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journal publication
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 …
Fibrolamellar carcinoma (FLC) is a rare, aggressive liver cancer affecting adolescents and young adults. Nearly all cases contain the same genetic abnormality — a fusion protein called DNAJ‑PKAc. This study provides clear evidence that the fusion protein fundamentally reshapes both the metabolism of the cancer and immune behavior. Importantly, this rewiring reveals a specific therapeutic …
This study sequenced the DNA from FLC tumors and compared the results to the DNA from non-cancerous tissue from the same patients to identify the specific mutations that had occurred in the cancer. In the 10 samples examined, the only consistent mutation was the DNAJB1-PRKACA fusion gene. Since the only consistent mutation found was the …
This study looked for associations between demographic, clinical factors and overall survival using data from the Fibrolamellar Registry. Several key findings emerged: In conclusion, while this study provides insights into factors associated with survival in FLC patients, further research is needed to better understand this rare cancer. The full text of the publication can be …
This is the first published case report of a near-complete clinical remission of fibrolamellar carcinoma (FLC) in a patient treated with immunotherapy. A 22-year-old woman had advanced disease with widespread metastases. Chemotherapy did not halt the progression of the cancer. Drs. Enrico De Toni and Daniel Roessler (University Hospital, Munich, Germany) elected to try a …
Historically, fibrolamellar carcinoma (FLC) has been diagnosed by analyzing the histology (microscopic anatomy) and morphology (shape, size and structure) of the tumor tissue. Frequently, that examination is combined with immunostaining for cytokeratin 7 and CD68. That immunostaining uses antibodies to see if proteins typically found in FLC are present. However, accurate diagnosis of fibrolamellar carcinoma …
This paper discusses a case study of a patient in her mid 30s whose FLC had recurred and persisted despite resection and multiple lines of treatment, including use of an immune checkpoint inhibitor (atezolizumab – an anti-PD-L1 agent). Following molecular profiling of her tumor and computational analysis of the tumor’s transcriptome (RNA), a combination of …
Fibrolamellar carcinoma (FLC) is driven by the fusion protein DNAJ-PKAc. That protein is made up of two different parts, one that helps with protein folding (DNAJB1) and another (protein kinase A) that’s typically involved in cell signaling. In this study, researchers have found that DNAJ-PKAc acts like a scaffold, bringing together other proteins that contribute …
The second Fibrolamellar Cancer Foundation Scientific Summit was held in Stamford, CT, USA, in November 2017. The goals of this meeting were to gather a diverse set of investigators, stimulate new collaborations, and devise new ways to inspire additional interest in the field. This meeting summary outlines the work supported by the Foundation and presented …