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FHCC
MCID: FBR086
MIFTS: 59

Fibrolamellar Carcinoma (FHCC)

Categories: Cancer diseases, Gastrointestinal diseases, Liver diseases, Rare diseases
Genes Variations Tissues Related diseases Publications Pathways Symptoms & Phenotypes Drugs
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Aliases & Classifications for Fibrolamellar Carcinoma

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MalaCards integrated aliases for Fibrolamellar Carcinoma:

Name: Fibrolamellar Carcinoma 12 20 15 17
Fibrolamellar Hepatocellular Carcinoma 12 20 58 44 70
Fibrolamellar Hepatocarcinoma 20 58
Fhcc 20 58
Eosinophilic Hepatocellular Carcinoma with Lamellar Fibrosis 20
Polygonal Cell Hepatocellular Carcinoma with Fibrous Stroma 20
Hepatocellular Carcinoma with Increased Stromal Fibrosis 20
Hepatocellular Carcinoma, Fibrolamellar 12
Eosinophilic Glassy Cell Hepatoma 20
Fibrolamellar Oncocytic Hepatoma 20
Oncocytic Hepatocellular Tumor 12
Fl-Hcc 20

Characteristics:

Orphanet epidemiological data:

58
fibrolamellar hepatocellular carcinoma
Inheritance: Not applicable; Prevalence: <1/1000000 (United States); Age of onset: Adolescent,Adult;

Classifications:

MalaCards categories:
Global: Rare diseases Cancer diseases
Anatomical: Liver diseases Gastrointestinal diseases
See all MalaCards categories (disease lists)
ICD10: 33
Orphanet: 58  
Rare hepatic diseases


External Ids:

Disease Ontology 12 DOID:5015
MeSH 44 C537258
NCIt 50 C4131
SNOMED-CT 67 15619004
ICD10 via Orphanet 33 C22.0
UMLS via Orphanet 71 C0334287
Orphanet 58 ORPHA401920
UMLS 70 C0334287
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Summaries for Fibrolamellar Carcinoma

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GARD : 20 Fibrolamellar carcinoma (FLC) is a rare form of liver cancer which is generally diagnosed in adolescents and young adults (before age 40). Many people with early FLC have no signs or symptoms of the condition. When present, symptoms are often nonspecific (i.e. abdominal pain, weight loss, malaise ) and blamed on other, more common conditions. The exact underlying cause of FLC is poorly understood. Unlike other forms of liver cancer, FLC typically occurs in the absence of underlying liver inflammation or scarring; thus, specific risk factors for this condition remain unidentified. FLC is typically treated with surgical resection.

MalaCards based summary : Fibrolamellar Carcinoma, also known as fibrolamellar hepatocellular carcinoma, is related to mixed fibrolamellar hepatocellular carcinoma and intrahepatic cholangiocarcinoma, and has symptoms including fever, abdominal pain and icterus. An important gene associated with Fibrolamellar Carcinoma is PRKACA (Protein Kinase CAMP-Activated Catalytic Subunit Alpha), and among its related pathways/superpathways are Actin Nucleation by ARP-WASP Complex and PI3K-Akt signaling pathway. The drugs nivolumab and Fluorouracil have been mentioned in the context of this disorder. Affiliated tissues include liver, lymph node and bone marrow, and related phenotypes are Decreased shRNA abundance (Z-score < -2) and Decreased shRNA abundance (Z-score < -2)

Disease Ontology : 12 A hepatocellular carcinoma characterized microscopically by laminated fibrous layers interspersed between the tumour cells. The polygonal, deeply eosinophilic tumor cells arise in non-cirrhotic livers.

Wikipedia : 73 Fibrolamellar hepatocellular carcinoma (FHCC) is a rare form of hepatocellular carcinoma (HCC) that... more...

