Special Issue "Pediatric Liver Tumors (Hepatoblastoma and Hepatocellular Carcinoma)"
Deadline for manuscript submissions: closed (31 May 2023) | Viewed by 7091
Interests: solid pediatric tumors; endocrine tumors; molecular genetics
Interests: liver pediatric tumors; biomarkers; molecular stratification; biology-driven therapies
Pediatric liver tumors, including hepatoblastomas (HB) and pediatric hepatocellular carcinomas (HCC), are rare but devastating malignancies, the incidence of which has been increasing over the past few decades. The pathogenesis of these tumors is only partially known, with HBs having an extremely low mutational burden, with beta catenin mutations and Wnt pathway dysregulation being the most frequent molecular abnormalities. Pediatric HCC may develop against the background of specific hereditary diseases, including tyrosinemia and progressive familial intrahepatic cholestasis or viral infections, or may not have any predisposing factors. In addition, several rare pediatric tumors exist, including mesenchymal hamartoma, undifferentiated embryonal sarcoma, malignant rhabdoid tumors, and calcifying nested stromal–epithelial tumors of the liver.
The current treatment for HB and HCC is based on an international effort that resulted in the Paediatric Hepatic International Tumour Trial (PHITT), which was launched in 2017. The PHITT is a randomised phase III trial that studies how well different drugs/protocols (i.e., cisplatin, doxorubicin, fluorouracil, vincristine sulfate, carboplatin, etoposide, irinotecan, sorafenib, gemcitabine, and oxaliplatin) work in children and young adults with HB or HCC and is stratified according to the new international clinical staging system that emerged from the Children's Hepatic tumours International Collaboration (CHIC). This stratification relies on clinical prognostic factors such as the PRETreatment EXTent of the disease stage, metastasis, patient age, AFP levels, and annotation factors related to dissemination. Despite significant advances, treatment options remain limited for ~20% of patients with aggressive tumours that are resistant to current pharmacological treatments, and survivors experience long-term effects from treatment toxicity.
Several studies have extensively reviewed the pathology of childhood liver cancer as well as explored the genetic, epigenetic, and transcriptomic profiles of pediatric liver tumors to better understand this rare disease and to identify novel biomarkers, therapeutic targets, and drugs that could improve current clinical management and move forward precision medicine in HB and pediatric HCC.
This current Special Issue of Cancers aims to collect a series of original and review articles on pediatric liver tumors from all angles of clinical and basic research, sharing both increases in the understanding of the pathogenesis of these tumors as well translational and clinical findings that may have direct effects on patient management.
Dr. Ronald de Krijger
Dr. Carolina Armengol
Prof. Dr. Sarangarajan Ranganathan
Manuscript Submission Information
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- hepatocellular carcinoma
- molecular genetics
- animal models