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FHCC
MCID: FBR086
MIFTS: 60

Fibrolamellar Carcinoma (FHCC)

Categories: Cancer diseases, Gastrointestinal diseases, Liver diseases, Rare diseases
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Aliases & Classifications for Fibrolamellar Carcinoma

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MalaCards integrated aliases for Fibrolamellar Carcinoma:

Name: Fibrolamellar Carcinoma 11 19 14 16
Fibrolamellar Hepatocellular Carcinoma 11 19 58 43 71 75
Fibrolamellar Hepatocarcinoma 19 58
Fhcc 19 58
Eosinophilic Hepatocellular Carcinoma with Lamellar Fibrosis 19
Polygonal Cell Hepatocellular Carcinoma with Fibrous Stroma 19
Hepatocellular Carcinoma with Increased Stromal Fibrosis 19
Hepatocellular Carcinoma, Fibrolamellar 11
Eosinophilic Glassy Cell Hepatoma 19
Fibrolamellar Oncocytic Hepatoma 19
Oncocytic Hepatocellular Tumor 11
Fl-Hcc 19

Characteristics:


Prevelance:

Fibrolamellar Hepatocellular Carcinoma: <1/1000000 (United States, Austria, Belgium, Bulgaria, Czech Republic, Estonia, Finland, France, Germany, Iceland, Ireland, Latvia, Norway, Poland, Portugal, Slovakia, Spain, Switzerland, Netherlands, United Kingdom, Europe) 1-9/1000000 (Italy) 58

Age Of Onset:

Fibrolamellar Hepatocellular Carcinoma: Adolescent,Adult 58

Classifications:

MalaCards categories:
Global: Rare diseases Cancer diseases
Anatomical: Liver diseases Gastrointestinal diseases
See all MalaCards categories (disease lists)
ICD10: 32
Orphanet: 58  
Rare hepatic diseases


External Ids:

Disease Ontology 11 DOID:5015
MeSH 43 C537258
NCIt 49 C4131
SNOMED-CT 68 15619004
ICD10 via Orphanet 32 C22.0
UMLS via Orphanet 72 C0334287
Orphanet 58 ORPHA401920
UMLS 71 C0334287
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Summaries for Fibrolamellar Carcinoma

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Orphanet: 58 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.

MalaCards based summary: Fibrolamellar Carcinoma, also known as fibrolamellar hepatocellular carcinoma, is related to mixed fibrolamellar hepatocellular carcinoma and hepatocellular adenoma, 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 Disease and Signal Transduction. The drugs Fluorouracil and Cisplatin have been mentioned in the context of this disorder. Affiliated tissues include liver, bone marrow and lymph node, and related phenotypes are Increased cell migration and Decreased viability after gemcitabine stimulation

GARD: 19 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.

