Fibrolamellar hepatocellular carcinoma

A distinctive type of liver cell carcinoma that arises in non-cirrhotic livers and occurs predominantly in young patients. The tumor cells are polygonal and deeply eosinophilic, and are embedded in a fibrous stroma.

Also Known As:

Networked: 100 relevant articles (3 outcomes, 3 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Neoplasms (Cancer)
2.	Liver Neoplasms (Liver Cancer)
3.	Gynecomastia
4.	Cushing Syndrome
5.	Hepatocellular Carcinoma (Hepatoma)

Experts

1.	Simon, Sanford M: 7 articles (12/2020 - 01/2015)
2.	Taylor, Susan S: 3 articles (12/2020 - 01/2018)
3.	Kenerson, Heidi L: 3 articles (08/2019 - 01/2015)
4.	Riehle, Kimberly J: 3 articles (08/2019 - 01/2015)
5.	Yeung, Raymond S: 3 articles (08/2019 - 01/2015)
6.	Lalazar, Gadi: 3 articles (02/2018 - 11/2015)
7.	Lu, Tsan-Wen: 2 articles (12/2020 - 01/2019)
8.	Zhang, Ping: 2 articles (12/2020 - 01/2019)
9.	Hernandez, Jonathan M: 2 articles (06/2020 - 06/2020)
10.	McDonald, James D: 2 articles (06/2020 - 06/2020)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Fibrolamellar hepatocellular carcinoma:

1.	Aromatase (CYP19)IBA 05/01/1998 - "Because aromatase is not expressed in the disease-free adult liver, the presence of extremely high levels of aromatase expression in this fibrolamellar hepatocellular carcinoma tissue is intriguing, particularly because there is preferential use of the proximally located P450arom promoters I.3 and II by the tumor, instead of the much more distally located fetal liver-type promoter I.4." 05/01/1998 - "Molecular basis of severe gynecomastia associated with aromatase expression in a fibrolamellar hepatocellular carcinoma." 02/01/1997 - "A childhood fibrolamellar hepatocellular carcinoma with increased aromatase activity and a near triploid karyotype." 07/01/2011 - "High aromatase activity and overexpression of epidermal growth factor receptor in fibrolamellar hepatocellular carcinoma in a child." 05/01/1998 - "This report represents the first study in the literature linking development of severe gynecomastia, in a 17 1/2-yr-old boy, to high levels of aromatase expression in a large fibrolamellar hepatocellular carcinoma, which gave rise to extremely elevated serum levels of estrone (1200 pg/mL) and estradiol-17 beta (312 pg/mL) that suppressed FSH and LH (1.3 and 2.8 IU/L, respectively), and consequently testosterone (1.53 ng/mL). "
2.	temsirolimusFDA Link 09/01/2023 - "Therapeutic Efficacy of Temsirolimus in a Patient-derived Model of Metastatic Fibrolamellar Hepatocellular Carcinoma."
3.	Tight Junction ProteinsIBA 06/01/2011 - "In the present study, tight junction protein expressions were studied to possibly clarify bipotential lineage of fibrolamellar hepatocellular carcinoma. "
4.	Testosterone (Sustanon)FDA Link 05/01/1998 - "This report represents the first study in the literature linking development of severe gynecomastia, in a 17 1/2-yr-old boy, to high levels of aromatase expression in a large fibrolamellar hepatocellular carcinoma, which gave rise to extremely elevated serum levels of estrone (1200 pg/mL) and estradiol-17 beta (312 pg/mL) that suppressed FSH and LH (1.3 and 2.8 IU/L, respectively), and consequently testosterone (1.53 ng/mL). "
5.	Hydrocortisone (Cortisol)FDA LinkGeneric 02/03/2015 - "Recent genomic studies have identified recurrent mutations in the catalytic subunit of PKA in tumors associated with Cushing's syndrome, a kidney disorder leading to excessive cortisol production, and also in tumors associated with fibrolamellar hepatocellular carcinoma (FL-HCC), a rare liver cancer. "
6.	EstroneFDA Link 05/01/1998 - "This report represents the first study in the literature linking development of severe gynecomastia, in a 17 1/2-yr-old boy, to high levels of aromatase expression in a large fibrolamellar hepatocellular carcinoma, which gave rise to extremely elevated serum levels of estrone (1200 pg/mL) and estradiol-17 beta (312 pg/mL) that suppressed FSH and LH (1.3 and 2.8 IU/L, respectively), and consequently testosterone (1.53 ng/mL). "
7.	Estradiol (Delestrogen)FDA LinkGeneric 05/01/1998 - "This report represents the first study in the literature linking development of severe gynecomastia, in a 17 1/2-yr-old boy, to high levels of aromatase expression in a large fibrolamellar hepatocellular carcinoma, which gave rise to extremely elevated serum levels of estrone (1200 pg/mL) and estradiol-17 beta (312 pg/mL) that suppressed FSH and LH (1.3 and 2.8 IU/L, respectively), and consequently testosterone (1.53 ng/mL). "
8.	A 17 (A-17)IBA 05/01/1998 - "This report represents the first study in the literature linking development of severe gynecomastia, in a 17 1/2-yr-old boy, to high levels of aromatase expression in a large fibrolamellar hepatocellular carcinoma, which gave rise to extremely elevated serum levels of estrone (1200 pg/mL) and estradiol-17 beta (312 pg/mL) that suppressed FSH and LH (1.3 and 2.8 IU/L, respectively), and consequently testosterone (1.53 ng/mL). "
9.	Cyclic AMP-Dependent Protein Kinases (cAMP-Dependent Protein Kinase)IBA 01/01/2016 - "Enhanced cAMP-stimulated protein kinase A activity in human fibrolamellar hepatocellular carcinoma." 01/01/2022 - "Novel protein kinase cAMP-Activated Catalytic Subunit Alpha (PRKACA) inhibitor shows anti-tumor activity in a fibrolamellar hepatocellular carcinoma model." 01/01/2019 - "Fibrolamellar hepatocellular carcinoma (FLHCC) is driven by J-PKAcα, a kinase fusion chimera of the J domain of DnaJB1 with PKAcα, the catalytic subunit of protein kinase A (PKA). " 01/01/2018 - "In fibrolamellar hepatocellular carcinoma a single genetic deletion results in the fusion of the first exon of the heat shock protein 40, DNAJB1, which encodes the J domain, with exons 2-10 of the catalytic subunit of protein kinase A, PRKACA. " 11/21/2023 - "Fibrolamellar hepatocellular carcinoma (FLC) is a rare liver cancer caused by a dominant recurrent fusion of the heat shock protein (DNAJB1) and the catalytic subunit of protein kinase A (PRKACA). "
10.	Phosphotransferases (Kinase)IBA 01/01/2018 - "Conformational Landscape of the PRKACA-DNAJB1 Chimeric Kinase, the Driver for Fibrolamellar Hepatocellular Carcinoma." 01/01/2023 - "Oncogenic Addiction of Fibrolamellar Hepatocellular Carcinoma to the Fusion Kinase DNAJB1-PRKACA." 10/27/2023 - "The DNAJB1-PRKACA oncogenic gene fusion results in an active kinase enzyme, J-PKAcα, that has been identified as an attractive antitumor target for fibrolamellar hepatocellular carcinoma (FLHCC). " 01/01/2023 - "Disruption of proteome by an oncogenic fusion kinase alters metabolism in fibrolamellar hepatocellular carcinoma." 01/01/2019 - "Fibrolamellar hepatocellular carcinoma (FLHCC) is driven by J-PKAcα, a kinase fusion chimera of the J domain of DnaJB1 with PKAcα, the catalytic subunit of protein kinase A (PKA). "

