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journal publication
This publication summarizes the current understanding of fibrolamellar carcinoma based on a review of currently available literature. It outlines the epidemiology, pathology, and biology of FLC, plus provides a high-level overview of the evidence supporting existing therapies for the disease — including surgical, radiotherapy, interventional radiology and systemic treatments. The full article can be read …
While FLC in almost all patients is caused by the DNAJB1-PRKACA gene fusion, the mechanism by which that fusion causes the cancer is still unclear. In order to get insight into that question, this team engineered the DNAJB1-PRKACADP fusion gene into “immortalized” liver cells and measured the impact that the change had on the functions …
This publication describes the results of the first large scale screening of known drugs and compounds for their effectiveness against FLC. It identified six new PDX lines derived by direct implantation of tumor specimens, both from the liver (one primary and one recurrent tumor) and metastases. These facilitated the first reported large-scale screening of compounds …
Although immune checkpoint inhibitors (ICIs) are becoming the standard of care in some types of cancers, few studies have looked at the safety and efficacy of ICIs for treating FLC. This multicenter, retrospective analysis aimed to determine if treatment with ICIs could be helpful for FLC patients. Patient data included in this study was identified …
Rare diseases like fibrolamellar are often poorly understood because of the difficulty in assembling accurate patient data and the lack of ICD billing codes specific to the disease. The most cited study of the incidence of FLC in the US, using data from the National Cancer Institute’s Surveillance, Epidemiology, and End Results (SEER) Program, reports …
Based on another study from the Simon lab, we have known that a gene fusion called DNAJB1-PRKACA produced a mutated protein kinase A (PKA) that causes 99% of cases of fibrolamellar carcinoma. In this study, the researchers found that this gene is not only important for the growth of FLC tumors but also that blocking …
Fibrolamellar carcinoma (FLC) is a rare, aggressive liver cancer primarily affecting young individuals, with limited treatment options and poor prognosis. The discovery of the DNAJB1-PRKACA gene fusion as the primary oncogenic driver in FLC over a decade ago has reshaped research efforts. The exact way the DNAJ-PKAc protein causes cancer is still unclear, but research …
In this study, the researchers used a method called mass spectrometry to study the proteins and metabolites inside FLC tumors and compared them to normal liver tissue. They found that the proteins in FLC tumors are different from those in healthy liver tissue, which could explain why FLC cells react differently to certain drugs and …
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 …