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Related Diseases for Fibrolamellar Carcinoma

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Diseases related to Fibrolamellar Carcinoma via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 211)
# Related Disease Score Top Affiliating Genes
1 mixed fibrolamellar hepatocellular carcinoma 32.3 PRKACA KRT7 DNAJB1
2 intrahepatic cholangiocarcinoma 31.1 KRT7 FGFR2 AFP
3 cholangiocarcinoma 30.8 KRT8 KRT7 FGFR2 CCKBR AFP
4 pancreatoblastoma 30.5 SYP NTS AFP
5 hemangioma 30.4 VIM SYP KRT7 GAST
6 cholangitis, primary sclerosing 30.4 NR0B2 KRT7 AFP
7 hepatocellular clear cell carcinoma 30.4 FGFR2 FGFR1 AFP
8 hepatoblastoma 30.2 YAP1 KRT8 KRT7 AFP
9 ovary neuroendocrine neoplasm 10.5 SYP KRT7
10 mucinous adenofibroma 10.5 SYP KRT7
11 fgfr craniosynostosis syndromes 10.5 FGFR2 FGFR1
12 auditory system benign neoplasm 10.5 SYP KRT7
13 pancreatic serous cystadenocarcinoma 10.5 SYP KRT7
14 proliferating trichilemmal cyst 10.5 KRT8 KRT7
15 vaginal tubulovillous adenoma 10.5 SYP KRT7
16 gallbladder small cell carcinoma 10.5 SYP KRT7
17 ureter small cell carcinoma 10.5 SYP KRT7
18 endolymphatic sac tumor 10.5 KRT8 KRT7
19 middle ear adenoma 10.5 SYP KRT7
20 subareolar duct papillomatosis 10.5 KRT8 KRT7
21 nipple benign neoplasm 10.5 KRT8 KRT7
22 krukenberg carcinoma 10.5 SYP KRT7
23 urinary bladder small cell neuroendocrine carcinoma 10.5 SYP KRT7
24 benign breast adenomyoepithelioma 10.5 KRT8 KRT7
25 papillary squamous carcinoma 10.5 SYP KRT7
26 jejunal neoplasm 10.5 SYP KRT7
27 sensory organ benign neoplasm 10.4 SYP KRT7
28 synovium neoplasm 10.4 KRT8 KRT7
29 intrahepatic gall duct cancer 10.4 KRT8 KRT7
30 ovarian large-cell neuroendocrine carcinoma 10.4 SYP KRT7
31 appendix adenocarcinoma 10.4 SYP KRT7
32 rosette-forming glioneuronal tumor 10.4 SYP FGFR1
33 mucinous ovarian cystadenoma 10.4 KRT7 GAST
34 meibomian cyst 10.4 SYP KRT7
35 plagiocephaly 10.4 FGFR2 FGFR1
36 gastrointestinal neuroendocrine benign tumor 10.4 SYP GAST
37 ureteral benign neoplasm 10.4 SYP KRT7
38 wolffian duct adenocarcinoma 10.4 KRT7 FGFR2
39 mixed ductal-endocrine carcinoma 10.4 SYP GAST
40 squamous cell bile duct carcinoma 10.4 KRT8 KRT7
41 rete testis neoplasm 10.4 KRT7 AFP
42 rete testis adenocarcinoma 10.4 KRT7 AFP
43 primary hepatic neuroendocrine carcinoma 10.4 VIM SYP
44 syndromic craniosynostosis 10.4 FGFR2 FGFR1
45 glomangiomyoma 10.4 VIM SYP
46 papillary tumor of the pineal region 10.4 VIM SYP
47 spinal cord oligodendroglioma 10.4 SYP FGFR1
48 testicular spermatocytic seminoma 10.4 NR0B2 FGFR2
49 benign metastasizing leiomyoma 10.4 VIM KRT7
50 thymus sarcomatoid carcinoma 10.4 SYP AFP

Graphical network of the top 20 diseases related to Fibrolamellar Carcinoma:



Diseases related to Fibrolamellar Carcinoma
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Symptoms & Phenotypes for Fibrolamellar Carcinoma

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UMLS symptoms related to Fibrolamellar Carcinoma:


fever; abdominal pain; icterus; malaise

GenomeRNAi Phenotypes related to Fibrolamellar Carcinoma according to GeneCards Suite gene sharing:

26
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Decreased shRNA abundance (Z-score < -2) GR00366-A-114 9.28 AFP
2 Decreased shRNA abundance (Z-score < -2) GR00366-A-120 9.28 AFP
3 Decreased shRNA abundance (Z-score < -2) GR00366-A-29 9.28 AFP
4 Decreased shRNA abundance (Z-score < -2) GR00366-A-6 9.28 KRT8
5 Decreased shRNA abundance (Z-score < -2) GR00366-A-63 9.28 AFP
6 Decreased shRNA abundance (Z-score < -2) GR00366-A-65 9.28 AFP
7 Decreased shRNA abundance (Z-score < -2) GR00366-A-71 9.28 KRT7 KRT8
8 Decreased shRNA abundance (Z-score < -2) GR00366-A-75 9.28 AFP

