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Page 8 of 17 Calinescu et al. Hepatoma Res 2021;7:59 https://dx.doi.org/10.20517/2394-5079.2021.26

Fahy et al. [21] , 2019 60 2001-2015 93% 78 months
[109]
Okur et al. , 2019 10 2009-2014 90% 13.5 months (8-120)
[110]
Feng et al. , 2019 93 2004-2016 10 years: 87.2% 60 months
[49]
Herden et al. , 2019 7 2007-2012 100% 7.1 years (5.7-10.7)
[80]
Laufermann et al. , 2019 21 2005-2018 5 years: 90% 22 months (0-127)
In an attempt to reduce the time between diagnosis and LT, an early referral practice was introduced in the
late 2000s, with a parallel evaluation of resection and potential LT; the major success of this approach was to
reduce the number of secondary LT .
[50]

Survival in the case of “rescue LT” seems worse at five years, at less than 30% [12,63] . Overall, a literature review
identified a survival of 41% for rescue LT . A Japanese study identified a 72% 5-year overall survival in a
[67]
series of 11 “rescue LT” patients .
[68]

LT outcomes for HBL patients with synchronous lung metastasis eradicated before LT are excellent at one
and five years, 93.3% ± 4.6% and 86.4% ± 6.3%, respectively . Single pulmonary metastasis and patients with
[69]
lesions visible only on CT vs. lesions visible on both CT and chest X-ray have a better outcome [35,70] .

The need for chemotherapy after LT is a matter of debate: a review did not identify a statistically significant
difference in survival rates with and without post-LT chemotherapy , nor did the Pediatric Liver
[6]
Unresectable Tumour Observatory registry . Some studies promote its use in the case of vascular invasion
[71]
[72]
or large proportion of viable tumor in the explanted liver . The survival rates seem improved even if
[73]
statistical significance was not reached in the series inquiring into this issue .
Recurrence after liver transplantation for hepatoblastoma
Tumor recurrence is the most frequent cause of death: up to 50% of the patients with tumor recurrence die,
usually within two years after LT [21,59,65] ; the longest interval between LT and recurrent HBL was 2.8 years in
the Japanese national survey . A review identified 14.6% of the relapse HBL patients after LT to be alive
[62]
and disease free .
[68]
Recurrence after LT for HBL presents itself mainly as metastatic disease and is encountered in as high as
40% of the cases [11,60] . It is thought to correspond to a more aggressive type of tumor to which
transplantation will not respond better than initial resection . This seems to be supported by the findings
[73]
of Khan et al. : in their institutional review, none of the HBL patients had a complete tumor response. As a
[74]
surrogate marker for recurrence, AFP levels after LT could be a valuable adjunct, with a series reporting a
normalization of AFP values in the subgroup of patients without recurrence and staying increased or even
further increasing in patients with recurrence [Table 2]. In cases with limited and regional relapse
[75]
(typically a single node in the abdominal area), re-resection strategy may offer a cure (personal
communication) .
[62]

The long-term survival of patients having a LT for HBL shows the following risk factors for tumor
recurrence: PRETEXT IV, tumor rupture, higher time spent within the waiting list (15 days vs. 31 days),
older age (78 months vs. 48 months) , macroscopic vascular invasion, extrahepatic lesions at the time of
[4]
LT, presence of viable tumor (tumor necrosis less than 50% and high preoperative AFP values), tumor
shrinkage rate of ≤ 30%, and high AFP at diagnosis and LT [11,59,62,76,24] .