Pediatric Oncology

Hepatoblastoma is the most common primary liver malignancy in children, typically presenting in the first decade of life with peak incidence in infancy [e291-c1, e291-c4]. Diagnosis relies on markedly elevated alpha-fetoprotein in conjunction with imaging; AFP >100 ng/mL in a one-year-old with a liver mass strongly suggests hepatoblastoma . The PRETEXT radiographic staging system (pre-treatment extent of disease) stratifies tumors by the number of contiguous liver sectors free of tumor, guiding surgical planning [e291-c20–c25]. Upfront resection is appropriate only for PRETEXT I–II lesions with ≥1 cm margin from major vessels and no annotation factors (V, P, E, F); all others require neoadjuvant chemotherapy [e291-c27, e291-c31]. Most tumor shrinkage occurs within the first two cycles of cisplatin-based therapy, after which resectability should be reassessed [e291-c45, e291-c46]. The overarching goal is safe complete resection with negative margins; gross residual disease is unacceptable [e291-c19, e291-c69]. Multifocal disease, PRETEXT IV, or inability to achieve R0 resection after chemotherapy warrants referral for liver transplantation [e291-c47, e291-c65]. Pulmonary metastases—present in a substantial minority—respond well to chemotherapy; rapid responders may not require metastasectomy, whereas persistent nodules should be excised [e291-c52–c54]. Histology drives risk stratification: pure well-differentiated fetal histology after upfront resection requires no adjuvant therapy, whereas small-cell undifferentiated or anaplastic elements confer high risk [e291-c28, e291-c42]. Age ≥8 years and low AFP (<100) are adverse prognostic features [e291-c50, e291-c51]. Surgical technique emphasizes vascular control, hypovolemic anesthesia, anatomic resection, and meticulous hemostasis; lymph node sampling is mandatory [e291-c57–c61]. Extreme resections (vena-cava or hepatic-vein reconstruction) carry higher morbidity than transplant without clear survival benefit and should be reserved for select cases [e291-c66–c68]. Local recurrence after negative-margin resection is an indication for salvage transplantation .