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Fibrolamellar hepatocellular carcinoma

MedGen UID:
83122
•Concept ID:
C0334287
•
Neoplastic Process
Synonyms: Fibrolamellar variant of hepatocellular carcinoma; Hepatocellular carcinoma (fibrolamellar variant)
SNOMED CT: Hepatocellular carcinoma, fibrolamellar (15619004); Fibrolamellar hepatocellular carcinoma (253018005)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0006210
Orphanet: ORPHA401920

Definition

A rare variant of hepatocellular carcinoma (HCC) presenting in adolescents or young adults with no underlying liver disease. Clinical presentation is non specific, with abdominal mass, abdominal discomfort or pain, fatigue and weight loss. Patients can also be asymptomatic. HCC markers (alpha fetoprotein) are normal. Fibrolamellar HCC presents as a unique, well-delimited mass at imagery and a biopsy confirms the diagnosis, showing well-differentiated tumor cells surrounded by thick collagen bands. [from ORDO]

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
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
Contemporary management of fibrolamellar hepatocellular carcinoma: diagnosis, treatment, outcome, prognostic factors, and recent developments.
Kassahun WT
World J Surg Oncol 2016 May 23;14(1):151. doi: 10.1186/s12957-016-0903-8. PMID: 27215576Free PMC Article

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) Hepatocellular Carcinoma, 2024

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
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
Contemporary management of fibrolamellar hepatocellular carcinoma: diagnosis, treatment, outcome, prognostic factors, and recent developments.
Kassahun WT
World J Surg Oncol 2016 May 23;14(1):151. doi: 10.1186/s12957-016-0903-8. PMID: 27215576Free PMC Article
Liver transplantation for malignancies.
Eghtesad B, Aucejo F
J Gastrointest Cancer 2014 Sep;45(3):353-62. doi: 10.1007/s12029-014-9590-2. PMID: 24604263
Fibrolamellar hepatocellular carcinoma.
Liu S, Chan KW, Wang B, Qiao L
Am J Gastroenterol 2009 Oct;104(10):2617-24; quiz 2625. Epub 2009 Jul 28 doi: 10.1038/ajg.2009.440. PMID: 19638962

Diagnosis

High-Level Procalcitonin in Patient with Mixed Fibrolamellar Hepatocellular Carcinoma: A Case Report and Literature Review.
Akbulut S, Tuncer A, Ogut Z, Sahin TT, Koc C
J Gastrointest Cancer 2022 Dec;53(4):1130-1134. Epub 2021 Nov 5 doi: 10.1007/s12029-021-00731-w. PMID: 34738189
Hepatocellular carcinoma in children.
Kelly D, Sharif K, Brown RM, Morland B
Clin Liver Dis 2015 May;19(2):433-47. Epub 2015 Feb 28 doi: 10.1016/j.cld.2015.01.010. PMID: 25921672
Fibrolamellar hepatocellular carcinoma.
Koudah S, El Mouhadi S, Arrivé L
Clin Res Hepatol Gastroenterol 2012 Feb;36(1):5-6. Epub 2011 Nov 8 doi: 10.1016/j.clinre.2011.09.008. PMID: 22070972
Hepatobiliary pathology.
Taylor SL, Haque S
Curr Opin Gastroenterol 2011 May;27(3):248-55. doi: 10.1097/MOG.0b013e3283457d43. PMID: 21423007
Fibrolamellar hepatocellular carcinoma.
Liu S, Chan KW, Wang B, Qiao L
Am J Gastroenterol 2009 Oct;104(10):2617-24; quiz 2625. Epub 2009 Jul 28 doi: 10.1038/ajg.2009.440. PMID: 19638962

