---
title: "Fibrolamellar hepatocellular carcinoma: clinicopathological and molecular analysis of nine cases"
id: "pubmed-42706111"
canonical_url: "https://medichelpline.com/clinical-feed/pubmed-42706111"
content_type: "clinical_feed_article"
specialty: "Oncology"
source_name: "PubMed / NCBI"
source_url: "https://pubmed.ncbi.nlm.nih.gov/42706111/"
doi: "10.3760/cma.j.cn112151-20260112-00027"
published_at: "2026-09-08T00:00:00.000Z"
evidence_level: "English Abstract"
license: "CC-BY-NC-4.0 / Informational Use"
---
# Fibrolamellar hepatocellular carcinoma: clinicopathological and molecular analysis of nine cases
## Provenance & Clinical Metadata
- **Canonical URL:** https://medichelpline.com/clinical-feed/pubmed-42706111
- **Specialty:** [Oncology](https://medichelpline.com/clinical-feed/oncology.md)
- **Primary Source:** PubMed / NCBI
- **Source URL:** [Original Journal Publication](https://pubmed.ncbi.nlm.nih.gov/42706111/)
- **DOI:** [10.3760/cma.j.cn112151-20260112-00027](https://doi.org/10.3760%2Fcma.j.cn112151-20260112-00027)
- **Published At:** 2026-09-08T00:00:00.000Z
- **Evidence Rating:** English Abstract
## Executive GIST (TL;DR)
- This single-center case series analyzed nine cases of **fibrolamellar hepatocellular carcinoma (FL-HCC)** diagnosed from January 2018 to December 2024 at Fudan University Shanghai Cancer Center. - Patients: 3 males and 6 females; median age reported as 18.0 years (interquartile range 10.5–26.0). - Clinical findings: serum AFP was slightly elevated in 1 of 9 patients; 2 patients had active hepatitis B virus infection. Tumor location: 5 in left hepatic lobe, 3 in right lobe, 1 with bilateral multifocal lesions. Tumor sizes ranged from 4.0 to 21.4 cm. - Histology: tumors composed of large polygonal neoplastic cells with abundant granular eosinophilic cytoplasm, large vesicular nuclei and prominent nucleoli; cells arranged in sheets or nests separated by dense collagen bundles forming cord-like or parallel lamellae consistent with laminated intratumoral fibrous stroma. - Immunophenotype: all tumors expressed **CK7**, **HepPar-1**, and **Arg-1** by immunohistochemistry. - Molecular genetics: RNA-based next-generation sequencing (NGS) identified the **DNAJB1::PRKACA** gene fusion in 6 cases; the remaining 3 showed **PRKACA** gene rearrangement detected by break-apart fluorescence in situ hybridization (FISH). - Follow-up and outcomes: follow-up duration ranged 7–80 months. One patient lost to follow-up; among eight evaluable patients, 3 were alive without recurrence, 1 recurred with lung metastasis, and 4 died of disease. - Conclusion: FL-HCC is a rare HCC variant defined by laminated fibrous stroma on histology and a characteristic **DNAJB1::PRKACA** fusion or PRKACA rearrangement on molecular testing. - Study methods: retrospective case collection with HE, immunohistochemistry, FISH and RNA-based NGS; literature review for prognostic context.
