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=Volume 11 - 2021

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=Pancreatoblastomas are uncommon tumors usually found in children. We report on two cases of metastatic pancreatoblastomas observed in young women. Systemic chemotherapy (FOLFIRINOX regimen) was associated with disease control in one case and partial response in the other, with improved general status for both. High-throughput sequencing of the tumor, twice identified alterations in the Wnt/β-catenin pathway: a CTNNB1 (S37C, exon 3) mutation in the first case and a breakage targeting APC (5q21–q22) in the second.