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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.
 
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

Alshareefy Y, Shen CY, Prekash RJ
Pathol Res Pract 2023 Aug;248:154655. Epub 2023 Jun 26 doi: 10.1016/j.prp.2023.154655. PMID: 37429175
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
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

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) Hepatocellular Carcinoma, 2023

Recent clinical studies

Etiology

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
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
Kassahun WT
World J Surg Oncol 2016 May 23;14(1):151. doi: 10.1186/s12957-016-0903-8. PMID: 27215576Free PMC Article
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
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

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
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
Lin CC, Yang HM
Arch Pathol Lab Med 2018 Sep;142(9):1141-1145. doi: 10.5858/arpa.2017-0083-RS. PMID: 30141990
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
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

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
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
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
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
Katzenstein HM, Krailo MD, Malogolowkin MH, Ortega JA, Qu W, Douglass EC, Feusner JH, Reynolds M, Quinn JJ, Newman K, Finegold MJ, Haas JE, Sensel MG, Castleberry RP, Bowman LC
Cancer 2003 Apr 15;97(8):2006-12. doi: 10.1002/cncr.11292. PMID: 12673731

Prognosis

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
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
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
Lin CC, Yang HM
Arch Pathol Lab Med 2018 Sep;142(9):1141-1145. doi: 10.5858/arpa.2017-0083-RS. PMID: 30141990
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

Clinical prediction guides

Alshareefy Y, Shen CY, Prekash RJ
Pathol Res Pract 2023 Aug;248:154655. Epub 2023 Jun 26 doi: 10.1016/j.prp.2023.154655. PMID: 37429175
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
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
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
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

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
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
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

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
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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, 2023
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) Hepatocellular Carcinoma, 2023

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