Case report: Two cases of metastatic pancreatoblastoma in adults: efficacy of Folfirinox and implication of the wnt/β-catenin pathway in genomic analysis

Jean-Luc Raoul (1) , Sandrine Oziel-Taieb (2) , Thierry Lecomte (3) , José Adelaide (4) , Arnaud Guille (4) , Max Chaffanet (2) , Flora Poizat (2) , Marie-Françoise Heymann (1) , Louise Barbier (3) , F. Bertucci (4)

1 UNICANCER/ICO - Institut de Cancérologie de l'Ouest [Angers/Nantes]
2 IPC - Institut Paoli-Calmettes
3 CHU Trousseau [Tours]
4 CRCM - Centre de Recherche en Cancérologie de Marseille

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 CTNNB1 (exon 3, c.110C>G, p.S37C, reported as a hotspot in COSMIC) in one case and a homozygous loss associated with breakage targeting APC (5q22.2) in the second.

Mots clés: pancreatoblastoma; adult; genomics; chemotherapy-oncology; Wnt/beta-catenin

Domaines: Cancer

Jean-Luc Raoul, Sandrine Oziel-Taieb, Thierry Lecomte, José Adelaide, Arnaud Guille, et al.. Case report: Two cases of metastatic pancreatoblastoma in adults: efficacy of Folfirinox and implication of the wnt/β-catenin pathway in genomic analysis. Frontiers in Oncology, 2021, 11, pp.1-8. ⟨10.3389/fonc.2021.564506⟩. ⟨hal-03623119⟩