Therapies and Procedures

1.	Therapeutics 10/27/2022 - "The DNAJB1-PRKACA fusion transcript is the oncogenic driver in fibrolamellar hepatocellular carcinoma, a lethal disease lacking specific therapies. " 06/01/2009 - "Fibrolamellar hepatocellular carcinoma: prolonged survival with multimodality therapy." 01/01/2022 - "Fibrolamellar hepatocellular carcinoma (FLC) is a rare and often lethal liver cancer with no proven effective systemic therapy. " 12/15/2018 - "Fibrolamellar Hepatocellular Carcinoma with Multiple Lung Metastases Treated with Multidisciplinary Therapy." 12/01/2018 - "Fibrolamellar hepatocellular carcinoma: multiphasic CT features of the primary tumor on pre-therapy CT and pattern of distant metastases."
2.	Liver Transplantation 08/01/2019 - "The fibrolamellar hepatocellular carcinoma invaded more than 80% of the hepatic parenchyma without surgical possibility or liver transplantation. " 04/09/2018 - "Benefits from liver transplant for patients with fibrolamellar hepatocellular carcinoma have not yet been reported. " 03/01/2017 - "Liver transplantation for fibrolamellar hepatocellular carcinoma: A national perspective." 08/01/2016 - "Successful Liver Transplantation for Hyperammonemic Fibrolamellar Hepatocellular Carcinoma." 12/01/2008 - ""Preemptive" live donor liver transplantation for fibrolamellar hepatocellular carcinoma: a case report."
3.	Drug Therapy (Chemotherapy) 10/01/2022 - "CT and liver MRI showed large right hepatic lobe lesion then TRU-CUT needle biopsy was performed which showed Fibrolamellar hepatocellular carcinoma and patient underwent surgical resection with no postoperative complication followed by multiple cycle of chemotherapy and no signs of relapse with 3 year follow up. " 01/01/2018 - "Curative lung metastectomy and complete pathological response after neo-adjuvant GEMOX chemotherapy for relapse fibrolamellar hepatocellular carcinoma." 07/01/2014 - "Fibrolamellar variant of hepatocellular carcinoma (FLHCC) does not have a favorable prognosis than conventional HCC, and there is no difference regarding the response to chemotherapy and the degree of surgical resectability. " 01/01/2012 - "Prolonged Complete Response after GEMOX Chemotherapy in a Patient with Advanced Fibrolamellar Hepatocellular Carcinoma." 04/01/2001 - "[Multiorgan resection in combination with intraoperative, hyperthermic chemotherapy in recurrent fibrolamellar hepatocellular carcinoma. "
4.	Hepatectomy 05/01/2009 - "We report a case of fibrolamellar hepatocellular carcinoma with a radiological-pathological correlation in a 37 year-old man with chronic viral hepatitis B without cirrhosis who underwent right hepatectomy." 05/01/2008 - "She underwent a right hepatectomy for possible malignant liver tumors, including intrahepatic cholangiocarcinoma or fibrolamellar hepatocellular carcinoma. " 06/01/2023 - "Atezolizumab and Bevacizumab Combination Therapy and Sequential Conversion Hepatectomy for Advanced Fibrolamellar Hepatocellular Carcinoma Presenting Pseudoprogression."
5.	Neoadjuvant Therapy 06/27/2014 - "Downstaging and resection after neoadjuvant therapy for fibrolamellar hepatocellular carcinoma." 08/01/2006 - "Cytological features were those of fibrolamellar hepatocellular carcinoma, confirmed by histological examination of the biopsy sample as well as the surgical specimen obtained after wide excision of the lesion following ineffective neoadjuvant chemotherapy."