Multidisciplinary management of congenital portosystemic shunt complicated by multifocal hepatoblastoma: a case report

Abstract

Congenital portosystemic shunt (CPSS) is a rare developmental malformation that predisposes individuals to an increased risk of hepatic tumors. Hepatoblastoma (HB) complicating CPSS is especially rare. This article reports a 2‑year‑old male with CPSS (Park type I) and HB (PRETEXT stage III), who underwent a multidisciplinary treatment, including anatomical segment IV resection with concurrent shunt closure, ultrasound‑guided microwave ablation (MWA), and chemotherapy. The shunt was successfully occluded and complete local tumor control was achieved. Postoperative follow‑up at 10 months showed no evidence of disease, supported by serial AFP monitoring, contrast‑enhanced abdominal MRI, and chest CT. Currently, standardized diagnostic and therapeutic protocols are lacking for managing congenital portosystemic shunts accompanied by multifocal HB. This case illustrates a unique multidisciplinary strategy combining upfront anatomical resection of the dominant tumor with concurrent shunt closure, followed by targeted MWA and adjuvant chemotherapy. Our experience suggests that this protocol is safe and feasible, and may serve as a reference model for similar complex cases.