Ann Liver Transplant.  2021 May;1(1):105-111. 10.52604/alt.21.0008.

Living donor liver transplantation in a pediatric patient with hepatic angiosarcoma: a case report

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

• KMID: 2560859
• DOI: http://doi.org/10.52604/alt.21.0008

Abstract

Hepatic angiosarcoma (HAS) is a rare malignant disease in pediatric patients. We report the case of a 3‐year‐old boy with HAS, which was treated with neoadjuvant chemotherapy and living donor liver transplantation (LDLT). A previously healthy 3‐ year‐old boy who presented with a firm mass in the upper quadrant of the abdomen was diagnosed with hepatoblastoma at a local general hospital and was referred to our institution. Percutaneous liver biopsy confirmed the diagnosis of HAS. The extent of the tumor was large, not allowing surgical resection; thus neoadjuvant chemotherapy was performed. The size of the tumor was markedly reduced after 2 cycles of chemotherapy for 2 months; thus LDLT was planned to remove the tumor completely. A left lateral section graft weighing 280 g was harvested from his 38-year-old father. The left lateral section graft was implanted according to the routine procedures of pediatric LDLT, including patch venoplasty of the recipient hepatic vein and portal vein. The explant liver showed a 9 cm-sized residual angiosarcoma with 60% regression. The patient recovered uneventfully and is doing well for 3 months with scheduled adjuvant chemotherapy. Although there are only a few pediatric liver transplantation cases showing prolonged survival, liver transplantation appears to be a viable treatment option for long‐term survival for pediatric patients with unresectable HAS.

Keyword

Hepatic malignancy; Chemotherapy; Venoplasty; Pediatric transplantation; Recurrence

Figure

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  Figure 1 Initial radiologic study findings. At three months before the liver transplantation operation, computed tomography (A, B) and magnetic resonance imaging (C) studies show huge multiple tumors occupying the whole liver, and the abdomen is distended by the enlarged liver. Whole-body positron emission tomography (D) shows a heterogenous mild hypermetabolic huge mass in the liver. The diagnosis from these imaging studies is hepatoblastoma and differential diagnoses include solid variant of mesenchymal harmatoma, inflammatory pseudotumor and hemangioma.

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  Figure 2 Pretransplant computed tomography findings. The tumors show noticeable interval decrease in size (A), encasement of the middle and left hepatic veins (B), encasement of the right and left portal veins (C), and encasement of the second-order right hepatic artery branches (D).

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  Figure 3 Intraoperative findings showing the extent of hepatic angiosarcoma at the dome (A) and hilar area (B).

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  Figure 4 Intraoperative findings showing patch venoplasty of the recipient hepatic vein stump. (A) The major hepatic vein stumps are transected without leaving any hepatic parenchymal tissues. (B) Because the tumor is located very close to the left hepatic vein trunk, the left hepatic vein stump is resected further. (C, D) The left hepatic vein stump appears to be very short and cannot be reconstructed directly; thus a small patch of iliac vein homograft (arrows) is attached.

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  Figure 5 Intraoperative findings showing patch venoplasty of the recipient portal vein. (A) The recipient portal vein appears relatively small due to removal of the hilar bifurcation portion. (B) A small longitudinal incision is made at the portal vein stump. (C, D) A small iliac vein homograft patch is attached to enlarge the portal vein orifice.

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  Figure 6 Intraoperative findings showing implantation of the left lateral section graft. (A–C) Size-matched reconstruction of the graft hepatic vein. The intervening vein patch is visible (arrow). (D, E) Size-matched reconstruction of the graft portal vein. The intervening vein patch is visible (arrow). (F) Gross finding of the left lateral section graft after portal vein reperfusion.

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  Figure 7 Gross photographs of the explanted liver. There is a large viable tumor with extensive necrosis.

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  Figure 8 Computed tomography findings at five days after transplantation. (A) The graft hepatic vein reconstruction shows only a slight stenosis at the anastomotic site (arrow). (B) The funnel-shaped recipient portal vein is well visualized (arrow).

Reference

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