MGI Mouse Phenotypes related to Fibrolamellar Carcinoma:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 homeostasis/metabolism MP:0005376 10.06 AFP CCKBR FGFR1 FGFR2 GAST ITGA1
2 digestive/alimentary MP:0005381 9.97 CCKBR FGFR1 FGFR2 GAST ITGAV ITGB4
3 endocrine/exocrine gland MP:0005379 9.91 AFP CCKBR FGFR1 FGFR2 GAST ITGAV
4 liver/biliary system MP:0005370 9.56 AFP CCKBR FGFR2 KRT8 NR0B2 PRKACA
5 renal/urinary system MP:0005367 9.28 CCKBR FGFR1 FGFR2 ITGA1 ITGAV ITGB4
Sources

Drugs & Therapeutics for Fibrolamellar Carcinoma

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Drugs for Fibrolamellar Carcinoma (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 24)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
nivolumab Approved Phase 1, Phase 2 946414-94-4
2
Fluorouracil Approved Phase 1, Phase 2 51-21-8 3385
3
Everolimus Approved Phase 2 159351-69-6 6442177 70789204
4
Letrozole Approved, Investigational Phase 2 112809-51-5 3902
5
Leuprolide Approved, Investigational Phase 2 53714-56-0 3911 657181
6
Sunitinib Approved, Investigational Phase 2 557795-19-4, 341031-54-7 5329102
7 Antineoplastic Agents, Immunological Phase 1, Phase 2
8 interferons Phase 1, Phase 2
9 Antiviral Agents Phase 1, Phase 2
10 Interferon alpha-2 Phase 1, Phase 2
11 Antimetabolites Phase 1, Phase 2
12 Interferon-alpha Phase 1, Phase 2
13 Estrogens Phase 2
14 Estrogen Receptor Antagonists Phase 2
15 Estrogen Antagonists Phase 2
16 Aromatase Inhibitors Phase 2
17 Angiogenesis Inhibitors Phase 2
18 Protein Kinase Inhibitors Phase 2
19
Ipilimumab Approved Phase 1 477202-00-9
20 Antibodies Phase 1
21 Antibodies, Blocking Phase 1
22 Vaccines Phase 1
23 Immunoglobulins Phase 1
24 Poly ICLC Phase 1

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 A Phase 2, Multi-center, Open-label Study of Oral ENMD-2076 for the Treatment of Patients With Advanced Fibrolamellar Carcinoma (FLC) Completed NCT02234986 Phase 2 ENMD-2076
2 A Phase I/II Study of Nivolumab Plus 5-Fluorouracil Plus Interferon-α2b for Unresectable Fibrolamellar Hepatocellular Carcinoma Recruiting NCT04380545 Phase 1, Phase 2 Fluorouracil
3 A Randomized Three Arm Phase II Study of (1) Everolimus, (2) Estrogen Deprivation Therapy (EDT) With Leuprolide + Letrozole and (3) Everolimus + EDT in Patients With Unresectable Fibrolamellar Hepatocellular Carcinoma (FLL-HCC) Active, not recruiting NCT01642186 Phase 2 everolimus;letrozole plus leuprolide;combination of everolimus, letrozole and leuprolide
4 A Multicenter National Phase II Open Study Coupled With Translational Assessment of Biomarkers Predictive of Response to Sunitinib in Patients With Advanced/Inoperable Fibrolamellar Hepatocellular Carcinoma. Terminated NCT01215565 Phase 2 Sutent
5 Sequential, Related Donor Partial Liver Transplantation Followed by Bone Marrow Transplantation for Fibrolamellar or Non-fibrolamellar Hepatocellular Carcinoma (HCC) Including Fibrolamellar HCC Withdrawn NCT02702960 Phase 2 Cyclophosphamide;Mesna;Filgrastim;Tacrolimus;mycophenolate mofetil;Prednisone;Antithymocyte globulin;fludarabine
6 Phase II Study of Oral Vancomycin in Patients With Unresectable Fibrolamellar Hepatocellular Carcinoma (FLC) Withdrawn NCT04025567 Phase 2 vancomycin
7 A Pilot Study of a DNAJB1-PRKACA Fusion Kinase Peptide Vaccine Combined With Nivolumab and Ipilimumab for Patients With Fibrolamellar Hepatocellular Carcinoma Recruiting NCT04248569 Phase 1 DNAJB1-PRKACA peptide vaccine;Nivolumab;Ipilimumab
8 A Natural History Study of Children and Adults With Fibrolamellar Hepatocellular Carcinoma Withdrawn NCT03748927

