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journal publication
In this study, researchers reviewed the detailed medical records of 262 patients under age 20 diagnosed with liver cancers, assembled from 19 different hospitals and covering the years 1990 to 2017. This represents the richest available source of data on these pediatric liver cancers. They found that 119 (45%) of the tumors had been classified …
“Organoids,” three-dimensional cell cultures that mimic critical features of normal human organs, are a novel tool to study fundamental steps in the development of cancers. The authors of this paper, a team from Utrecht, The Netherlands, wanted to understand how specific genetic alterations contribute to the transformation of normal liver cells into fibrolamellar carcinoma (FLC). …
Nearly all FLC tumors carry the fusion gene DNAJB1-PRKACA and the expressed protein chimera DNAJ-PKAc which is believed to be the driver of FLC formation. However, to identify potential new therapies for FLC, it is important to understand the specific mechanisms that drive the progression of the disease. In a previous study, the Sethupathy lab …
Fibrolamellar carcinoma (FLC) is driven by a unique fusion protein called DNAJB1::PRKACA (DP). This fusion is responsible for the growth of the tumor cells, and without it, the tumor dies. Unfortunately, targeting this fusion with drugs has proven extremely difficult, because the fusion’s active site is almost identical to that of normal protein kinase A …
In this study, published in March 2024, the researchers looked at how a patient’s immune system responds to the protein produced by the fusion of the genes DNAJB1 and PRKACA in FLC. Specifically, the team aimed to understand how the immune system’s “killer” T cells (CD8s) respond to this mutation and evaluate T cell receptors …
This paper was written by a large team of researchers at the University of Washington, Fred Hutchinson Cancer Center, and Cornell University, led by senior authors Dr. Venu G. Pillarisetty and Dr. Praveen Sethupathy. The group includes surgeons, cancer biologists, immunologists, and data scientists who focus on fibrolamellar carcinoma (FLC). The goal of the study …
The European Association for the Study of the Liver (EASL) convened an international, multidisciplinary panel to evaluate and summarize current knowledge on rare primary liver cancers. The resulting paper focused in three major rare liver cancers, combined hepatocellular–cholangiocarcinoma (cHCC‑CCA), fibrolamellar carcinoma (FLC), and hepatic epithelioid hemangioendothelioma (HEHE), as well as several ultra‑rare varieties. Due to …
This study represents one of the most comprehensive molecular analyses performed on fibrolamellar carcinoma (FLC). Using more than 1,400 tumor and normal liver samples, including 220 FLC and FLC‑like tumors, the research team used techniques including RNA sequencing, DNA sequencing, proteomics, methylation analysis, and spatial single‑cell mapping to better understand FLC at the molecular level. …
This paper reports an important research tool, the first example of a tumor line from a human fibrolamellar carcinoma (FLC) patient that can be maintained and expanded by growing the tumors in special mice with a compromised immune system. Such so-called “patient-derived xenografts” (PDX) can be used to provide large amounts of tumor tissue for …