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 …

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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 …

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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 …

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