Search NIH Clinical Center for Fibrolamellar Carcinoma

Cochrane evidence based reviews: fibrolamellar hepatocellular carcinoma

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Genetic Tests for Fibrolamellar Carcinoma

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Anatomical Context for Fibrolamellar Carcinoma

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MalaCards organs/tissues related to Fibrolamellar Carcinoma:

40
Liver, Lymph Node, Bone Marrow, Bone, Pancreas, Ovary, Pancreatic Islet
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Publications for Fibrolamellar Carcinoma

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Articles related to Fibrolamellar Carcinoma:

(show top 50) (show all 485)
# Title Authors PMID Year
1
Fibrolamellar hepatocellular carcinoma treated with atezolizumab and bevacizumab: two case reports. 61
33722275 2021
2
Bilateral Diffuse Nodular Pulmonary Ossification Mimicking Metastatic Disease in a Patient with Fibrolamellar Hepatocellular Carcinoma. 61
33809687 2021
3
Early Experiences with Triple Immunochemotherapy in Adolescents and Young Adults with High-Risk Fibrolamellar Carcinoma. 61
33690232 2021
4
Defective internal allosteric network imparts dysfunctional ATP/substrate-binding cooperativity in oncogenic chimera of protein kinase A. 61
33692454 2021
5
In situ hybridisation for albumin RNA in paediatric liver cancers compared with common immunohistochemical markers. 61
32471888 2021
6
Fibrolamellar Carcinoma With Predominantly Pseudoglandular Architecture: A Potential Diagnostic Pitfall. 61
32508199 2021
7
New insights into the pathophysiology and clinical care of rare primary liver cancers. 61
33205035 2021
8
Immunohistochemical detection of procalcitonin in fibrolamellar hepatocellular carcinoma. 61
33566306 2021
9
Hyperammonemic Encephalopathy Mimicking Ornithine Transcarbamylase Deficiency in Fibrolamellar Hepatocellular Carcinoma: Successful Treatment with Continuous Venovenous Hemofiltration and Ammonia Scavengers. 61
32898940 2021
10
Fibrolamellar Hepatocellular Carcinoma: A Population-Based Observational Study. 61
32052215 2021
11
Liver Transplantation in a Patient With Hepatic Angiosarcoma. 61
33585099 2021
12
Imaging findings of fibrolamellar hepatocellular carcinomas on ultrasonography: A comparison with conventional hepatocellular carcinomas. 61
32924993 2021
13
Phase II Multicenter, Open-Label Study of Oral ENMD-2076 for the Treatment of Patients with Advanced Fibrolamellar Carcinoma. 61
32154962 2020
14
Hyperammonemic Encephalopathy Exacerbated by Parenteral Nutrition in Fibrolamellar Hepatocellular Carcinoma. 61
32739410 2020
15
Extrahepatic transarterial radioembolization to treat fibrolamellar hepatocellular carcinoma: A case report. 61
33088374 2020
16
[A Case of Fibrolamellar Hepatocellular Carcinoma in a 25-Year-Old Vietnamese Male]. 61
33468949 2020
17
Structural analyses of the PKA RIIβ holoenzyme containing the oncogenic DnaJB1-PKAc fusion protein reveal protomer asymmetry and fusion-induced allosteric perturbations in fibrolamellar hepatocellular carcinoma. 61
33370777 2020
18
Clinicopathological Features of Two Extremely Rare Hepatocellular Carcinoma Variants: a Brief Review of Fibrolamellar and Scirrhous Hepatocellular Carcinoma. 61
32860202 2020
19
Using dual checkpoint blockade to treat fibrolamellar hepatocellular carcinoma. 61
32051207 2020
20
A Multicenter Randomized Three-Arm Phase II Study of (1) Everolimus, (2) Estrogen Deprivation Therapy (EDT) with Leuprolide + Letrozole, and (3) Everolimus + EDT in Patients with Unresectable Fibrolamellar Carcinoma. 61
32400000 2020
21
Unusual Primary Neoplasms of the Adult Liver: Review of Imaging Appearances and Differential Diagnosis. 61
