Selected research highlights
The following journal publications, posters and presentations/discussions about fibrolamellar carcinoma and related topics have been recommended by our scientific advisors. Each of the listed scientific articles also includes a brief “plain English” overview that summarizes the results and implications of the published effort.
To view and search a complete listing of the nearly 600 articles published on fibrolamellar carcinoma since 1956, click here.
DNAJB1-PRKACA fusion drives fibrolamellar liver cancer through impaired SIK signaling and CRTC2/p300-mediated transcriptional reprogrammingSeptember 27, 2024 │ Gritti, Wan, … & Gujral and Bardeesy
Proteo-metabolomics and patient tumor slice experiments point to amino acid centrality for rewired mitochondria in fibrolamellar carcinomaAugust 28, 2024 │ Long, Chan, Han, Kamdar, ... , Vacanti, Gujral & Sethupathy
DNAJB1-PRKACA fusion protein-regulated LINC00473 promotes tumor growth and alters mitochondrial fitness in fibrolamellar carcinomaMarch 21, 2024 │ Ma, Tsai, Farghli, ..., Broom, Barrow & Sethupathy
DNAJB1-PRKACA fusion neoantigens elicit rare endogenous T cell responses that potentiate cell therapy for fibrolamellar carcinomaMarch 19, 2024 │ Kirk, Crawford, Chou, Guy, ..., Strome & Thomas
Reversing immunosuppression in the tumor microenvironment of fibrolamellar carcinoma via PD‑1 and IL‑10 blockadeMarch 1, 2024 │ Daniel, Sullivan, Dickerson, ... , Riehle, Yeung, Carter, Barry & Pillarisetty
NCI intramural program approach to rare tumors: Natural history study of rare solid tumors in children and adults: A longitudinal, comprehensive data and biospecimen collection protocolJune 21, 2023 │ Wedekind, Reilly, Del Rivero, Lockridge, Allen, Raygada, Bernstein, Thomas, Vivelo, ... , Sandler & Widemann
Disruption of proteome by an oncogenic fusion kinase alters metabolism in fibrolamellar hepatocellular carcinomaJune 21, 2023 │ Levin, Tomasini, Knox, Shirani, ... & Simon
Organoid models of fibrolamellar carcinoma mutations reveal hepatocyte transdifferentiation through cooperative BAP1 and PRKAR2A lossMay 3, 2023 │ Rüland, Andreatta, Massalini, Chuva de Sousa Lopes, Clevers, Hendriks & Artegiani
Defining incidence and complications of fibrolamellar liver cancer through tiered computational analysis of clinical dataMarch 23, 2023 │ Zack, Losert, Maisel, Wild, ... & Gordan
Oncogenic Addiction of Fibrolamellar Hepatocellular Carcinoma to the Fusion Kinase DNAJB1-PRKACAJanuary 4, 2023 │ Neumayer, Ng, Jiang, Qureshi, ... & Simon
Fibrolamellar carcinoma transcriptomic-based treatment prediction: complete response after nivolumab and ipilimumabDecember 9, 2022 │ Berger, Dinstag, Tirosh, Schiff, Kleiner, Aldape, Ruppin, Beker, Kurzrock
Clinical Outcomes in Fibrolamellar Hepatocellular Carcinoma Treated with Immune Checkpoint InhibitorsOctober 30, 2022 │ Chen, Popovic, Hsiehchen, Baretti, ... & Yarchoan
The oncogenic fusion protein DNAJB1-PRKACA can be specifically targeted by peptide-based immunotherapy in fibrolamellar hepatocellular carcinomaOctober 27, 2022 │ Bauer, Kohler, Maringer, Bucher, Bilich, Zwich, Dicks, Nelde, ... & Walz
Clinical and demographic predictors of survival for fibrolamellar carcinoma patients—A patient community, registry‐based studyOctober 17, 2022 │ Berkovitz, Migler, Qureshi, Rosemore,Torbenson, Vaughan, Marcotte & Simon
β-catenin cancer–enhancing genomic regions axis is involved in the development of fibrolamellar hepatocellular carcinomaSeptember 8, 2022 │ Gulati, Johnston, Rivas, Cast, ... , Shin & Timchenko
