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Review
. 2018 Jan 15:7:53.
doi: 10.12688/f1000research.12239.1. eCollection 2018.

Hepatoblastoma: current understanding, recent advances, and controversies

Piotr Czauderna  1 , Hanna Garnier  1
Affiliations
Review

Hepatoblastoma: current understanding, recent advances, and controversies

Piotr Czauderna et al. F1000Res. .

Abstract

Introduction: Hepatoblastoma (HB) is the most common primary malignant liver neoplasm in children. Its increasing survival rate is related to the progress in modern imaging, surgical techniques, and new chemotherapy regimens. Clinical approach: One of the past achievements was the development of the pretreatment extension of disease (PRETEXT) system. Gradually, the HB therapeutic approach has become more individualized with better stratification of patients. Controversies: These include the need for preoperative chemotherapy and its optimal duration; intensity of preoperative chemotherapy required for locally advanced cases (PRETEXT 4); optimal surgical treatment for locally advanced tumors: aggressive hepatic resections versus liver transplantation; the role of postoperative chemotherapy in the post-transplant setting; the timing and role of metastasectomy in patients with disseminated disease who undergo partial liver resection; and the prognostic significance of several HB pathology variants. Hepatoblastoma biology: Beta-catenin mutations and the beta-catenin/Wnt pathway play an important role in HB development. There have been at least two molecular signatures in HB published. Unluckily, all of these findings are based on relatively small clinical series and require confirmation. Conclusion: The treatment of HB started from one and the same therapy for all patients and aimed at increased treatment individualization, but the future seems to lie in biology-driven patient-tailored therapies.

Keywords: PRETEXT; biology; chemotherapy; hepatoblastoma; liver transplantation; surgical treatment.

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Conflict of interest statement

No competing interests were disclosed.No competing interests were disclosed.No competing interests were disclosed.No competing interests were disclosed.

References

    1. Czauderna P, Haeberle B, Hiyama E, et al. : The Children's Hepatic tumors International Collaboration (CHIC): Novel global rare tumor database yields new prognostic factors in hepatoblastoma and becomes a research model. Eur J Cancer. 2016;52:92–101. 10.1016/j.ejca.2015.09.023 - DOI - PMC - PubMed
    1. Chopra A, Iyer VK, Agarwala S, et al. : Apoptotic protein expression, glycogen content, DNA ploidy and cell proliferation in hepatoblastoma subtyping and their role in prognostication. Pediatr Surg Int. 2010;26(12):1173–8. 10.1007/s00383-010-2699-x - DOI - PubMed
    1. Baheti AD, Luana Stanescu A, Li N, et al. : Contrast-enhanced CT features of hepatoblastoma: Can we predict histopathology? Clin Imaging. 2017;44:33–7. 10.1016/j.clinimag.2017.03.023 - DOI - PubMed

    2. F1000 Recommendation

    1. Aronson DC, Czauderna P, Maibach R, et al. : The treatment of hepatoblastoma: Its evolution and the current status as per the SIOPEL trials. J Indian Assoc Pediatr Surg. 2014;19(4):201–7. 10.4103/0971-9261.142001 - DOI - PMC - PubMed
    1. Zsiros J, Brugieres L, Brock P, et al. : Dose-dense cisplatin-based chemotherapy and surgery for children with high-risk hepatoblastoma (SIOPEL-4): a prospective, single-arm, feasibility study. Lancet Oncol. 2013;14(9):834–42. 10.1016/S1470-2045(13)70272-9 - DOI - PMC - PubMed

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