33199074 2020
22
Fibrolamellar Carcinoma: A Multimodal Approach. 61
33251292 2020
23
Fibrolamellar hepatocellular carcinoma with paraneoplastic neuropsychiatric manifestations. 61
31965677 2020
24
Molecular profiling and analysis of genetic aberrations aimed at identifying potential therapeutic targets in fibrolamellar carcinoma of the liver. 61
32663328 2020
25
Fibrolamellar carcinoma: Challenging the challenge. 61
32768872 2020
26
Fibrolamellar Carcinoma. 61
32498783 2020
27
Fibrolamellar Carcinoma: Novel Insights into a Rare Subtype. 61
32056041 2020
28
ASO Author Reflections: Fibrolamellar Hepatocellular Carcinoma and Alpha-Fetoprotein. 61
32189170 2020
29
Elevated Serum α-Fetoprotein is Associated with Abbreviated Survival for Patients with Fibrolamellar Hepatocellular Carcinoma Who Undergo a Curative Resection. 61
31925595 2020
30
Clinical features and surgical outcomes of fibrolamellar hepatocellular carcinoma: retrospective analysis of a single-center experience. 61
32397993 2020
31
BAP1 mutations define a homogeneous subgroup of hepatocellular carcinoma with fibrolamellar-like features and activated PKA. 61
31862487 2020
32
DNAJB1-PRKACA fusions occur in oncocytic pancreatic and biliary neoplasms and are not specific for fibrolamellar hepatocellular carcinoma. 61
31676785 2020
33
Fibrolamellar hepatocellular carcinoma-related hyperammonemic encephalopathy: Up to now and next steps. 61
31679315 2020
34
DnaJ-PKAc fusion induces liver inflammation in a zebrafish model of fibrolamellar carcinoma. 61
32102783 2020
35
Hotspots of Aberrant Enhancer Activity in Fibrolamellar Carcinoma Reveal Candidate Oncogenic Pathways and Therapeutic Vulnerabilities. 61
32294439 2020
36
Acute Onset Hyperammonemic Encephalopathy Related to Fibrolamellar Carcinoma: Another One Bites the Dust. 61
32014302 2020
37
Cytoreductive Surgery and HIPEC for Recurrent Fibrolamellar Hepatocellular Carcinoma with Peritoneal Carcinomatosis. 61
30989576 2020
38
Imaging Features of Fibrolamellar Hepatocellular Carcinoma with Contrast-Enhanced Ultrasound. 61
32102105 2020
39
Predictors of Outcome in Patients With Fibrolamellar Carcinoma: Analysis of the National Cancer Database. 61
32014928 2020
40
DNAJB1-PRKACA-positive metastatic fibrolamellar carcinoma with unknown primary in a pediatric patient. 61
31736218 2020
41
Single-Cell RNA Sequencing Identifies Yes-Associated Protein 1-Dependent Hepatic Mesothelial Progenitors in Fibrolamellar Carcinoma. 61
31669305 2020
42
Hyperammonemic encephalopathy: An unusual presentation of fibrolamellar hepatocellular carcinoma. 61
31422648 2020
43
Clavicle tumor as an initial manifestation of fibrolamellar hepatocellular carcinoma. 61
31677896 2020
44
MDM4 expression in fibrolamellar hepatocellular carcinoma. 61
31322272 2019
45
Emerging Roles of GLI-Similar Krüppel-like Zinc Finger Transcription Factors in Leukemia and Other Cancers. 61
31474360 2019
46
Inhibition of the chimeric DnaJ-PKAc enzyme by endogenous inhibitor proteins. 61
30938854 2019
47
Hepatocellular Carcinoma of Fibrolamellar Type in an Adolescent: Case Report and Literature Review. 61
31602376 2019
48
Neurotensin as a source of cyclic AMP and co-mitogen in fibrolamellar hepatocellular carcinoma. 61
31489118 2019
49
A prognostic nomogram for patients with resected fibrolamellar hepatocellular carcinoma. 61
31489303 2019
50
Hyperammonemic encephalopathy in a patient with fibrolamellar hepatocellular carcinoma: case report and literature review. 61
31183212 2019
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Variations for Fibrolamellar Carcinoma

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Cosmic variations for Fibrolamellar Carcinoma:

9 (show top 50) (show all 15431)
# Cosmic Mut ID Gene Symbol COSMIC Disease Classification
(Primary site, Site subtype, Primary histology, Histology subtype)		 Mutation CDS Mutation AA GRCh38 Location Conf
1 COSM97724722 ZXDB liver,NS,carcinoma,hepatocellular carcinoma c.1308C>A p.D436E 23:57593356-57593356 3
2 COSM94430614 ZSWIM1 liver,NS,carcinoma,hepatocellular carcinoma c.1399A>C p.S467R 20:45883991-45883991 3
3 COSM94436083 ZSWIM1 liver,NS,carcinoma,hepatocellular carcinoma c.1399A>C p.S467R 20:45883991-45883991 3
4 COSM94063771 ZSCAN20 liver,NS,carcinoma,hepatocellular carcinoma c.2314G>T p.G772W 1:33494658-33494658 3
5 COSM88264637 ZRSR2 liver,NS,carcinoma,hepatocellular carcinoma c.304G>T p.E102* 23:15803788-15803788 3
6 COSM102741209 ZNRF3 liver,NS,carcinoma,hepatocellular carcinoma c.859A>G p.M287V 22:29049340-29049340 3
7 COSM131494492 ZNRF3 liver,NS,carcinoma,hepatocellular carcinoma c.1159A>G p.M387V 22:29049340-29049340 3
8 COSM103081572 ZNRF3 liver,NS,carcinoma,hepatocellular carcinoma c.859A>G p.M287V 22:29049340-29049340 3
9 COSM95977701 ZNFX1 liver,NS,carcinoma,hepatocellular carcinoma c.2580G>A p.L860= 20:49257501-49257501 3
10 COSM102131817 ZNFX1 liver,NS,carcinoma,hepatocellular carcinoma c.2580G>A p.L860= 20:49257501-49257501 3
11 COSM95960776 ZNFX1 liver,NS,carcinoma,hepatocellular carcinoma c.2580G>A p.L860= 20:49257501-49257501 3
12 COSM113317148 ZNF853 liver,NS,carcinoma,hepatocellular carcinoma c.1610G>T p.G537V 7:6622601-6622601 3
13 COSM99592496 ZNF829 liver,NS,carcinoma,hepatocellular carcinoma c.829T>A p.F277I 19:36891962-36891962 3
14 COSM128686611 ZNF829 liver,NS,carcinoma,hepatocellular carcinoma c.1072T>A p.F358I 19:36891962-36891962 3
15 COSM123358576 ZNF827 liver,NS,carcinoma,hepatocellular carcinoma c.691C>T p.L231= 4:145885684-145885684 3
16 COSM123949383 ZNF827 liver,NS,carcinoma,hepatocellular carcinoma c.1741C>T p.L581= 4:145885684-145885684 3
17 COSM101741672 ZNF827 liver,NS,carcinoma,hepatocellular carcinoma c.1741C>T p.L581= 4:145885684-145885684 3
18 COSM86004822 ZNF653 liver,NS,carcinoma,hepatocellular carcinoma c.1553A>C p.H518P 19:11485673-11485673 3
19 COSM98561269 ZNF615 liver,NS,carcinoma,hepatocellular carcinoma c.141G>A p.V47= 19:52002156-52002156 3
20 COSM142353152 ZNF615 liver,NS,carcinoma,hepatocellular carcinoma c.141G>A p.V47= 19:52002156-52002156 3
21 COSM142221290 ZNF615 liver,NS,carcinoma,hepatocellular carcinoma c.141G>A p.V47= 19:52002156-52002156 3
22 COSM99854658 ZNF615 liver,NS,carcinoma,hepatocellular carcinoma c.156G>A p.V52= 19:52002156-52002156 3
23 COSM139729931 ZNF615 liver,NS,carcinoma,hepatocellular carcinoma c.141G>A p.V47= 19:52002156-52002156 3
24 COSM141470046 ZNF615 liver,NS,carcinoma,hepatocellular carcinoma c.141G>A p.V47= 19:52002156-52002156 3
25 COSM141819438 ZNF615 liver,NS,carcinoma,hepatocellular carcinoma c.141G>A p.V47= 19:52002156-52002156 3