## Clinical Analysis & Structured Key Points
Objective: To investigate the clinicopathological and molecular features of fibrolamellar hepatocellular carcinoma (FL-HCC). Methods: The clinicopathological and prognostic information of 9 FL-HCC cases diagnosed at the Fudan University Shanghai Cancer Center, Shanghai, China from January 2018 to December 2024 were collected and analyzed. The FL-HCC samples were examined with immunohistochemical staining, fluorescence in situ hybridization (FISH), and RNA-based next-generation sequencing (NGS). The related literature was also reviewed. Results: There were 3 males and 6 females. The patients' age was 18.0 (10.5, 26.0) years. One of the 9 patients had slightly elevated serum AFP level, and 2 patients had current infection of hepatitis B virus. Five cases occurred in the left lobe of the liver, 3 cases in the right lobe of the liver, and 1 case had multiple lesions involving both the left and right lobes. The tumor sizes ranged from 4.0 to 21.4 cm. Microscopically, neoplastic cells were mainly arranged in sheets or nests, separated by dense collagen bundles frequently arranged in cord-like or parallel lamellae. The tumor cells were large and polygonal, with abundant granular and eosinophilic cytoplasm, large vesicular nuclei, and prominent nucleoli. All tumor cells expressed CK7, HepPar-1, and Arg-1. Molecular analysis revealed that 6 cases harbored the DNAJB1::PRKACA gene fusion by NGS, while the other 3 cases showed PRKACA gene rearrangement by FISH using a break-apart probe. The follow-up period ranged from 7 to 80 months. One patient was lost to follow-up after diagnosis, 3 patients survived without recurrence, 1 patient relapsed with lung metastasis, and 4 patients died of the disease. Conclusion: FL-HCC is a rare type of hepatocellular carcinoma characterized by laminated intratumoral fibrous stroma and specific fusion (DNAJB1::PRKACA) or rearrangement of the PRKACA gene. &#x76ee;&#x7684;&#xff1a; &#x63a2;&#x8ba8;&#x7ea4;&#x7ef4;&#x677f;&#x5c42;&#x578b;&#x809d;&#x7ec6;&#x80de;&#x764c;&#xff08;FL-HCC&#xff09;&#x7684;&#x4e34;&#x5e8a;&#x75c5;&#x7406;&#x53ca;&#x5206;&#x5b50;&#x9057;&#x4f20;&#x5b66;&#x7279;&#x5f81;&#x3002; &#x65b9;&#x6cd5;&#xff1a; &#x6536;&#x96c6;&#x590d;&#x65e6;&#x5927;&#x5b66;&#x9644;&#x5c5e;&#x80bf;&#x7624;&#x533b;&#x9662;2018&#x5e74;1&#x6708;&#x81f3;2024&#x5e74;12&#x6708;&#x8bca;&#x65ad;&#x7684;9&#x4f8b;FL-HCC&#x7684;&#x4e34;&#x5e8a;&#x8d44;&#x6599;&#x3001;&#x75c5;&#x7406;&#x4fe1;&#x606f;&#x53ca;&#x9884;&#x540e;&#x6570;&#x636e;&#xff0c;&#x91c7;&#x7528;HE&#x67d3;&#x8272;&#x3001;&#x514d;&#x75ab;&#x7ec4;&#x7ec7;&#x5316;&#x5b66;&#x67d3;&#x8272;&#x3001;&#x8367;&#x5149;&#x539f;&#x4f4d;&#x6742;&#x4ea4;&#x548c;&#x9ad8;&#x901a;&#x91cf;&#x6d4b;&#x5e8f;&#x8fdb;&#x884c;&#x68c0;&#x6d4b;&#xff0c;&#x7ed3;&#x5408;&#x6587;&#x732e;&#x5bf9;&#x9884;&#x540e;&#x8fdb;&#x884c;&#x5206;&#x6790;&#x3002; &#x7ed3;&#x679c;&#xff1a; 