Annals of Diagnostic Paediatric Pathology
Annals of Diagnostic Paediatric Pathology
Annals of Diagnostic Paediatric Pathology
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112<br />
tiple postoperative chemotherapy courses, and were qualified<br />
for total hepatectomy <strong>of</strong> the remaining liver and orthotopic<br />
liver transplantation. Another 2 children were transplanted<br />
immediately after initial chemotherapy. In 1 patient only palliative<br />
treatment (chemotherapy) was administered.<br />
Patients with HCC<br />
There were 19 patients aged 3 to 18 years treated for HCC. In<br />
9 children initial chemotherapy was administered, followed<br />
by partial liver resection, in 2 <strong>of</strong> them despite multiple resections<br />
tumor recurred and salvage transplantation was done in<br />
both <strong>of</strong> them. In 5 children liver transplantation was performed<br />
as primary surgical treatment. All <strong>of</strong> them exceeded Milano<br />
criteria for transplantation in patients with HCC. Initial chemotherapy<br />
was given in 4 <strong>of</strong> them. In 4 pts liver transplantation<br />
was performed due to suspected tumor development in<br />
cirrhotic liver (on the basis <strong>of</strong> CT or ultrasound findings or<br />
growing serum alphafetoprotein concentration). No<br />
pretransplant chemotherapy was administered to these patients.<br />
In 1 child only palliative chemotherapy was given.<br />
reccurence. One patient is alive after extensive resection, however<br />
with poor prognosis due to extrahepatic invasion <strong>of</strong> the<br />
tumor at the time <strong>of</strong> surgery.<br />
Among patients transplanted, 9/11 (81,8%) are alive,<br />
while 2 deaths were not related to tumor reccurence (early<br />
graft failure in 1 and chronic rejection in 1 (Fig. 2).<br />
In all 4 patients with cirrhosis and suspected tumor<br />
small HCC was found in explanted liver. One <strong>of</strong> these patients<br />
died <strong>of</strong> graft primary non-function. One <strong>of</strong> two patients<br />
who underwent multiple resections before transplantation presented<br />
pulmonary metastases almost 2 years after transplantation<br />
and extensive metastasectomy was performed, however<br />
prognosis for this patient is rather poor. No recurrence or<br />
metastatic dissemination was noted until now among 5 patients<br />
who underwent primary liver transplantation after total<br />
hepatectomy.<br />
Results<br />
Hepatoblastoma<br />
Twelve out <strong>of</strong> 23 (52,2%) children with HBL are alive and<br />
without active disease, while 11 patients died (47,8%). Best<br />
results were obtained with standard therapy consisting <strong>of</strong> chemotherapy,<br />
tumor resection and postoperative chemotherapy<br />
with long term survival <strong>of</strong> 11/20 pts (55,0%), including two<br />
patients who died after transplantation performed due to tumor<br />
recurrence. Among 2 patients who were transplanted primarily<br />
- 1 died 6 months after transplantation due to<br />
posttransplant lymphoproliferative disease. Another one is<br />
alive and well 25 months after Tx.Two additional children<br />
transplanted for tumor recurrence after previous resections died<br />
<strong>of</strong> metastatic disease despite <strong>of</strong> posttransplant chemotherapy.<br />
No graft involvement was observed in these patients (Fig. 1).<br />
Hepatocellular carcinoma<br />
Overall survival among 19 children with HCC is 12/19<br />
(63,2%), 42,9% (3/7) children are alive and free <strong>of</strong> tumor<br />
after CHT and resection, while all other died <strong>of</strong> tumor<br />
3<br />
3<br />
00000000000000000<br />
alive&well<br />
e e ea<br />
e a e alive wi<br />
Fig. 2 Hepatocarcinoma - actual results according to type <strong>of</strong> treatment (1990-<br />
2004)<br />
Posttransplant observation is still relatively short in survivors<br />
(range: 5 - 40 months). AFP concentration remains within<br />
normal limits in all, however it is necessary to say that it was<br />
not elevated in 2 <strong>of</strong> 11 before transplantation (Fig. 3). Chemotherapy<br />
(1-3 courses) was given after transplantation to 5<br />
patiens. It had to be stopped in most <strong>of</strong> them after 1-2 courses<br />
due to complications. Liver graft function is good in all but<br />
one patient who developed severe cholangitis after chemotherapy,<br />
further complicated by intrahepatic bile duct strictures.<br />
1<br />
1<br />
11<br />
0000<br />
000<br />
AFP (ng/ml)<br />
0000<br />
000<br />
0000<br />
1 1<br />
1<br />
000<br />
0<br />
before treatment before a t<br />
0000000000000000000<br />
alive&well<br />
e e ea<br />
e a e<br />
Fig. 1 Hepatoblastoma - results according to type <strong>of</strong> treatment (1990-2004)<br />
Fig. 3 Alphafetoprotein (AFP) in 9 patients with HCC after liver transplantation<br />
(follow up 5-40 months)
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