Molecular testing for the clinical diagnosis of fibrolamellar carcinoma

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 …

Read more

An acquired scaffolding function of the DNAJ-PKAc fusion contributes to oncogenic signaling in fibrolamellar carcinoma

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 …

Read more

Using dual checkpoint blockade to treat fibrolamellar hepatocellular carcinoma

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 …

Read more

Predictors of Outcome in Patients With Fibrolamellar Carcinoma: Analysis of the National Cancer Database

This publication retrospectively analyzes the clinicopathological factors and treatments affecting overall survival in FLC. Using the National Cancer Database, it identified 496 patients diagnosed with FLC between 2004 and 2015. Of those patients, resection was performed on 254 (51.2%), liver-directed therapy on 13 (2.6%), and liver transplantation on 15 (3.0%) patients. Median overall survival times …

Read more

β-catenin cancer–enhancing genomic regions axis is involved in the development of fibrolamellar hepatocellular carcinoma

The purpose of this study was to examine the role of human genomic regions, called cancer-enhancing genomic regions or aggressive liver cancer domains (CEGRs/ALCDs), in the development of FLC. In previous investigations of the mutation-independent pathways of pediatric liver cancer hepatoblastoma, researchers identified CEGRs/ALCDs that control the expression of a group of cancer-causing genes. In …

Read more

Targeting BCL-XL in fibrolamellar hepatocellular carcinoma

Fibrolamellar carcinoma (FLC) is a cancer that doesn’t respond well to current treatments. In past research, the Rockefeller University team conducted a broad-based, small-molecule drug screen to identify potential drug candidates to use against FLC. That effort identified several potential drugs including napabucasin, panobinostat, irinotecan (a topoisomerase I inhibitor), and navitoclax (an inhibitor of the …

Read more

Fibrolamellar carcinoma: An entity all its own

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 …

Read more