9&#x4f8b;&#x60a3;&#x8005;&#x4e2d;&#x7537;&#x6027;3&#x4f8b;&#x3001;&#x5973;&#x6027;6&#x4f8b;&#xff0c;&#x5e74;&#x9f84;18.0&#xff08;10.5&#xff0c;26.0&#xff09;&#x5c81;&#xff0c;&#x5176;&#x4e2d;&#x4ec5;1&#x4f8b;&#x8840;&#x6e05;&#x7532;&#x80ce;&#x86cb;&#x767d;&#x7565;&#x5347;&#x9ad8;&#xff0c;2&#x4f8b;&#x4f34;&#x6709;&#x4e59;&#x809d;&#x75c5;&#x6bd2;&#x611f;&#x67d3;&#x3002;5&#x4f8b;&#x53d1;&#x751f;&#x4e8e;&#x809d;&#x5de6;&#x53f6;&#xff0c;3&#x4f8b;&#x4e3a;&#x809d;&#x53f3;&#x53f6;&#xff0c;1&#x4f8b;&#x4e3a;&#x809d;&#x5de6;&#x53f6;&#x53ca;&#x809d;&#x53f3;&#x53f6;&#x591a;&#x53d1;&#x5360;&#x4f4d;&#xff0c;&#x80bf;&#x7624;&#x6700;&#x5927;&#x5f84;4.0~21.4 cm&#x3002;&#x955c;&#x4e0b;&#x5f62;&#x6001;&#x8868;&#x73b0;&#x4e3a;&#x4e30;&#x5bcc;&#x7684;&#x6761;&#x7d22;&#x72b6;&#x3001;&#x5c42;&#x72b6;&#x7ea4;&#x7ef4;&#x5c06;&#x80bf;&#x7624;&#x7ec6;&#x80de;&#x5206;&#x9694;&#x4e3a;&#x56e2;&#x5757;&#x72b6;&#x3001;&#x5c0f;&#x5de2;&#x72b6;&#xff0c;&#x7624;&#x7ec6;&#x80de;&#x8f83;&#x5927;&#xff0c;&#x5f62;&#x6001;&#x4e0d;&#x89c4;&#x5219;&#xff0c;&#x8868;&#x73b0;&#x4e3a;&#x591a;&#x8fb9;&#x5f62;&#xff0c;&#x80de;&#x8d28;&#x4e30;&#x5bcc;&#xff0c;&#x55dc;&#x9178;&#x6027;&#xff0c;&#x7ec6;&#x80de;&#x6838;&#x5927;&#x7a7a;&#x6ce1;&#x72b6;&#xff0c;&#x53ef;&#x89c1;&#x660e;&#x663e;&#x7684;&#x6838;&#x4ec1;&#x3002;&#x514d;&#x75ab;&#x7ec4;&#x7ec7;&#x5316;&#x5b66;&#x67d3;&#x8272;&#x663e;&#x793a;&#x6240;&#x6709;&#x80bf;&#x7624;&#x7ec6;&#x80de;&#x5747;&#x8868;&#x8fbe;CK7&#x3001;HepPar-1&#x3001;Arg-1&#x3002;6&#x4f8b;&#x884c;&#x9ad8;&#x901a;&#x91cf;&#x6d4b;&#x5e8f;&#xff0c;&#x68c0;&#x6d4b;&#x7ed3;&#x679c;&#x663e;&#x793a;&#x5747;&#x5b58;&#x5728;DNAJB1&#xff1a;&#xff1a;PRKACA&#x57fa;&#x56e0;&#x878d;&#x5408;&#x6539;&#x53d8;&#xff0c;3&#x4f8b;&#x884c;&#x8367;&#x5149;&#x539f;&#x4f4d;&#x6742;&#x4ea4;&#x65ad;&#x88c2;-&#x5206;&#x79bb;&#x63a2;&#x9488;&#x68c0;&#x6d4b;&#xff0c;&#x7ed3;&#x679c;&#x663e;&#x793a;PRKACA&#x57fa;&#x56e0;&#x5206;&#x79bb;&#x3002;&#x672c;&#x7ec4;&#x75c5;&#x4f8b;&#x968f;&#x8bbf;&#x65f6;&#x95f4;7~80&#x4e2a;&#x6708;&#xff0c;&#x5176;&#x4e2d;1&#x4f8b;&#x786e;&#x8bca;&#x540e;&#x5373;&#x5931;&#x8bbf;&#xff0c;&#x5728;&#x53ef;&#x968f;&#x8bbf;&#x7684;8&#x4f8b;&#x4e2d;&#xff0c;3&#x4f8b;&#x65e0;&#x75c5;&#x751f;&#x5b58;&#xff0c;1&#x4f8b;&#x590d;&#x53d1;&#x4f34;&#x80ba;&#x8f6c;&#x79fb;&#xff0c;4&#x4f8b;&#x6b7b;&#x4ea1;&#x3002; &#x7ed3;&#x8bba;&#xff1a; FL-HCC&#x662f;&#x4e00;&#x7c7b;&#x5c11;&#x89c1;&#x7684;&#x809d;&#x7ec6;&#x80de;&#x764c;&#xff0c;&#x5177;&#x6709;&#x7279;&#x5f81;&#x6027;&#x7684;&#x677f;&#x5c42;&#x72b6;&#x7ea4;&#x7ef4;&#x95f4;&#x8d28;&#x548c;&#x7279;&#x5f02;&#x6027;&#x7684;DNAJB1&#xff1a;&#xff1a;PRKACA&#x57fa;&#x56e0;&#x878d;&#x5408;&#x3002;.
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