Abstract
INTRODUCTION: Reports of congenital hepatoblastoma are rare, and there is limited experience in its management. CASE PRESENTATION: We present a challenging case of congenital hepatoblastoma that was large at the time of presentation, occupying the first and second hepatic portals and deemed inoperable. Although liver tumors was detected in the child during the mother's pregnancy, the initial diagnosis was hepatic hemangioma. The diagnosis of hepatoblastoma was ultimately confirmed after a biopsy. Neoadjuvant chemotherapy, guided by 3D visual analysis based on enhanced CT, enabled successful block resection of the tumor. Despite a transient cholestatic parcel effusion post-operation, the child achieved good therapeutic outcomes with subsequent drainage and chemotherapy. DISCUSSION: Regular monitoring of alpha-fetoprotein (AFP) levels and performing abdominal ultrasounds are useful for the differential diagnosis of liver tumors; however, pathology remains the gold standard for confirming malignancy. Chemotherapy is safe and effective for treating congenital hepatoblastoma in the perinatal period. 3D visual analysis is valuable tools in performing surgeries on children with large, strategically positioned tumors. Lens culinaris agglutinin-reactive fraction of AFP (AFP-L3) has been assessed for its adjuvant therapeutic efficacy in adult hepatocellular carcinoma, and we have preliminarily investigated its potential role in evaluating the treatment efficacy of congenital hepatoblastoma. CONCLUSION: Puncture biopsy is a definitive and safe diagnostic method for congenital hepatoblastoma, while neoadjuvant chemotherapy is effective and facilitates subsequent complete tumor resection. Additionally, 3D visual analysis shows significant potential in the surgical treatment of pediatric liver masses.