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Adult Fibrolamellar Carcinoma

MedGen UID:
859696
•Concept ID:
C3900102
•
Neoplastic Process

Definition

Fibrolamellar variant of hepatocellular carcinoma that occurs during adulthood. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAdult Fibrolamellar Carcinoma

Professional guidelines

PubMed

Fibrolamellar Hepatocellular Carcinoma: Comprehensive Review of Diagnosis, Imaging, and Management.
Aziz H, Brown ZJ, Panid Madani S, Kamel IR, Pawlik TM
J Am Coll Surg 2023 Feb 1;236(2):399-410. Epub 2022 Nov 4 doi: 10.1097/XCS.0000000000000476. PMID: 36648268
Unusual Primary Neoplasms of the Adult Liver: Review of Imaging Appearances and Differential Diagnosis.
Razik A, Malla S, Goyal A, Gamanagatti S, Kandasamy D, Das CJ, Sharma R, Gupta AK
Curr Probl Diagn Radiol 2022 Jan-Feb;51(1):73-85. Epub 2020 Nov 2 doi: 10.1067/j.cpradiol.2020.10.001. PMID: 33199074
Management of fibrolamellar hepatocellular carcinoma.
Chaudhari VA, Khobragade K, Bhandare M, Shrikhande SV
Chin Clin Oncol 2018 Oct;7(5):51. doi: 10.21037/cco.2018.08.08. PMID: 30395718

Recent clinical studies

Etiology

Treatment and Prognosis of Fibrolamellar Hepatocellular Carcinoma: a Systematic Review of the Recent Literature and Meta-analysis.
Glavas D, Bao QR, Scarpa M, Ruffolo C, Brown ZJ, Pawlik TM, Spolverato G
J Gastrointest Surg 2023 Apr;27(4):705-715. Epub 2023 Feb 16 doi: 10.1007/s11605-023-05621-z. PMID: 36797535Free PMC Article
Hepatoblastoma and Pediatric Hepatocellular Carcinoma: An Update.
Ranganathan S, Lopez-Terrada D, Alaggio R
Pediatr Dev Pathol 2020 Mar-Apr;23(2):79-95. Epub 2019 Sep 25 doi: 10.1177/1093526619875228. PMID: 31554479
State-of-the-art MR Imaging of Uncommon Hepatocellular Tumours: Fibrolamellar Hepatocellular Carcinoma and Combined Hepatocellularcholangiocarcinoma.
Anysz-Grodzicka A, Podgorska J, Cieszanowski A
Curr Med Imaging Rev 2019;15(3):269-280. doi: 10.2174/1573405614666180927113622. PMID: 31989878
Liver Cancer Cell of Origin, Molecular Class, and Effects on Patient Prognosis.
Sia D, Villanueva A, Friedman SL, Llovet JM
Gastroenterology 2017 Mar;152(4):745-761. Epub 2016 Dec 30 doi: 10.1053/j.gastro.2016.11.048. PMID: 28043904
Fibrolamellar hepatocellular carcinoma.
Friedman AC, Lichtenstein JE, Goodman Z, Fishman EK, Siegelman SS, Dachman AH
Radiology 1985 Dec;157(3):583-7. doi: 10.1148/radiology.157.3.2997835. PMID: 2997835

Diagnosis

Fighting rare cancers: lessons from fibrolamellar hepatocellular carcinoma.
Simon SM
Nat Rev Cancer 2023 May;23(5):335-346. Epub 2023 Mar 17 doi: 10.1038/s41568-023-00554-w. PMID: 36932129Free PMC Article
Fibrolamellar Hepatocellular Carcinoma: Comprehensive Review of Diagnosis, Imaging, and Management.
Aziz H, Brown ZJ, Panid Madani S, Kamel IR, Pawlik TM
J Am Coll Surg 2023 Feb 1;236(2):399-410. Epub 2022 Nov 4 doi: 10.1097/XCS.0000000000000476. PMID: 36648268
Hepatoblastoma and Pediatric Hepatocellular Carcinoma: An Update.
Ranganathan S, Lopez-Terrada D, Alaggio R
Pediatr Dev Pathol 2020 Mar-Apr;23(2):79-95. Epub 2019 Sep 25 doi: 10.1177/1093526619875228. PMID: 31554479
Update on the pathology of liver neoplasms.
Matsukuma KE, Yeh MM
Ann Diagn Pathol 2019 Feb;38:126-137. Epub 2018 Oct 12 doi: 10.1016/j.anndiagpath.2018.10.005. PMID: 30597357
Management of fibrolamellar hepatocellular carcinoma.
Chaudhari VA, Khobragade K, Bhandare M, Shrikhande SV
Chin Clin Oncol 2018 Oct;7(5):51. doi: 10.21037/cco.2018.08.08. PMID: 30395718

