Childhood Liver Tumours

Childhood liver tumours are a heterogeneous group of neoplasms encompassing individually rare histological varieties. This chapter discusses the two most frequent subtypes—hepatoblastoma and hepatocellular carcinoma. In relation to hepatoblastoma, it covers the biology, epidemiology, clinical presentation, diagnosis, and discusses the different treatment strategies including risk-adapted treatment and orthotopic liver transplantation. The chapter reviews the renewed histological classification of paediatric primary liver tumours, the revised PRETEXT (pre-treatment extent) system to describe tumour extension, and the novel risk-stratification system for hepatoblastoma. It also reviews the treatment results and the prognosis of patients with hepatoblastoma. Finally, the chapter discusses the even rarer tumour, hepatocellular carcinoma.

How Do Synchronous Lung Metastases Influence the Surgical Management of Children with Hepatoblastoma? An Update and Systematic Review of the Literature

Approximately 20% of children with hepatoblastoma (HB) have metastatic disease at diagnosis, most frequently in the lungs. In children with HB, lung metastatic disease is associated with poorer prognosis. Its treatment has been approached with a variety of methods that integrate chemotherapy and surgical resection. The timing and feasibility of complete extirpation of lung metastases, by chemotherapy and/or metastasectomy, is crucial for the surgical treatment of the primary liver tumor, which can vary from major hepatic resections to liver transplantation (LT). In children with unresectable HB, which can be surgically treated only by LT, the persistence of unresectable metastases after neoadjuvant chemotherapy excludes the possibility of recurring to LT with consequent negative impact on patients’ outcomes. Due to limited evidence and experience, there is no consensus amongst oncologists and surgeons across institutions regarding the surgical treatment for HB with synchronous metastatic lung disease. This narrative review aimed to update the current management of pulmonary metastasis in children with HB and to define its role in the decision-making strategy for the surgical approach to primary liver tumours.

State-of-the-art MR Imaging of Uncommon Hepatocellular Tumours: Fibrolamellar Hepatocellular Carcinoma and Combined Hepatocellularcholangiocarcinoma

Background: Fibrolamellar Carcinoma (FLC) and Combined Hepatocellular- Cholangiocarcinoma (CHC) are rare primary liver tumours, which are related to different clinical settings. In both tumours, correlation with clinical data and laboratory tests are extremely important. </P><P> Discussion: Typically, FLC is diagnosed in young patients without any chronic disease and with normal biochemical tests, whereas CHC arises in cirrhotic patients with elevated tumour markers: AFP and/or CA 19-9. </P><P> The review describes epidemiology, aetiology, pathogenesis, radiological features and treatment of these tumours. </P><P> Imaging features typical for FLC are: The presence of central scar, calcifications, the large size, heterogeneous and early contrast-enhancement. Conclusion: The diagnosis of CHC may be suggested in case of elevation of both AFP and CA 19- 9 or inconsistency between elevated tumour markers and imaging findings (i.e., elevated CA 19-9 and radiological features of HCC, or elevated AFP with imaging findings characteristic of ICC).