Fibrolamellar carcinoma in the Carney complex: PRKAR1A loss instead of the classic DNAJB1‐PRKACA fusion
Rondell P. Graham, Carolin Lackner, Luigi Terracciano, Yessica González‐Cantú, Joseph J. Maleszewski, Patricia T. Greipp, Sanford M. Simon, Michael S. Torbenson – 9 December 2017 – Fibrolamellar carcinomas are characterized by activation of protein kinase A, a kinase composed of catalytic and regulatory subunits. PRKACA encodes a catalytic subunit of protein kinase A, and almost all fibrolamellar carcinomas have a heterozygous 400‐kb deletion that leads to the fusion of DNAJB1 and PRKACA.