Therapy

Refractory Hyperammonemic encephalopathy in Fibrolamellar hepatocellular carcinoma, a case report and literature review.
Ahmed A, Ata F, Gaber M, Petkar M, Mahfouz A, Schirmacher P, Musa S, Hashim A
Curr Probl Cancer 2022 Jun;46(3):100847. Epub 2022 Mar 2 doi: 10.1016/j.currproblcancer.2022.100847. PMID: 35276469
A framework for fibrolamellar carcinoma research and clinical trials.
Dinh TA, Utria AF, Barry KC, Ma R, Abou-Alfa GK, Gordan JD, Jaffee EM, Scott JD, Zucman-Rossi J, O'Neill AF, Furth ME, Sethupathy P
Nat Rev Gastroenterol Hepatol 2022 May;19(5):328-342. Epub 2022 Feb 21 doi: 10.1038/s41575-022-00580-3. PMID: 35190728Free PMC Article
Clinical features and surgical outcomes of fibrolamellar hepatocellular carcinoma: retrospective analysis of a single-center experience.
Lemekhova A, Hornuss D, Polychronidis G, Mayer P, Rupp C, Longerich T, Weiss KH, Büchler M, Mehrabi A, Hoffmann K
World J Surg Oncol 2020 May 12;18(1):93. doi: 10.1186/s12957-020-01855-2. PMID: 32397993Free PMC Article
Clinical heterogeneity of pediatric hepatocellular carcinoma.
D'Souza AM, Towbin AJ, Gupta A, Alonso M, Nathan JD, Bondoc A, Tiao G, Geller JI
Pediatr Blood Cancer 2020 Jun;67(6):e28307. Epub 2020 Apr 19 doi: 10.1002/pbc.28307. PMID: 32307899
Fibrolamellar Carcinoma: Recent Advances and Unresolved Questions on the Molecular Mechanisms.
Lalazar G, Simon SM
Semin Liver Dis 2018 Feb;38(1):51-59. Epub 2018 Feb 22 doi: 10.1055/s-0037-1621710. PMID: 29471565Free PMC Article

Prognosis

Treatment and Prognosis of Fibrolamellar Hepatocellular Carcinoma: a Systematic Review of the Recent Literature and Meta-analysis.
Glavas D, Bao QR, Scarpa M, Ruffolo C, Brown ZJ, Pawlik TM, Spolverato G
J Gastrointest Surg 2023 Apr;27(4):705-715. Epub 2023 Feb 16 doi: 10.1007/s11605-023-05621-z. PMID: 36797535Free PMC Article
Hepatoblastoma and Pediatric Hepatocellular Carcinoma: An Update.
Ranganathan S, Lopez-Terrada D, Alaggio R
Pediatr Dev Pathol 2020 Mar-Apr;23(2):79-95. Epub 2019 Sep 25 doi: 10.1177/1093526619875228. PMID: 31554479
Management of fibrolamellar hepatocellular carcinoma.
Chaudhari VA, Khobragade K, Bhandare M, Shrikhande SV
Chin Clin Oncol 2018 Oct;7(5):51. doi: 10.21037/cco.2018.08.08. PMID: 30395718
Liver Cancer Cell of Origin, Molecular Class, and Effects on Patient Prognosis.
Sia D, Villanueva A, Friedman SL, Llovet JM
Gastroenterology 2017 Mar;152(4):745-761. Epub 2016 Dec 30 doi: 10.1053/j.gastro.2016.11.048. PMID: 28043904
Fibrolamellar hepatocellular carcinoma.
Friedman AC, Lichtenstein JE, Goodman Z, Fishman EK, Siegelman SS, Dachman AH
Radiology 1985 Dec;157(3):583-7. doi: 10.1148/radiology.157.3.2997835. PMID: 2997835

Clinical prediction guides

Clinical and demographic predictors of survival for fibrolamellar carcinoma patients-A patient community, registry-based study.
Berkovitz A, Migler RD, Qureshi A, Rosemore C, Torbenson MS, Vaughan R, Marcotte E, Simon SM
Hepatol Commun 2022 Dec;6(12):3539-3549. Epub 2022 Oct 17 doi: 10.1002/hep4.2105. PMID: 36245434Free PMC Article
Prognostic Role of Lymph Node Sampling in Adolescent and Young Adults With Fibrolamellar Carcinoma.
Schultz SP, Holtestaul T, Marenco CW, Bader JO, Horton JD, Nelson DW
J Surg Res 2022 Aug;276:261-271. Epub 2022 Apr 7 doi: 10.1016/j.jss.2022.03.006. PMID: 35398630
Fibrolamellar Hepatocellular Carcinoma: A Population-Based Observational Study.
Ramai D, Ofosu A, Lai JK, Gao ZH, Adler DG
Dig Dis Sci 2021 Jan;66(1):308-314. Epub 2020 Feb 12 doi: 10.1007/s10620-020-06135-3. PMID: 32052215
Management of fibrolamellar hepatocellular carcinoma.
Chaudhari VA, Khobragade K, Bhandare M, Shrikhande SV
Chin Clin Oncol 2018 Oct;7(5):51. doi: 10.21037/cco.2018.08.08. PMID: 30395718
mTORC1 and FGFR1 signaling in fibrolamellar hepatocellular carcinoma.
Riehle KJ, Yeh MM, Yu JJ, Kenerson HL, Harris WP, Park JO, Yeung RS
Mod Pathol 2015 Jan;28(1):103-10. Epub 2014 Jun 13 doi: 10.1038/modpathol.2014.78. PMID: 24925055

Recent systematic reviews

Treatment and Prognosis of Fibrolamellar Hepatocellular Carcinoma: a Systematic Review of the Recent Literature and Meta-analysis.
Glavas D, Bao QR, Scarpa M, Ruffolo C, Brown ZJ, Pawlik TM, Spolverato G
J Gastrointest Surg 2023 Apr;27(4):705-715. Epub 2023 Feb 16 doi: 10.1007/s11605-023-05621-z. PMID: 36797535Free PMC Article
Fibrolamellar Hepatocellular Carcinoma: Comprehensive Review of Diagnosis, Imaging, and Management.
Aziz H, Brown ZJ, Panid Madani S, Kamel IR, Pawlik TM
J Am Coll Surg 2023 Feb 1;236(2):399-410. Epub 2022 Nov 4 doi: 10.1097/XCS.0000000000000476. PMID: 36648268

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