Disease Ontology: 11 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: 75 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 273)
# Related Disease Score Top Affiliating Genes
1 mixed fibrolamellar hepatocellular carcinoma 32.3 GOLGA6L2 DNAJB1
2 hepatocellular adenoma 30.9 GPC3 GLUL CTNNB1 AFP
3 cholangiocarcinoma 30.7 TP53 KRT7 KRT19 GPC3 CTNNB1 AFP
4 krukenberg carcinoma 30.7 KRT7 AFP
5 wilms tumor 1 30.5 TP53 LINC00473 KRT7 GPC3 CTNNB1 AFP
6 cholangitis, primary sclerosing 30.5 TP53 KRT7 KRT19 AFP
7 gallbladder disease 30.5 TP53 KRT7 CTNNB1 AFP
8 sclerosing hepatic carcinoma 30.4 KRT19 GPC3 AFP
9 peliosis hepatis 30.4 GLUL AFP
10 adult hepatocellular carcinoma 30.3 TP53 GPC3 GLUL CTNNB1 AFP
11 mixed cell type cancer 30.3 TP53 KRT7 CTNNB1 AFP
12 intrahepatic cholangiocarcinoma 30.3 TP53 KRT7 KRT19 GPC3 CTNNB1 AFP
13 adenoma 30.1 TP53 PRKAR1A KRT7 KRT19 CTNNB1
14 carney complex variant 29.9 TP53 PRKX PRKAR2B PRKAR1A PRKACB PRKACA
15 hepatoblastoma 29.9 TP53 KRT7 KRT19 GPC3 GLUL CTNNB1
16 hepatocellular carcinoma 10.6
17 rete testis neoplasm 10.4 KRT7 AFP
18 choriocarcinoma of the testis 10.4 KRT7 AFP
19 rete testis adenocarcinoma 10.4 KRT7 AFP
20 mixed hepatoblastoma 10.4 GPC3 AFP
21 rectum neuroendocrine neoplasm 10.4 KRT7 AFP
22 epididymal neoplasm 10.4 KRT7 AFP
23 breast hemangiopericytoma 10.4 KRT7 KRT19
24 ovarian embryonal carcinoma 10.4 GPC3 AFP
25 adenosquamous gallbladder carcinoma 10.4 KRT7 KRT19
26 adenosquamous bile duct carcinoma 10.4 KRT7 KRT19
27 mucinous ovarian cystadenoma 10.4 KRT7 AFP
28 colonic lymphangioma 10.4 KRT7 AFP
29 pancreatic serous cystic neoplasm 10.4 KRT7 KRT19
30 ornithine transcarbamylase deficiency, hyperammonemia due to 10.4
31 bile duct cystadenocarcinoma 10.4 KRT7 AFP
32 cervical clear cell adenocarcinoma 10.4 KRT7 KRT19
33 lipoma of spermatic cord 10.4 KRT19 AFP
34 vulvar apocrine adenocarcinoma 10.4 KRT7 KRT19
35 acth-independent cushing syndrome 10.4 PRKAR1A PRKACA
36 biliary papillomatosis 10.4 KRT7 KRT19
37 paratesticular lipoma 10.4 KRT19 AFP
38 testicular malignant germ cell cancer 10.4 GPC3 AFP
39 pancreatic foamy gland adenocarcinoma 10.4 TP53 KRT7
40 malignant spiradenoma 10.4 TP53 KRT7
41 vulvar sebaceous carcinoma 10.4 TP53 KRT7
42 liver lipoma 10.4 CTNNB1 AFP
43 adult teratoma 10.4 TP53 AFP
44 ovarian endodermal sinus tumor 10.4 KRT7 GPC3 AFP
45 vulvar keratinizing squamous cell carcinoma 10.4 TP53 KRT19
46 endodermal sinus tumor 10.4 KRT7 GPC3 AFP
47 endometrial squamous cell carcinoma 10.4 TP53 KRT7
48 renal pelvis adenocarcinoma 10.4 KRT7 KRT19 AFP
49 ovarian primitive germ cell tumor 10.4 KRT7 GPC3 AFP
50 necrotizing sialometaplasia 10.4 TP53 KRT7

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:

25
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Increased cell migration GR00055-A-1 9.26 CTNNB1 PRKACA
2 Increased cell migration GR00055-A-3 9.26 CTNNB1 PRKACA
3 Decreased viability after gemcitabine stimulation GR00107-A-2 8.8 PRKACB PRKAR1A PRKAR2B

MGI Mouse Phenotypes related to Fibrolamellar Carcinoma:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 liver/biliary system MP:0005370 9.56 AFP CTNNB1 GLUL KRT19 PRKACA PRKAR1A
2 homeostasis/metabolism MP:0005376 9.47 AFP AGR2 ATP1B1 CTNNB1 GLUL GPC3
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Drugs & Therapeutics for Fibrolamellar Carcinoma