Targeting BCL-XL in fibrolamellar hepatocellular carcinomaSeptember 8, 2022 │ Sheb, Ng, Lalazar, Rosemore, Finkelstein, ... & Simon
Histologic type predicts disparate outcomes in pediatric hepatocellular neoplasms: A Pediatric Surgical Oncology Research Collaborative studyMay 13, 2022 │ Short, Kastenberg, Wei, Bondoc, Dasgupta, Tiao, Watters, Heaton, Lotakis, La Quaglia, ... , Utria, Meyers & Riehle
Multi-omic analysis of microRNA-mediated regulation reveals a proliferative axis involving miR-10b in fibrolamellar carcinomaApril 28, 2022 │ Francisco, … , Bardeesy & Sethupathy
A framework for fibrolamellar carcinoma research and clinical trialsFebruary 22, 2022 │ Dinh, Utria, Barry, Ma, Abou- Alfa, Gordan, Jaffee, Scott, Zucman- Rossi, O’Neill, Furth & Sethupathy
DNAJB1-PRKACA in HEK293T cells induces LINC00473 overexpression that depends on PKA signalingFebruary 15, 2022 │ Kim, Kycia, Karski, Ma, Bordt, Kwan, ..., Sethupathy, & Vakili
Identification of Novel Therapeutic Targets for Fibrolamellar Carcinoma Using Patient Derived Xenografts and Direct from Patient ScreeningOctober 1, 2021 │ Lalazar, Requena, ... & Simon
Fibrolamellar carcinoma: An entity all its ownJuly 18, 2021 │ O'Neill, Church, Perez-Atayde, ... & Vakili
Using dual checkpoint blockade to treat fibrolamellar hepatocellular carcinomaFebruary 12, 2020 │ De Toni & Roessler
Predictors of Outcome in Patients With Fibrolamellar Carcinoma: Analysis of the National Cancer DatabaseFebruary 5, 2020 │ Assi, Mukherjee, Machiorlatti, Vesely, Pareek & Hatoum
An acquired scaffolding function of the DNAJ-PKAc fusion contributes to oncogenic signaling in fibrolamellar carcinomaMay 7, 2019 │ Turnham, Smith, Kenerson, Omar, Golkowski, Garcia, Bauer, Lau, Sullivan, ..., Yeung & Scott
MicroRNA-375 Suppresses the Growth and Invasion of Fibrolamellar CarcinomaFebruary 11, 2019 │ Dinh, Jewell, ... & Sethupathy
Road map for fibrolamellar carcinoma: progress and goals of a diversified approachJanuary 31, 2019 │ Kastenhuber, Craig, ... & Abou-Alfa
CRISPR/Cas9 Engineering of Adult Mouse Liver Demonstrates that the DNAJB1-PRKACA Gene Fusion Is Sufficient to Induce Tumors Resembling Fibrolamellar Hepatocellular CarcinomaDecember 1, 2017 │ Engelholm, Riaz, ... & Frödin
Molecular testing for the clinical diagnosis of fibrolamellar carcinomaSeptember 1, 2017 │ Graham, Yeh, Lam-Himlin, Roberts, Terracciano, Cruise, Greipp, ... , & Torbenson
Model of fibrolamellar hepatocellular carcinomas reveals striking enrichment in cancer stem cellsOctober 6, 2015 │ Oikawa, Wauthier, Dinh, Selitsky, Reyna-Neyra, ... , Sethupathy & Reid
The genomic landscape of fibrolamellar hepatocellular carcinoma: whole genome sequencing of ten patientsDecember 1, 2014 │ Darcy, Chiaroni-Clarke, ... & Simon
Detection of a recurrent DNAJB1-PRKACA chimeric transcript in fibrolamellar hepatocellular carcinomaFebruary 28, 2014 │ Honeyman, Simon, ... & Simon
A pilot study of a DNAJB1-PRKACA fusion kinase peptide vaccine combined with nivolumab and ipilimumab for patients with fibrolamellar hepatocellular carcinomaNovember 1, 2023 │ Baretti, Ladle, Nauroth, Wang, Durham, Thoburn, Ferguson, Thompson, ... , Jaffee & Yarchoan
Gemcitabine-Oxaliplatin-Lenvatinib (GEMOX-LEN) For Unresectable Fibrolamellar Carcinoma: Promising Results in First 16 PatientsMay 1, 2021 │ Reid Davison, Paul Kent
Seeking a Cure: The Promise of ImmunotherapyJune 24, 2021 │ Elizabeth Jaffee, Paul Thomas & Mark Furth
