BMC Pediatrics· 2026Q1· Case report
Multidisciplinary management of congenital portosystemic shunt complicated by multifocal hepatoblastoma: a case report
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- Q1SCImago
- 2026year
Short summary
A 2-year-old male with congenital portosystemic shunt (CPSS) and multifocal hepatoblastoma (HB) achieved complete disease control via a multidisciplinary approach combining anatomical resection with shunt closure, microwave ablation, and chemotherapy.
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Key points
- A 2-year-old male presented with congenital portosystemic shunt (Park type I) and multifocal hepatoblastoma (PRETEXT stage III).
- A multidisciplinary treatment strategy included anatomical segment IV resection with concurrent shunt closure, microwave ablation, and chemotherapy.
- The patient achieved complete local tumor control and shunt occlusion.
- Postoperative follow-up at 10 months showed no evidence of disease, confirmed by AFP monitoring, MRI, and CT scans.
AI-generated from the title and abstract; the full text is not read.
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.
The authors' abstract, as published at the source. BMC Pediatrics, 2026 · DOI ↗
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Field: Hepatology
HepatologyMedicine