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

(show top 50) (show all 54)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Fluorouracil Approved Phase 2, Phase 3 51-21-8 3385
2
Cisplatin Approved Phase 2, Phase 3 15663-27-1 2767 5702198 441203
3
Carboplatin Approved Phase 2, Phase 3 41575-94-4 10339178 38904
4
Etoposide Approved Phase 2, Phase 3 33419-42-0 36462
5
Irinotecan Approved, Investigational Phase 2, Phase 3 97682-44-5, 100286-90-6 60838
6
Vincristine Approved, Investigational Phase 2, Phase 3 2068-78-2, 57-22-7 5978
7
Picropodophyllin Approved, Investigational Phase 2, Phase 3 518-28-5, 477-47-4 10607 72435
8
Sorafenib Approved, Investigational Phase 2, Phase 3 284461-73-0 216239
9
Gemcitabine Approved Phase 2, Phase 3 95058-81-4, 122111-03-9 60750
10
Daunorubicin Approved Phase 2, Phase 3 20830-81-3 30323
11
Doxorubicin Approved, Investigational Phase 2, Phase 3 23214-92-8 31703
12
Oxaliplatin Approved, Investigational Phase 2, Phase 3 61825-94-3 43805 11947679 6857599
13 Immunologic Factors Phase 2, Phase 3
14 Antiviral Agents Phase 2, Phase 3
15 Anti-Infective Agents Phase 2, Phase 3
16 Antimetabolites Phase 2, Phase 3
17 Immunosuppressive Agents Phase 2, Phase 3
18 Anti-Bacterial Agents Phase 2, Phase 3
19 Liver Extracts Phase 2, Phase 3
20 Antibiotics, Antitubercular Phase 2, Phase 3
21 Protein Kinase Inhibitors Phase 2, Phase 3
22
Etoposide phosphate Phase 2, Phase 3 16760419
23 Antimitotic Agents Phase 2, Phase 3
24 topoisomerase I inhibitors Phase 2, Phase 3
25 Keratolytic Agents Phase 2, Phase 3
26 Tubulin Modulators Phase 2, Phase 3
27 Liposomal doxorubicin Phase 2, Phase 3
28 Dermatologic Agents Phase 2, Phase 3
29
Everolimus Approved Phase 2 159351-69-6 70789204 6442177
30
Leuprolide Approved, Investigational Phase 2 53714-56-0 657181
31
Letrozole Approved, Investigational Phase 2 112809-51-5 3902
32
Nivolumab Approved Phase 1, Phase 2 946414-94-4
33
Pembrolizumab Approved Phase 2 1374853-91-4 254741536
34
Sunitinib Approved, Investigational Phase 2 557795-19-4 5329102
35 Estrogens Phase 2
36 Estrogen Receptor Antagonists Phase 2
37 Estrogen Antagonists Phase 2
38 Aromatase Inhibitors Phase 2
39 Immune Checkpoint Inhibitors Phase 1, Phase 2
40 interferons Phase 1, Phase 2
41 Antineoplastic Agents, Immunological Phase 1, Phase 2
42 Interferon alpha-2 Phase 1, Phase 2
43 Interferon-alpha Phase 1, Phase 2
44 Angiogenesis Inhibitors Phase 2
45
Ipilimumab Approved Phase 1 477202-00-9
46
Aflibercept Approved Phase 1 862111-32-8 124490314
47 Vaccines Phase 1
48 Immunoglobulins Phase 1
49 Poly ICLC Phase 1
50 Antibodies, Blocking Phase 1

Interventional clinical trials:

(show all 14)
# Name Status NCT ID Phase Drugs
1 Pediatric Hepatic Malignancy International Therapeutic Trial (PHITT) Recruiting NCT03533582 Phase 2, Phase 3 Carboplatin;Cisplatin;Doxorubicin;Etoposide;Fluorouracil;Gemcitabine;Irinotecan;Oxaliplatin;Sorafenib;Vincristine Sulfate
2 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) Completed NCT01642186 Phase 2 everolimus;letrozole plus leuprolide;combination of everolimus, letrozole and leuprolide
3 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
4 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
5 A Multi-center, Open-label, Phase 1/2a Trial to Evaluate the Safety, Tolerability, Pharmacokinetics, and Preliminary Efficacy of KAT-101 in Subjects With HCC Recruiting NCT05603572 Phase 1, Phase 2 KAT-101;KAT-201
6 The Role of Checkpoint Inhibition in Relapsed/Refractory Pediatric Hepatocellular Carcinoma: Clinical Efficacy and Biologic Correlates - A Phase II Study Recruiting NCT04134559 Phase 2 Pembrolizumab
7 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
8 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
9 Phase II Study of Oral Vancomycin in Patients With Unresectable Fibrolamellar Hepatocellular Carcinoma (FLC) Withdrawn NCT04025567 Phase 2 vancomycin
10 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
11 A Phase 1 Study of AGEN1181, an Fc-Engineered Anti-CTLA-4 Monoclonal Antibody as Monotherapy and in Combination With AGEN2034 (Balstilimab), an Anti-PD-1 Monoclonal Antibody, in Subjects With Advanced Cancer Recruiting NCT03860272 Phase 1 Botensilimab;Balstilimab
12 Phase I Study of MLN0128 (TAK-228) (NSC# 768435) in Combination With Ziv-Aflibercept (NSC# 724770) in Patients With Advanced Cancers Active, not recruiting NCT02159989 Phase 1 Sapanisertib
13 Fibrolamellar Carcinoma Global Dynamic Registry Recruiting NCT04874519
14 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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Organs/tissues related to Fibrolamellar Carcinoma:

MalaCards : Liver, Bone Marrow, Lymph Node, Endothelial, Bone, Pancreatic Islet, Lung
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Publications for Fibrolamellar Carcinoma

