AUTHOR=Raoul Jean-Luc , Oziel-Taieb Sandrine , Lecomte Thierry , Adelaide José , Guille Arnaud , Chaffanet Max , Poizat Flora , Heymann Marie-Françoise , Barbier Louise , Bertucci François 

TITLE=Case Report: Two Cases of Metastatic Pancreatoblastoma in Adults: Efficacy of Folfirinox and Implication of the Wnt/β-Catenin Pathway in Genomic Analysis

JOURNAL=Frontiers in Oncology

VOLUME=11

YEAR=2021

URL=https://www.frontiersin.org/journals/oncology/articles/10.3389/fonc.2021.564506

DOI=10.3389/fonc.2021.564506

ISSN=2234-943X

ABSTRACT=<p>Pancreatoblastomas are unfrequent tumors usually found in children. We report two cases of metastatic pancreatoblastomas observed in young women. A systemic chemotherapy (FOLFIRINOX regimen) was associated with a disease control in one case and a partial response in the second with an improvement of general status for both. A high-throughput sequencing of the tumor described in both cases alteration in the Wnt/β-catenin pathway: a mutation in <italic>CTNNB1</italic> (exon 3, c.110C&gt;G, p.S37C, reported as a hotspot in COSMIC) in one case and a homozygous loss associated with breakage targeting <italic>APC</italic> (5q22.2) in the second.</p>