Korean J Transplant.  2022 Jun;36(2):148-153. 10.4285/kjt.21.0008.

Pediatric living donor liver transplantation for biliary embryonal rhabdomyosarcoma: a case report of a case showing disease-free survival over 2 years

• Namgoong JM
• Hwang S
• Park GC
• Kwon H
• Ha S
• Oh SH
• Kim KM

• KMID: 2531139
• DOI: http://doi.org/10.4285/kjt.21.0008

Abstract

Biliary rhabdomyosarcoma is a rare tumor, but it is still the most common tumor of the biliary tract in children. We report a case of a 6-year-old boy with biliary embryonal rhabdomyosarcoma and liver metastasis, which were treated with neoadjuvant and adjuvant chemotherapy combined with living donor liver transplantation (LDLT). Initial imaging studies showed a low-attenuation intraductal mass from the left hepatic duct to the intrapancreatic common bile duct with diffuse upstream dilatation of the intrahepatic duct and liver metastasis. Endoscopic biopsy revealed embryonal rhab- domyosarcoma. After tumor size reduction through neoadjuvant chemotherapy, LDLT was planned to remove the tumor completely. A left lateral section graft weighing 330 g was harvested from his 38-year-old mother and the graft-to-recipient weight ratio was 1.94%. Routine pediatric LDLT operation was performed with deep excavation of intra-pancreatic distal bile duct. The explant liver showed minimal residual embryonal rhabdomyosarcoma with no lymph node metastasis. The patient recovered uneventfully from LDLT operation. Scheduled adjuvant chemotherapy was performed for 6 months. The patient is doing well without any evidence of tumor recurrence for 26 months after LDLT. In conclusion, liver transplantation could be an effective treatment for unresectable biliary rhabdomyosarcoma in children according to the location of tumor.

Keyword

Hepatic malignancy; Chemotherapy; Living donors; Liver transplantation; Sarcoma; Metastasis

Figure

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  Fig. 1 Initial radiologic findings. Computed tomography shows a low-attenuation intraductal mass from the left hepatic duct (A) to the intrapancreatic distal bile duct (B). Arrows indicate the intraductal mass.

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  Fig. 2 Whole body fluorodeoxyglucose-positron emission tomography reveals a hypermetabolic mass in the left hepatic duct and common bile duct, suggesting a biliary tract tumor (arrow).

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  Fig. 3 Pretransplant radiologic study findings after chemotherapy for 3 months. The intraductal mass shown on the magnetic resonance cholangiography (A) and computed tomography (B) is reduced in size. Arrows indicate the intraductal mass.

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  Fig. 4 Gross finding of the explant liver. (A) The mass is confined to the liver. (B) The liver parenchyma shows fatty change, multifocal sinusoidal dilatation, and congestion.

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  Fig. 5 Computed tomography taken 2 days after transplantation. There is no abnormal finding in the graft parenchyma (A) and graft outflow vein reconstruction (B).

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  Fig. 6 Serial follow-up of computed tomography scans. There is no evidence of tumor recurrence in the images taken at 5 (A), 12 (B), 18 (C), and 24 (D) months after transplantation.

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