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Articles related to Fibrolamellar Carcinoma:

(show top 50) (show all 570)
# Title Authors PMID Year
1
Clinical and demographic predictors of survival for fibrolamellar carcinoma patients-A patient community, registry-based study. 62
36245434 2022
2
High-Level Procalcitonin in Patient with Mixed Fibrolamellar Hepatocellular Carcinoma: A Case Report and Literature Review. 62
34738189 2022
3
β-catenin cancer-enhancing genomic regions axis is involved in the development of fibrolamellar hepatocellular carcinoma. 62
36000549 2022
4
Advances in the treatment of pediatric solid tumors: A 50-year perspective. 62
36087080 2022
5
Clinical Outcomes in Fibrolamellar Hepatocellular Carcinoma Treated with Immune Checkpoint Inhibitors. 62
36358766 2022
6
Development and validation of a nomogram for predicting the cancer-specific survival of fibrolamellar hepatocellular carcinoma patients. 62
35713784 2022
7
The oncogenic fusion protein DNAJB1-PRKACA can be specifically targeted by peptide-based immunotherapy in fibrolamellar hepatocellular carcinoma. 62
36302754 2022
8
Oncogenic addiction of Fibrolamellar hepatocellular carcinoma to the fusion kinase DNAJB1-PRKACA. 62
36302174 2022
9
Novel protein kinase cAMP-Activated Catalytic Subunit Alpha (PRKACA) inhibitor shows anti-tumor activity in a fibrolamellar hepatocellular carcinoma model. 62
35839742 2022
10
Targeting BCL-XL in fibrolamellar hepatocellular carcinoma. 62
36073545 2022
11
Cabozantinib plus atezolizumab versus sorafenib for advanced hepatocellular carcinoma (COSMIC-312): a multicentre, open-label, randomised, phase 3 trial. 62
35798016 2022
12
Human liver organoids for disease modeling of fibrolamellar carcinoma. 62
35803261 2022
13
Prognostic Role of Lymph Node Sampling in Adolescent and Young Adults With Fibrolamellar Carcinoma. 62
35398630 2022
14
Histologic type predicts disparate outcomes in pediatric hepatocellular neoplasms: A Pediatric Surgical Oncology Research Collaborative study. 62
35561331 2022
15
Integrated analysis using ToppMiR uncovers altered miRNA- mRNA regulatory networks in pediatric hepatocellular carcinoma-A pilot study. 62
35859536 2022
16
Refractory Hyperammonemic encephalopathy in Fibrolamellar hepatocellular carcinoma, a case report and literature review. 62
35276469 2022
17
Fibrolamellar hepatocellular carcinoma: A rare but unpleasant event. 62
35949219 2022
18
Multiomic analysis of microRNA-mediated regulation reveals a proliferative axis involving miR-10b in fibrolamellar carcinoma. 62
35482409 2022
19
The Use of Fluorescence in situ Hybridization to Confirm PRKACA Gene Rearrangement in Fibrolamellar Hepatocellular Carcinoma: A Validation Study. 62
35777788 2022
20
Trans-arterial therapy for Fibrolamellar carcinoma: A case report and literature review. 62
35421728 2022
21
Survival characteristics of fibrolamellar hepatocellular carcinoma: A Surveillance, Epidemiology, and End Results database study. 62
35662983 2022
22
A framework for fibrolamellar carcinoma research and clinical trials. 62
35190728 2022
23
Polyendocrine Autoimmunity and Diabetic Ketoacidosis Following Anti-PD-1 and Interferon α. 62
35274131 2022
24
Optical characterization of the liver tissue affected by fibrolamellar hepatocellular carcinoma based on internal filters of laser-induced fluorescence. 62