Therapy

Immune checkpoint inhibitors in hepatocellular carcinoma: emerging challenges in clinical practice.
Pinter M, Scheiner B, Pinato DJ
Lancet Gastroenterol Hepatol 2023 Aug;8(8):760-770. Epub 2023 Jun 14 doi: 10.1016/S2468-1253(23)00147-4. PMID: 37327807
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
Integrated Genomic Analysis Identifies Driver Genes and Cisplatin-Resistant Progenitor Phenotype in Pediatric Liver Cancer.
Hirsch TZ, Pilet J, Morcrette G, Roehrig A, Monteiro BJE, Molina L, Bayard Q, Trépo E, Meunier L, Caruso S, Renault V, Deleuze JF, Fresneau B, Chardot C, Gonzales E, Jacquemin E, Guerin F, Fabre M, Aerts I, Taque S, Laithier V, Branchereau S, Guettier C, Brugières L, Rebouissou S, Letouzé E, Zucman-Rossi J
Cancer Discov 2021 Oct;11(10):2524-2543. Epub 2021 Apr 23 doi: 10.1158/2159-8290.CD-20-1809. PMID: 33893148Free PMC Article
Fibrolamellar Hepatocellular Carcinoma: Treatment with Yttrium-90 and Subsequent Surgical Resection.
Mafeld S, French J, Tiniakos D, Haugk B, Manas D, Littler P
Cardiovasc Intervent Radiol 2018 May;41(5):816-820. Epub 2018 Feb 21 doi: 10.1007/s00270-018-1903-6. PMID: 29468286Free PMC Article
Hepatocellular carcinoma in children.
Kelly D, Sharif K, Brown RM, Morland B
Clin Liver Dis 2015 May;19(2):433-47. Epub 2015 Feb 28 doi: 10.1016/j.cld.2015.01.010. PMID: 25921672

Prognosis

High-Level Procalcitonin in Patient with Mixed Fibrolamellar Hepatocellular Carcinoma: A Case Report and Literature Review.
Akbulut S, Tuncer A, Ogut Z, Sahin TT, Koc C
J Gastrointest Cancer 2022 Dec;53(4):1130-1134. Epub 2021 Nov 5 doi: 10.1007/s12029-021-00731-w. PMID: 34738189
Integrated Genomic Analysis Identifies Driver Genes and Cisplatin-Resistant Progenitor Phenotype in Pediatric Liver Cancer.
Hirsch TZ, Pilet J, Morcrette G, Roehrig A, Monteiro BJE, Molina L, Bayard Q, Trépo E, Meunier L, Caruso S, Renault V, Deleuze JF, Fresneau B, Chardot C, Gonzales E, Jacquemin E, Guerin F, Fabre M, Aerts I, Taque S, Laithier V, Branchereau S, Guettier C, Brugières L, Rebouissou S, Letouzé E, Zucman-Rossi J
Cancer Discov 2021 Oct;11(10):2524-2543. Epub 2021 Apr 23 doi: 10.1158/2159-8290.CD-20-1809. PMID: 33893148Free PMC Article
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
Fibrolamellar Carcinoma: What Is New and Why It Matters.
Graham RP
Surg Pathol Clin 2018 Jun;11(2):377-387. Epub 2018 Mar 21 doi: 10.1016/j.path.2018.02.006. PMID: 29751881
Fibrolamellar hepatocellular carcinoma.
Koudah S, El Mouhadi S, Arrivé L
Clin Res Hepatol Gastroenterol 2012 Feb;36(1):5-6. Epub 2011 Nov 8 doi: 10.1016/j.clinre.2011.09.008. PMID: 22070972

Clinical prediction guides

Increased Protein Kinase A Activity Induces Fibrolamellar Hepatocellular Carcinoma Features Independent of DNAJB1.
Shirani M, Levin S, Shebl B, Requena D, Finkelstein TM, Johnson DS, Ng D, Lalazar G, Heissel S, Hojrup P, Molina H, de Jong YP, Rice CM, Singhi AD, Torbenson MS, Coffino P, Lyons B, Simon SM
Cancer Res 2024 Aug 15;84(16):2626-2644. doi: 10.1158/0008-5472.CAN-23-4110. PMID: 38888469Free PMC Article
GalNAc-conjugated siRNA targeting the DNAJB1-PRKACA fusion junction in fibrolamellar hepatocellular carcinoma.
Neumayer C, Ng D, Requena D, Jiang CS, Qureshi A, Vaughan R, Prakash TP, Revenko A, Simon SM
Mol Ther 2024 Jan 3;32(1):140-151. Epub 2023 Nov 21 doi: 10.1016/j.ymthe.2023.11.012. PMID: 37980543Free PMC Article
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
Contemporary management of fibrolamellar hepatocellular carcinoma: diagnosis, treatment, outcome, prognostic factors, and recent developments.
Kassahun WT
World J Surg Oncol 2016 May 23;14(1):151. doi: 10.1186/s12957-016-0903-8. PMID: 27215576Free PMC Article

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
A systematic review: treatment and prognosis of patients with fibrolamellar hepatocellular carcinoma.
Mavros MN, Mayo SC, Hyder O, Pawlik TM
J Am Coll Surg 2012 Dec;215(6):820-30. Epub 2012 Sep 13 doi: 10.1016/j.jamcollsurg.2012.08.001. PMID: 22981432

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    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Curated

    • NCCN, 2024
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) Hepatocellular Carcinoma, 2024

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    Reviews

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