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Case Report - 1
Biliary tract rhabdomyosarcoma requiring liver transplantation: A challenging surgical and oncological dilemma
Betül Dölek 1, Nurşah Eker 2, Turan Kanmaz 3, Gürsu Kıyan 1, Kıvılcım Karadeniz Cerit 1
1 Marmara University Faculty of Medicine, Department of Pediatric Surgery, Istanbul
2 Marmara University School of Medicine, Department of Pediatric Oncology, İstanbul
3 Koc University, School of Medicine, Department of Pediatric Surgery
Introduction: Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children, yet its occurrence in the biliary tract is exceedingly rare. This anatomical region poses significant challenges in both diagnosis and management due to its complexity and proximity to vital vascular structures. This study underscores the limitations of current treatment strategies and the difficulty in determining transplantation candidacy.
Case Presentation: A 7-year-old girl presented with obstructive jaundice and an abdominal mass. Imaging revealed a 7×5 cm mixed cystic-solid lesion in the caudate lobe of the liver. Intraoperative findings showed a 7×8 cm infiltrative mass invading the biliary tract and displacing surrounding organs, without initial metastasis. Biopsy confirmed embryonal-type RMS. The patient received 12 cycles of chemotherapy (VAC). However, residual mass at the hepatic hilum remained unresectable due to risk of portal vein injury. Postoperatively, chemotherapy and radiotherapy were administered, however no regression in the tumor mass was observed. Eventually, liver transplantation was planned. During pre-transplant evaluation, tumor invasion into the portal vein led to hypersplenism and upper gastrointestinal bleeding. A suspicious lesion detected on endoscopy was biopsied twice; both results were negative for malignancy. After open biopsy confirmation, liver transplantation was performed. Postoperatively, complications included pancreatic fistula and sepsis. Despite multiple lines of chemotherapy (VAC, ifosfamide, etoposide, carboplatin), the disease progressed and the patient died due to gastrointestinal hemorrhage and septic complications.
Conclusion: Biliary RMS represents a rare and formidable clinical challenge. Even with aggressive multimodal treatment including chemotherapy, attempted surgical resection, and ultimately liver transplantation, outcomes may remain poor.