35414645 2022
25
Magnetic resonance imaging of pediatric liver tumors. 62
33852026 2022
26
Successful Liver Transplantation of Recurrent Fibrolamellar Carcinoma following Clinical and Pathologic Complete Response to Triple Immunochemotherapy: A Case Report. 62
35537414 2022
27
Epidemiological and Clinical Characteristics of Five Rare Pathological Subtypes of Hepatocellular Carcinoma. 62
35463333 2022
28
DNAJB1-PRKACA in HEK293T cells induces LINC00473 overexpression that depends on PKA signaling. 62
35167623 2022
29
[A case of fibrolamellar hepatocellular carcinoma mimicking a liver abscess]. 62
36351623 2022
30
A Multidisciplinary Approach to the Management of Fibrolamellar Carcinoma: Current Perspectives and Future Prospects. 62
36212724 2022
31
Unusual Primary Neoplasms of the Adult Liver: Review of Imaging Appearances and Differential Diagnosis. 62
33199074 2022
32
Factors Influencing Overall Survival for Patients with Fibrolamellar Hepatocellular Carcinoma: Analysis of the Surveillance, Epidemiology, and End Results Database. 62
35035232 2022
33
Differential Diagnosis of Hepatic Mass with Central Scar: Focal Nodular Hyperplasia Mimicking Fibrolamellar Hepatocellular Carcinoma. 62
35054211 2021
34
Transarterial Radioembolization Treatment as a Bridge to Surgical Resection in Pediatric Hepatocellular Carcinoma. 62
33769387 2021
35
Surgical Debulking for Refractory Hyperammonemic Encephalopathy in Fibrolamellar Hepatocellular Carcinoma. 62
34105830 2021
36
Identification of Novel Therapeutic Targets for Fibrolamellar Carcinoma Using Patient-Derived Xenografts and Direct-from-Patient Screening. 62
34127480 2021
37
Integrated Phosphoproteomics for Identifying Substrates of Human Protein Kinase A (PRKACA) and Its Oncogenic Mutant DNAJB1-PRKACA. 62
34436901 2021
38
A case report of fibrolamellar hepatocellular carcinoma, with particular reference to preoperative diagnosis, value of molecular genetic diagnosis, and cell origin. 62
34533614 2021
39
Fibrolamellar carcinoma: An entity all its own. 62
34272087 2021
40
Novel strategy to treat lung metastases: Hybrid therapy involving surgery and radiofrequency ablation. 62
34106518 2021
41
Mixed fibrolamellar hepatocellular carcinoma. 62
34194803 2021
42
Immunohistochemical detection of procalcitonin in fibrolamellar hepatocellular carcinoma. 62
33566306 2021
43
Pediatric fibrolamellar hepatocellular carcinoma: case report and review of the literature. 62
34082642 2021
44
Imaging Features of Fibrolamellar Hepatocellular Carcinoma with Contrast-Enhanced Ultrasound. 62
32102105 2021
45
Bilateral Diffuse Nodular Pulmonary Ossification Mimicking Metastatic Disease in a Patient with Fibrolamellar Hepatocellular Carcinoma. 62
33809687 2021
46
Fibrolamellar Carcinoma. 62
35531303 2021
47
Management of Diagnosis and Treatment in a Case of Fibrolamellar Carcinoma. 62
35399695 2021
48
Defective internal allosteric network imparts dysfunctional ATP/substrate-binding cooperativity in oncogenic chimera of protein kinase A. 62
33692454 2021
49
Fibrolamellar hepatocellular carcinoma treated with atezolizumab and bevacizumab: two case reports. 62
33722275 2021
50
In situ hybridisation for albumin RNA in paediatric liver cancers compared with common immunohistochemical markers. 62
32471888 2021
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Variations for Fibrolamellar Carcinoma

