Hepatoblastoma and hepatocellular carcinoma in children

1. HEPATOBLASTOMA AND HEPATOCELLULAR
CARCINOMA IN CHILDREN
Chinsu Liu1, Hsin-Lin Tsai1, Taiwai Chin1, Choufu Wei1, 2
1Division of Pediatric Surgery, Department of Surgery, Taipei
Veterans General Hospital, National Yang-Ming University,
2National Taipei Medical University, Taiwan
1

2. Background and Methods
Background. The aim is to evaluate the
surgical results of HB in Taipei VGH since
1996 and discuss the better surgical
strategy to HB.
Methods. Retrospective chart reviews.
2

3. Algorithm for management of HB (J Pediatr 2005)
Need C/T before
Resection?
Is Biopsy necessary for
every case?
What is Resectable?
Is it necessary for C/T
After transplant?
What is Resectable?3

4. Resectability of HB
 PRETEXT or POSTTEXT (post-C/T) I,
II
 Distant lung metastasis is not
contraindication to resection of HB.
 PRETEXT III ? C/T first
 PRETEXT IV ??? C/T first
 POSTTEXT (post-C/T) III? resection
or primary liver transplantation
 POSTTEXT (post-C/T) IV resection
or primary liver transplantation
 Liver recurrence after C/T and
resection re-resection or rescue liver
transplantation
4

5. Patients
 Totally 14 patients
 12 surgical fresh cases. 2 referred recurrent cases.
 Gender: male predominant. M/F=11/3
 Ages: 2 to 36 m/o (mean, 18 m/o) for fresh cases, 4
y/o and 13 y/o for 2 referred cases.
 3 cases were twins (21.4 %, 3/14)
 AFP values: very high in 13 (92.8%) slightly high in 1
case (87 U/L)
5

6. Fresh cases (n=12)
 Hepatectomy before chemotherapy (n= 4), hepatectomy after chemotherapy (n= 8).
 One case with distal lung metastasis (8.3%), the lung lesions disappeared after C/T and liver
resection, proved by exploratory thoracotomy.
 Resectable before chemotherapy (PRE-TEXT II, n=6, Gr. R). Became resectable after
chemotherapy (PRE-TEXT IV to POST-TEXT II, n=3, Gr. Rc). Resectability was controversial and
highly dependent on surgeon’s personal experience (PRE-TEXT IV to POST-TEXT III or IV, n=3,
Gr. Rqc).
 Results: (F/U: 1 to 17 yrs; mean: 6.8 yrs; median:5yrs)
 Lt lobectomy in 2, Rt lobectomy in 6, Extended Rt lobectomy in 4.
 Complications: bile leak (n=2)
 One case in Gr R received chemotherapy first had recurrent HB and received live donor liver
transplantation (LDLT) immediately after recurrence and got a long term tumor free survival.
(F/U: 6 yr)
 One case in Gr R received chemotherapy first died of other congenital anomaly (trisomy 18).
 A long-term tumor free survival is achieved in 91.7% of our fresh cases.
6

7. Case in Gr. R (Hepatectomy before chemotherapy, PRE-TEXT II)
Blood loss: 600 cc
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8. Case 1 of three Gr. Rqc (PRE-TEXT IV to POST-TEXT III)
(Pre-C/T)
(Post-C/T)
Surgical key points:
Hepatic hilar dissection
Blood loss: 80 cc
8

9. Case 2 of three Gr. Rqc (PER-TEXT IV to POST-TEXT III)
(Pre-C/T) (after 4 course of C/T)
(after 6 course of C/T)
Surgical key points:
Hepatic hilar dissection
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10. Blood loss: 300 cc
10

11. Case 3 in three Gr. Rqc cases (PRE-TEXT IV to POST-TEXT IV)
(pre-C/T)
(post-C/T)
Surgical key points:
1. Hilar dissection
2. Dissection of
common trunk of
LHV and MHV
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12. Delicate dissection of hepatic hilum
12

13. Delicate dissection of common trunk of LHV and MHV
Blood loss: 40 cc
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14. The case in Gr R received chemotherapy first had recurrent HB and received live donor liver
transplantation (LDLT) immediately after recurrence and gets a long term tumor free survival.
(F/U: 6 yr)
3 months after right lobectomy
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15. Patient survival
Our data (12 fresh patients,
1996-2012)
-pulmonary meta in 1 at
diagnosis
-1 died of unrelated disease
J Formos Med Assoc 2011 (LKCGMH)
--35 pts during 1990-2009
--pulmonary meta in 4 at diagnosis.
--liver resection in 31 pts
--2 peri-operative death, 6 died of
progressive disease (4 with lung meta)
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16. Indications and outcomes to liver transplantation
for hepatoblastoma
 If aggressive resection is necessary or bilobar disease
persist, primary transplantation is recommended. --J
Pediatr 2005
 (Post-C/T) POST-TEXT IV or III in contact with hepatic
vein or portal vein, or centrally located was
recommended as primary surgery.-- Pediatr Transpl
2005
 6-year survival of primary vs rescue (incomplete tumor
resection) liver transplantation: 82% vs 30%-- Pediatr
Transpl 2005
 1, 5, and 10-year survival of liver transplant for HB
(total n=25, 8 received exp. lapa and 2 of them
received hepatic resection): 91 %, 77.6% and 77.6%
respectively.-liver transpl 2008
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17. Debates on liver resection and transplantation
(Lautz TB, et al. Cancer 2011)
 Successful nontransplant resection of POST-TEXT III and
IV HB.
 Excellent survival was obtained with aggressive
resection in POST-TEXT III and IV HB meeting criteria for
transplant referral: 1, 2, and 5-year survival (total
n=14, 1 received transplant after liver resection): 93 %,
91% and 75% respectively.
 PRETEXT/POST-TEXT system overstages in up to 40%,
and operative exploration is frequently needed to
determine resectabililty.
 Referral to institutions with expertise in both pediatric
liver transplantation and hepatobiliary surgery is
essential.
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18. Two referred recurrent cases
 One 4 y/o boy s/p hepatectomy and
wedge resection of lung at the age of 2.
Live donor liver transplant was done but
tumor recurrence was noted 4 months after.
 One 13 y/o boy s/p multiple hepatectomy.
Tumor recurrence involving right diaphragm
and IVC. Referred for liver transplantation.
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19. Auto-transplantation: the reason and the
feasibility
 Liver transplant: poor survival
and difficult to reconstruct hepatic
outflow in living donor liver
transplantation
 Liver resection and en bloc
resection of involved vena
cava: high risk to control bleeding
and difficult to reconstruct venous
return ff lower torso and left hepatic
vein.
 Autotransplant: no
immunosuppressive medication,
reconstruct IVC by artificial graft and
reconstruct left hepatic vein outflow by
residual vena cava graft.
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20. Results: Successful operation but
compartment syndrome happened
 The course of operation is smooth.
 Blood loss: 400 cc
 Compartment syndrome
happened after closure of
abdomen and caused graft
necrosis.
 Patient died 9 days after
operation.
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21. Conclusions
 C/T before resection is not necessary in the PRE-TEXT I and II resectable
HB.
 Aggressive and skillful resection of HB may cure some image-
unresectable cases. Sometimes exploratory laparotomy is necessary
and it is highly dependent on personal experience.
 Primary liver transplant should be arranged for unresectable HB as
soon as after 4 to 6 course of C/T.
 Rescue liver transplant after tumor recurrence, particular in cases of
incomplete resection should be performed as soon as possible.
 Auto-transplant surgery may be considered for cases of great vessels
involvement or no living donor available.
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