26 COSM145038099 ZNF615 liver,NS,carcinoma,hepatocellular carcinoma c.141G>A p.V47= 19:52002156-52002156 3
27 COSM91868668 ZNF536 liver,NS,carcinoma,hepatocellular carcinoma c.3900G>T p.K1300N 19:30557161-30557161 3
28 COSM91880764 ZNF536 liver,NS,carcinoma,hepatocellular carcinoma c.2171-78T>G p.? 19:30534769-30534769 3
29 COSM140721836 ZNF521 liver,NS,carcinoma,hepatocellular carcinoma c.2998+205G>A p.? 18:25194955-25194955 3
30 COSM131506983 ZNF521 liver,NS,carcinoma,hepatocellular carcinoma c.3658+205G>A p.? 18:25194955-25194955 3
31 COSM94799980 ZNF521 liver,NS,carcinoma,hepatocellular carcinoma c.3658+205G>A p.? 18:25194955-25194955 3
32 COSM90582798 ZNF518B liver,NS,carcinoma,hepatocellular carcinoma c.1624G>T p.E542* 4:10444705-10444705 3
33 COSM87577038 ZNF462 liver,NS,carcinoma,hepatocellular carcinoma c.6715G>T p.V2239L 9:106974156-106974156 3
34 COSM109096265 ZNF462 liver,NS,carcinoma,hepatocellular carcinoma c.3433G>T p.V1145L 9:106974156-106974156 3
35 COSM104985080 ZNF385B liver,NS,carcinoma,hepatocellular carcinoma c.298+23785G>T p.? 2:179745718-179745718 3
36 COSM104427909 ZNF385B liver,NS,carcinoma,hepatocellular carcinoma c.20G>T p.S7I 2:179745718-179745718 3
37 COSM151538615 ZNF385B liver,NS,carcinoma,hepatocellular carcinoma c.253+23785G>T p.? 2:179745718-179745718 3
38 COSM127708351 ZNF345 liver,NS,carcinoma,hepatocellular carcinoma c.47-856A>T p.? 19:36891962-36891962 3
39 COSM135887725 ZNF276 liver,NS,carcinoma,hepatocellular carcinoma c.1168C>G p.L390V 16:89734008-89734008 3
40 COSM84511025 ZNF276 liver,NS,carcinoma,hepatocellular carcinoma c.1219C>G p.L407V 16:89734008-89734008 3
41 COSM111609487 ZNF276 liver,NS,carcinoma,hepatocellular carcinoma c.1444C>G p.L482V 16:89734008-89734008 3
42 COSM92353291 ZNF26 liver,NS,carcinoma,hepatocellular carcinoma c.1010C>T p.T337I 12:133010889-133010889 3
43 COSM130415812 ZNF26 liver,NS,carcinoma,hepatocellular carcinoma c.950C>T p.T317I 12:133010889-133010889 3
44 COSM88697750 ZNF217 liver,NS,carcinoma,hepatocellular carcinoma c.*194A>T p.? 20:53569094-53569094 3
45 COSM95253159 ZNF217 liver,NS,carcinoma,hepatocellular carcinoma c.*194A>T p.? 20:53569094-53569094 3
46 COSM94505616 ZMYND12 liver,NS,carcinoma,hepatocellular carcinoma c.425-1G>T p.? 1:42440026-42440026 3
47 COSM89943752 ZMYM4 liver,NS,carcinoma,hepatocellular carcinoma c.86-8201G>T p.? 1:35350724-35350724 3
48 COSM101398456 ZIC2 liver,NS,carcinoma,hepatocellular carcinoma c.-18C>T p.? 13:99982047-99982047 3
49 COSM113521587 ZGPAT liver,NS,carcinoma,hepatocellular carcinoma c.*197C>A p.? 20:63736116-63736116 3
50 COSM94679661 ZGPAT liver,NS,carcinoma,hepatocellular carcinoma c.*197C>A p.? 20:63736116-63736116 3
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Expression for Fibrolamellar Carcinoma

