text

Topics

Compare and analyze relevant papers that matches with your search

Papers

Get insights from PDFs and bookmarked papers from your library

My library

Recent searches

Try searching for:

Catch AI-generated content in scholarly and non-scholarly content

{ai} Detector

Ai Writer

Get PDF Summaries, highlighted text explanations 

Chat with PDF

Effortlessly create in-text citations and bibliographies in APA and 2,500 other formats

Citation generator

Get explanations, summaries, and answers on academic papers

Ease up your research workflow with {scispace}'s cohort of exciting AI tools

Elevate your academic writing skills and convey your ideas the way you want

Paraphraser

Explore our range of reading and writing tools

Your file is being prepared and should be ready in a few minutes. If it's a large file, it might take a bit longer. You can close this window, and we'll email you the file when it's done.

You have reached a maximum limit of <strong>{limit}</strong> columns in the table. Remove at least <strong>1</strong> column to add or create another one.

Kalyani A Patel

Author Tools

Chat about Author

Papers

Case of Advanced Hepatocellular Carcinoma Treated With Transarterial Radioembolization and Subsequent Liver Transplantation in a 22-Month-old.

Surgical Management and Outcomes of Patients with Multifocal Hepatoblastoma.