Congenital hepatoblastoma: Expanding knowledge, improving outcomes.

Autor: Gigola F; School of Pediatric Surgery, University of Florence, Florence, Italy.; Department of Pediatric and Neonatal Surgery, Meyer Children's Hospital IRCCS, Florence, Italy., Morini F; Department of Pediatric and Neonatal Surgery, Meyer Children's Hospital IRCCS, Florence, Italy.; Department of Neurosciences, Psychology, Drug Research and Child Health (NEUROFARBA), University of Florence, Florence, Italy., Libro G; School of Pediatric Surgery, University of Florence, Florence, Italy.; Department of Pediatric and Neonatal Surgery, Meyer Children's Hospital IRCCS, Florence, Italy., Morabito A; Department of Pediatric and Neonatal Surgery, Meyer Children's Hospital IRCCS, Florence, Italy.; Department of Neurosciences, Psychology, Drug Research and Child Health (NEUROFARBA), University of Florence, Florence, Italy., Grimaldi C; Department of Pediatric and Neonatal Surgery, Meyer Children's Hospital IRCCS, Florence, Italy.
Jazyk: angličtina
Zdroj: Pediatric blood & cancer [Pediatr Blood Cancer] 2024 Sep; Vol. 71 (9), pp. e31132. Date of Electronic Publication: 2024 Jul 02.
DOI: 10.1002/pbc.31132
Abstrakt: 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.
(© 2024 The Author(s). Pediatric Blood & Cancer published by Wiley Periodicals LLC.)
Databáze: MEDLINE
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