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Abstract
Background
Primary liver tumors account for less than 2% of pediatric malignancies, and the best treatment is complete surgical excision. The aim of this study was to review the results of liver transplantation (LT) for primary hepatomas in children.
Methods
The medical records of patients who underwent LT for unresectable primary hepatoma between May 1996 and December 2009 were reviewed retrospectively.
Results
Seven of 130 patients (5.3%, M:F=4:3) underwent LT for unresectable hepatoma. The median age at transplantation was 9 years (range, 6 months-14 years). Two patients were transplanted for hepatitis B virus-associated hepatocellular carcinoma (HCC), 2 for hepatoblastoma, 1 for hemangioendothelioma, 1 for angiosarcoma, and 1 for intrahepatic cholangiocarcinoma after a Kasai operation for biliary atresia. There was no post-LT treatment except in patients with HCC who were taking immunoglobulin prophylaxis against hepatitis B. Four patients (2 HCC, 1 hepatoblastoma, 1 hemangioendothelioma) are now alive and well after 7.8, 7.2, 7.7, 6.3 years of follow-up, respectively. Three patients died after transplantation; 1 for the recurrent cholangiocarcinoma in the transplanted liver 1 year after the transplantation and 1 who underwent LT for the recurrent hepatoblastoma for the primary non-function 10 days after the transplantation. One patient died of metastatic angiosarcoma (bone) 2.5 years after LT.
Conclusions
LT can be tried for unresectable primary hepatoma in children and, although limited, the outcome was successful in patients with HCC, hepatoblastoma, or hemangioendothelioma. Careful patient selection, based on the pre-transplant histological diagnosis, seems to be related to better outcome.
References
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Fig. 1.
Abdominal CT scan of a child with multiple hepatoblastoma. Abbreviation: CT, computed tomography.
Fig. 2.
Abdominal CT scan of a child with centrally located large, unresectable angiosarcoma. Abbreviation: CT, computed tomography.
Fig. 3.
Abdominal CT scan of a child with intrahepatic cholangiocarcinoma, who underwent kasai operation for biliary atresia. Abbreviation: CT, computed tomography.
Table 1.
Clinical characteristics and treatment outcome of each patients
| Case No. | Sex | Age a | LT type | Post-LT diagnosis | Post-LT treatment | F/U duration b | Current status | Cause of death |
|---|---|---|---|---|---|---|---|---|
| 1 | M | 110 | C | HCC | HBIG | 7.8 | NED | – |
| 2 | M | 116 | L | HCC | HBIG | 7.2 | NED | – |
| 3 | M | 19 | L | Hepatoblastoma | – | 7.7 | NED | – |
| 4 | M | 103 | L | Hepatoblastoma | – | 0.0 | DOD | Hepatic failure |
| 5 | F | 6 | L | IHHE | – | 6.3 | NED | – |
| 6 | F | 35 | L | Angiosarcoma | CTx | 2.5 | DOD | Recurrence |
| 7 | F | 163 | L | Cholangiocarnoma | – | 1.0 | DOD | Recurrence |
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