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Cosmic variations for Fibrolamellar Carcinoma:

8 (show top 50) (show all 15388)
# 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 19
2 COSM94430614 ZSWIM1 liver,NS,carcinoma,hepatocellular carcinoma c.1399A>C p.S467R 20:45883991-45883991 19
3 COSM94436083 ZSWIM1 liver,NS,carcinoma,hepatocellular carcinoma c.1399A>C p.S467R 20:45883991-45883991 19
4 COSM94063771 ZSCAN20 liver,NS,carcinoma,hepatocellular carcinoma c.2314G>T p.G772W 1:33494658-33494658 19
5 COSM88264637 ZRSR2 liver,NS,carcinoma,hepatocellular carcinoma c.304G>T p.E102* 23:15803788-15803788 19
6 COSM102741209 ZNRF3 liver,NS,carcinoma,hepatocellular carcinoma c.859A>G p.M287V 22:29049340-29049340 19
7 COSM131494492 ZNRF3 liver,NS,carcinoma,hepatocellular carcinoma c.1159A>G p.M387V 22:29049340-29049340 19
8 COSM103081572 ZNRF3 liver,NS,carcinoma,hepatocellular carcinoma c.859A>G p.M287V 22:29049340-29049340 19
9 COSM95977701 ZNFX1 liver,NS,carcinoma,hepatocellular carcinoma c.2580G>A p.L860= 20:49257501-49257501 19
10 COSM102131817 ZNFX1 liver,NS,carcinoma,hepatocellular carcinoma c.2580G>A p.L860= 20:49257501-49257501 19
11 COSM95960776 ZNFX1 liver,NS,carcinoma,hepatocellular carcinoma c.2580G>A p.L860= 20:49257501-49257501 19
12 COSM113317148 ZNF853 liver,NS,carcinoma,hepatocellular carcinoma c.1610G>T p.G537V 7:6622601-6622601 19
13 COSM99592496 ZNF829 liver,NS,carcinoma,hepatocellular carcinoma c.829T>A p.F277I 19:36891962-36891962 19
14 COSM128686611 ZNF829 liver,NS,carcinoma,hepatocellular carcinoma c.1072T>A p.F358I 19:36891962-36891962 19
15 COSM123358576 ZNF827 liver,NS,carcinoma,hepatocellular carcinoma c.691C>T p.L231= 4:145885684-145885684 19
16 COSM123949383 ZNF827 liver,NS,carcinoma,hepatocellular carcinoma c.1741C>T p.L581= 4:145885684-145885684 19
17 COSM101741672 ZNF827 liver,NS,carcinoma,hepatocellular carcinoma c.1741C>T p.L581= 4:145885684-145885684 19
18 COSM86004822 ZNF653 liver,NS,carcinoma,hepatocellular carcinoma c.1553A>C p.H518P 19:11485673-11485673 19
19 COSM98561269 ZNF615 liver,NS,carcinoma,hepatocellular carcinoma c.141G>A p.V47= 19:52002156-52002156 19
20 COSM142353152 ZNF615 liver,NS,carcinoma,hepatocellular carcinoma c.141G>A p.V47= 19:52002156-52002156 19
21 COSM142221290 ZNF615 liver,NS,carcinoma,hepatocellular carcinoma c.141G>A p.V47= 19:52002156-52002156 19
22 COSM99854658 ZNF615 liver,NS,carcinoma,hepatocellular carcinoma c.156G>A p.V52= 19:52002156-52002156 19
23 COSM139729931 ZNF615 liver,NS,carcinoma,hepatocellular carcinoma c.141G>A p.V47= 19:52002156-52002156 19
24 COSM141470046 ZNF615 liver,NS,carcinoma,hepatocellular carcinoma c.141G>A p.V47= 19:52002156-52002156 19
25 COSM141819438 ZNF615 liver,NS,carcinoma,hepatocellular carcinoma c.141G>A p.V47= 19:52002156-52002156 19
