Pediatric Transplantation. 2018;e13113. wileyonlinelibrary.com/journal/petr
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https://doi.org/10.1111/petr.13113
© 2018 John Wiley & Sons A/S.
Published by John Wiley & Sons Ltd
Accepted: 7 December 2017
DOI: 10.1111/petr.13113
ORIGINAL ARTICLE
Prognostic and therapeutic factors influencing the clinical
outcome of hepatoblastoma after liver transplantation: A
single-institute experience
Katsutsugu Umeda
1
| Hideaki Okajima
2
| Koji Kawaguchi
1,3
| Seishiro Nodomi
1
|
Satoshi Saida
1
| Itaru Kato
1
| Hidefumi Hiramatsu
1
| Eri Ogawa
2
| Atsushi Yoshizawa
2
|
Shinya Okamoto
2
| Shinji Uemoto
2
| Kenichiro Watanabe
1,3
| Souichi Adachi
4
Abbreviations: 5FU, 5-fluorouracil; ACR, acute cellular rejection; ADR, adriamycin; AFP, alpha-fetoprotein; ANC, absolute neutrophil count; CBDCA, carboplatin; CDDP, cisplatin; Cy, cyclophos-
phamide; DOD, died of disease; HB, hepatoblastoma; HD, high dose; ICG, indocyanine green; IFO, ifosfamide; IRI, irinotecan; LT, liver transplantation; Mel, melphalan; mPSL, methylprednisolone;
N.E., not evaluated; NA, not available; NED, no evidence of disease; OS, overall survival; PBSCT, peripheral blood stem cell transplantation; Plt, absolute platelet count; PRETEXT, pretreatment
extent of disease; TAC, tacrolimus; Tepa, thiotepa; THP-ADR, tetrahydropyranyl-adriamycin; Topo, topotecan; UFT, tegafur-uracil; VCR, vincristine; VP16, etoposide.
1
Department of Pediatrics, Graduate School of
Medicine, Kyoto University, Kyoto, Japan
2
Department of Pediatric Surgery, Graduate
School of Medicine, Kyoto University, Kyoto,
Japan
3
Department of Hematology and
Oncology, Shizuoka Children’s Hospital,
Shizuoka, Japan
4
Human Health Sciences, Graduate School of
Medicine, Kyoto University, Kyoto, Japan
Correspondence
Souichi Adachi, Human Health Sciences,
Graduate School of Medicine, Kyoto
University, Kyoto, Japan.
Email: adachiso@kuhp.kyoto-u.ac.jp
Abstract
LT has contributed to an elevation in cure rates for patients with unresectable HB;
however, patients with recurrent HB after LT have poor prognosis. To analyze the
prognostic and therapeutic factors that influence the clinical outcome of patients
with HB receiving LT, we retrospectively analyzed 24 patients with HB who under-
went LT between 1997 and 2015. The 5-year OS rate of all patients was 69.6±9.7%.
The 5-year OS rate of 11 patients receiving salvage LT for recurrent tumor after a
primary resection was comparable to that of 13 patients receiving primary LT.
Among 12 evaluable patients receiving primary LT, six of 10 patients with a decline
of serum AFP >95% at LT are currently alive and in remission, whereas two pa-
tients with a decline of AFP ≤95% experienced post-LT relapse. Among 9 evalua-
ble patients receiving salvage LT, all three patients with any decline of AFP at LT
are currently alive in remission, and three of six patients with no response to pre-
LT salvage chemotherapy are also alive and in remission. Response to chemother-
apy may be a reliable marker for prediction of post-LT relapse, even for patients
receiving salvage LT.
KEYWORDS
AFP, HB, high-dose chemotherapy, LT, recurrence
1 | INTRODUCTION
Recent progress in both surgical techniques and neoadjuvant and ad-
juvant chemotherapy has resulted in improved prognosis for patients
with HB, a malignant tumor that primarily occurs in childhood.
1,2
Furthermore, LT performed as primary surgery (primary LT), or for
treatment of a recurrent tumor after a primary resection (salvage LT),
has recently contributed to an elevation of cure rates for patients with
unresectable HB tumors.
3-9
Post-LT relapse is observed in approximately 20%-30% of pa-
tients with HB, and the prognoses for such patients are extremely
dismal
3-9
; however, there are numerous unresolved problems