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Review
. 2024 Sep;71(9):e31132.
doi: 10.1002/pbc.31132. Epub 2024 Jul 2.

Congenital hepatoblastoma: Expanding knowledge, improving outcomes

Francesca Gigola  1   2 , Francesco Morini  2   3 , Giorgia Libro  1   2 , Antonino Morabito  2   3 , Chiara Grimaldi  2
Affiliations
Review

Congenital hepatoblastoma: Expanding knowledge, improving outcomes

Francesca Gigola et al. Pediatr Blood Cancer. 2024 Sep.

Abstract

Hepatoblastoma (HB) is a rare liver tumour, and its congenital counterpart (CHB) is even less frequent. CHB has a clinically challenging management and a generally perceived worse outcome. This study aims to review the literature on CHB to better define presentation, diagnosis, available treatments and management options. The analysis of outcomes suggests that a significant portion of mortality is unrelated to the malignant nature of the tumour. Key factors influencing overall outcomes were identified: mortality linked to the 'mass effect' during both the prenatal (22%) and perinatal (32%) stages, as well as 'oncological' mortality encompassing tumour and/or treatment-related factors (46%). Overall, after birth, CHB does not seem to confer a worse oncological prognosis per se, and should be managed similarly to older children, if patients are stable enough to undergo proper staging and treatment. A deeper knowledge and better outcomes would come from a large, homogeneous, collection of data possibly allowing a global protocol, focusing on a comprehensive management of CHB.

Keywords: children; hepatic tumour; liver; newborn; prenatal tumour.

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References

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