26 COSM145038099 ZNF615 liver,NS,carcinoma,hepatocellular carcinoma c.141G>A p.V47= 19:52002156-52002156 19
27 COSM91868668 ZNF536 liver,NS,carcinoma,hepatocellular carcinoma c.3900G>T p.K1300N 19:30557161-30557161 19
28 COSM91880764 ZNF536 liver,NS,carcinoma,hepatocellular carcinoma c.2171-78T>G p.? 19:30534769-30534769 19
29 COSM140721836 ZNF521 liver,NS,carcinoma,hepatocellular carcinoma c.2998+205G>A p.? 18:25194955-25194955 19
30 COSM131506983 ZNF521 liver,NS,carcinoma,hepatocellular carcinoma c.3658+205G>A p.? 18:25194955-25194955 19
31 COSM94799980 ZNF521 liver,NS,carcinoma,hepatocellular carcinoma c.3658+205G>A p.? 18:25194955-25194955 19
32 COSM90582798 ZNF518B liver,NS,carcinoma,hepatocellular carcinoma c.1624G>T p.E542* 4:10444705-10444705 19
33 COSM87577038 ZNF462 liver,NS,carcinoma,hepatocellular carcinoma c.6715G>T p.V2239L 9:106974156-106974156 19
34 COSM109096265 ZNF462 liver,NS,carcinoma,hepatocellular carcinoma c.3433G>T p.V1145L 9:106974156-106974156 19
35 COSM104985080 ZNF385B liver,NS,carcinoma,hepatocellular carcinoma c.298+23785G>T p.? 2:179745718-179745718 19
36 COSM104427909 ZNF385B liver,NS,carcinoma,hepatocellular carcinoma c.20G>T p.S7I 2:179745718-179745718 19
37 COSM151538615 ZNF385B liver,NS,carcinoma,hepatocellular carcinoma c.253+23785G>T p.? 2:179745718-179745718 19
38 COSM127708351 ZNF345 liver,NS,carcinoma,hepatocellular carcinoma c.47-856A>T p.? 19:36891962-36891962 19
39 COSM135887725 ZNF276 liver,NS,carcinoma,hepatocellular carcinoma c.1168C>G p.L390V 16:89734008-89734008 19
40 COSM84511025 ZNF276 liver,NS,carcinoma,hepatocellular carcinoma c.1219C>G p.L407V 16:89734008-89734008 19
41 COSM111609487 ZNF276 liver,NS,carcinoma,hepatocellular carcinoma c.1444C>G p.L482V 16:89734008-89734008 19
42 COSM92353291 ZNF26 liver,NS,carcinoma,hepatocellular carcinoma c.1010C>T p.T337I 12:133010889-133010889 19
43 COSM130415812 ZNF26 liver,NS,carcinoma,hepatocellular carcinoma c.950C>T p.T317I 12:133010889-133010889 19
44 COSM88697750 ZNF217 liver,NS,carcinoma,hepatocellular carcinoma c.*194A>T p.? 20:53569094-53569094 19
45 COSM95253159 ZNF217 liver,NS,carcinoma,hepatocellular carcinoma c.*194A>T p.? 20:53569094-53569094 19
46 COSM94505616 ZMYND12 liver,NS,carcinoma,hepatocellular carcinoma c.425-1G>T p.? 1:42440026-42440026 19
47 COSM89943752 ZMYM4 liver,NS,carcinoma,hepatocellular carcinoma c.86-8201G>T p.? 1:35350724-35350724 19
48 COSM101398456 ZIC2 liver,NS,carcinoma,hepatocellular carcinoma c.-18C>T p.? 13:99982047-99982047 19
49 COSM113521587 ZGPAT liver,NS,carcinoma,hepatocellular carcinoma c.*197C>A p.? 20:63736116-63736116 19
50 COSM94679661 ZGPAT liver,NS,carcinoma,hepatocellular carcinoma c.*197C>A p.? 20:63736116-63736116 19
Sources