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Search GEO for disease gene expression data for Fibrolamellar Carcinoma.
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Pathways for Fibrolamellar Carcinoma

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Pathways related to Fibrolamellar Carcinoma according to GeneCards Suite gene sharing:

(show all 20)
# Super pathways Score Top Affiliating Genes
1
Actin Nucleation by ARP-WASP Complex 63
Show member pathways
 12.81 ITGB4 ITGAV ITGA1 FGFR2 FGFR1
2
PI3K-Akt signaling pathway 36
Show member pathways
 12.79 PRKACA ITGB4 ITGAV ITGA1 FGFR2 FGFR1
3
Beta-Adrenergic Signaling 63
Show member pathways
 12.64 PRKAR2B PRKACA ITGB4 ITGAV ITGA1
4
DREAM Repression and Dynorphin Expression 63
Show member pathways
 12.5 PRKAR2B PRKACA ITGB4 ITGAV ITGA1
5
NFAT and Cardiac Hypertrophy 63
Show member pathways
 12.44 PRKAR2B PRKACA ITGB4 ITGAV ITGA1 FGFR2
6
Regulation of actin cytoskeleton 1 36
12.42 ITGB4 ITGAV ITGA1 FGFR2 FGFR1
7
Calcium signaling pathway 36
12.24 PRKACA FGFR2 FGFR1 CCKBR
8
G-protein signaling RAC1 in cellular process 23
Show member pathways
 12.23 ITGB4 ITGAV ITGA1 FGFR2 FGFR1
9
Dilated cardiomyopathy 36
Show member pathways
 12.09 PRKACA ITGB4 ITGAV ITGA1
10
Actin Dynamics Signaling Pathway 66
12.05 PRKACA ITGB4 ITGAV ITGA1
11
Angiogenesis (CST) 4
11.72 ITGAV FGFR2 FGFR1
12
Phagocytosis of Microbes 63
11.69 ITGB4 ITGAV ITGA1
13
Neural Stem Cell Differentiation Pathways and Lineage-specific Markers 64
11.64 VIM SYP FGFR1
14
MAPK Signaling: Mitogen Stimulation Pathway 64
Show member pathways
 11.62 YAP1 ITGB4 ITGAV ITGA1 FGFR2 FGFR1
15
Primary Focal Segmental Glomerulosclerosis FSGS 1
11.43 VIM KRT8 ITGB4 ITGAV
16
Cell adhesion_Endothelial cell contacts by non-junctional mechanisms 23
11.18 ITGB4 ITGAV ITGA1
17
14-3-3 and Regulation of BAD Activity 63
11 YAP1 PRKAR2B PRKACA
18
G-protein signaling_RhoA regulation pathway 23
10.86 PRKAR2B PRKACA ITGB4 ITGAV ITGA1 FGFR2
19
G-protein signaling_Rap2B regulation pathway 23
10.75 VIM ITGB4 ITGAV ITGA1 FGFR2 FGFR1
20
Secretion of Hydrochloric Acid in Parietal Cells 1
10.13 GAST CCKBR
Sources

GO Terms for Fibrolamellar Carcinoma

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Cellular components related to Fibrolamellar Carcinoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular exosome GO:0070062 9.61 VIM PRKAR2B PRKACA KRT8 KRT7 ITGB4
2 cAMP-dependent protein kinase complex GO:0005952 9.16 PRKAR2B PRKACA
3 integrin complex GO:0008305 8.8 ITGB4 ITGAV ITGA1

Biological processes related to Fibrolamellar Carcinoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 positive regulation of cell proliferation GO:0008284 9.65 YAP1 ITGAV FGFR2 FGFR1 CCKBR
2 integrin-mediated signaling pathway GO:0007229 9.54 ITGB4 ITGAV ITGA1
3 cell-matrix adhesion GO:0007160 9.5 ITGB4 ITGAV ITGA1
4 mesodermal cell differentiation GO:0048333 9.16 ITGB4 FGFR2
5 positive regulation of phospholipase activity GO:0010518 8.96 FGFR2 FGFR1
6 bud elongation involved in lung branching GO:0060449 8.62 YAP1 FGFR2

Molecular functions related to Fibrolamellar Carcinoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 protein binding GO:0005515 10.23 YAP1 VIM SYP PRKAR2B PRKACA NTS
2 protein domain specific binding GO:0019904 9.62 VIM PRKAR2B PRKACA NR0B2
3 insulin-like growth factor I binding GO:0031994 9.37 ITGB4 ITGAV
4 neuregulin binding GO:0038132 9.16 ITGB4 ITGAV
5 fibroblast growth factor-activated receptor activity GO:0005007 8.96 FGFR2 FGFR1
6 fibroblast growth factor binding GO:0017134 8.8 ITGAV FGFR2 FGFR1
Sources

Sources for Fibrolamellar Carcinoma

About this section
3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Apr-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
The MalaCards human disease database index: 1-9 A B C D E F G H I J K L M N O P Q R S T U V W X Y Z
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