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.
Highlights
Hepatoblastoma (HB) is the most common primary malignant liver neoplasm in children
In the vast majority of cases, it is associated with elevated alpha-fetoprotein (AFP), which is helpful in diagnosis and monitoring response to treatment as well as the follow-up
HB is associated with very good survival, in the range of 70–80%, before the introduction of chemotherapy (CHT) it was below 30%2
Summary
Hepatoblastoma (HB) is the most common primary malignant liver neoplasm in children[1]. It is clear from the basic study by Otte et al that salvage LTX in HB (for example, performed for local relapse or incomplete previous tumor resection) is associated with significantly inferior survival when compared with primary LTX (70% versus less than 30%)[12] This observation became a cornerstone to favor LTX over aggressive liver resections. In patients who underwent partial liver resection, a different policy was used: pulmonary metastasectomy followed hepatectomy and usually was preceded by one or two CHT courses This approach resulted from two facts: a fear that hepatic growth factors are excreted in the regeneration process after hepatectomy which may contribute to the development/growth of lung metastases and a chance for complete regression of metastases in the course of postoperative CHT17. Competing interests The authors declare that they have no competing interests
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