Expression for Fibrolamellar Carcinoma

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

Pathways for Fibrolamellar Carcinoma

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

(show all 48)
# Super pathways Score Top Affiliating Genes
1
Disease 66
Show member pathways
 13.77 ATP1B1 CTNNB1 GPC3 PRKACA PRKACB PRKAR1A
2
Signal Transduction 66
13.74 TP53 PRKX PRKAR2B PRKAR1A PRKACB PRKACA
3
Transmission across Chemical Synapses 66
Show member pathways
 13.1 GLUL PRKACA PRKACB PRKAR1A PRKAR2B PRKX
4
PIP3 activates AKT signaling 66
Show member pathways
 13.01 TP53 PRKX PRKAR2B PRKAR1A PRKACB PRKACA
5
Sertoli-Sertoli Cell Junction Dynamics 64
Show member pathways
 12.88 PRKAR2B PRKAR1A PRKACB PRKACA CTNNB1
6
Beta-Adrenergic Signaling 64
Show member pathways
 12.85 PRKAR2B PRKAR1A PRKACB PRKACA CTNNB1
7
CCR5 Pathway in Macrophages 64
Show member pathways
 12.8 DNAJB1 PRKACA PRKACB PRKAR1A PRKAR2B PRKX
8
DREAM Repression and Dynorphin Expression 64
Show member pathways
 12.72 PRKAR2B PRKAR1A PRKACB PRKACA CTNNB1
9
Calmodulin induced events 66
Show member pathways
 12.7 PRKACA PRKACB PRKAR1A PRKAR2B PRKX
10
G-protein signaling G-Protein alpha-i signaling cascades 22
Show member pathways
 12.63 PRKAR2B PRKAR1A PRKACB PRKACA CTNNB1
11
Neuropathic Pain-Signaling in Dorsal Horn Neurons 64
Show member pathways
 12.62 PRKAR2B PRKAR1A PRKACB PRKACA ATP1B1
12
Beta-2 adrenergic-dependent CFTR expression 22
Show member pathways
 12.55 PRKX PRKAR2B PRKAR1A PRKACB PRKACA
13
Myometrial relaxation and contraction pathways 74
Show member pathways
 12.48 PRKAR2B PRKAR1A PRKACB PRKACA ATP1B1
14
Integration of energy metabolism 66
Show member pathways
 12.46 PRKAR2B PRKAR1A PRKACB PRKACA
15
wtCFTR and delta508-CFTR traffic / Generic schema (norm and CF) 22
Show member pathways
 12.41 PRKAR2B PRKAR1A PRKACB PRKACA
16
Signaling by Hedgehog 66
Show member pathways
 12.4 PRKAR2B PRKAR1A PRKACB PRKACA
17
Dopamine-DARPP32 Feedback onto cAMP Pathway 64
12.32 PRKAR2B PRKAR1A PRKACB PRKACA
18
Activation of NMDA receptors and postsynaptic events 66
Show member pathways
 12.29 PRKX PRKAR2B PRKAR1A PRKACB PRKACA
19
Development Ligand-independent activation of ESR1 and ESR2 22
Show member pathways
 12.28 PRKAR2B PRKAR1A PRKACB PRKACA
20
Development A3 receptor signaling 22
Show member pathways
 12.26 PRKAR2B PRKAR1A PRKACB PRKACA CTNNB1
21
AMPK Enzyme Complex Pathway 64
Show member pathways
 12.15 PRKAR2B PRKAR1A PRKACB PRKACA
22
Regulation of CFTR activity (norm and CF) 22
Show member pathways
 12.14 PRKACA PRKACB PRKAR1A PRKAR2B
23
G alpha (s) signalling events 66
Show member pathways
 12.12 PRKAR2B PRKAR1A PRKACB PRKACA
24
Apoptosis and survival BAD phosphorylation 22
Show member pathways
 12.11 PRKAR2B PRKAR1A PRKACB PRKACA
25
Sudden infant death syndrome (SIDS) susceptibility pathways 74
12.11 PRKAR2B PRKAR1A PRKACB PRKACA CTNNB1
26
Aquaporin-mediated transport 66
Show member pathways
 11.92 PRKAR2B PRKAR1A PRKACB PRKACA
27
Development A2B receptor- action via G-protein alpha s 22
Show member pathways
 11.92 PRKAR2B PRKAR1A PRKACB PRKACA CTNNB1
28
Neurophysiological process Glutamate regulation of Dopamine D1A receptor signaling 22
Show member pathways
 11.82 PRKAR2B PRKAR1A PRKACB PRKACA
29
Signal transduction_PKA signaling 22
11.8 PRKAR2B PRKAR1A PRKACB PRKACA
30
Glucocorticoid receptor regulatory network 61
11.73 TP53 PRKACA AFP
31
G-protein signaling_RhoA regulation pathway 22
11.69 PRKAR2B PRKAR1A PRKACB PRKACA
32
Signal transduction_Erk Interactions- Inhibition of Erk 22
11.69 PRKACA PRKACB PRKAR1A PRKAR2B
33
AP-1 transcription factor network 61
11.67 TP53 NTS CTNNB1
34
Development_Leptin signaling via PI3K-dependent pathway 22
11.51 PRKAR2B PRKAR1A PRKACB PRKACA
35
Netrin Signaling 64
11.47 PRKAR2B PRKAR1A PRKACB PRKACA
36
NgR-p75(NTR)-Mediated Signaling 64
11.42 PRKAR2B PRKAR1A PRKACB PRKACA
37
Melanocyte Development and Pigmentation 64
11.32 PRKACA PRKACB PRKAR1A PRKAR2B
38
Colorectal Cancer Metastasis 64
11.32 TP53 PRKAR2B PRKAR1A PRKACB PRKACA CTNNB1
39
Alpha4 beta1 integrin signaling events 61
11.31 PRKAR1A PRKACB PRKACA
40
Development_Hedgehog and PTH signaling pathways in bone and cartilage development 22
11.26 PRKAR2B PRKAR1A PRKACB PRKACA
41
miRs in Muscle Cell Differentiation 74
11.15 PRKAR2B PRKAR1A PRKACB PRKACA
42
14-3-3 and Regulation of BAD Activity 64
11.06 PRKAR2B PRKAR1A PRKACB PRKACA
43
Rap1 signalling 66
10.9 PRKACB PRKACA
44
G-protein signaling_Rap1B regulation pathway 22
10.84 PRKACB PRKACA
45
Transcription_CREM signaling in testis 22
10.82 PRKAR2B PRKAR1A PRKACB PRKACA
46
Dopamine metabolism 74
10.8 PRKACB PRKACA
47
ROBO receptors bind AKAP5 66
10.62 PRKACB PRKACA
48
Cannabinoid receptor signaling 74
Show member pathways
 10.58 PRKAR2B PRKAR1A PRKACB PRKACA
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 ciliary base GO:0097546 9.56 PRKAR2B PRKAR1A PRKACB PRKACA
2 cAMP-dependent protein kinase complex GO:0005952 9.32 PRKX PRKAR2B PRKAR1A PRKACB PRKACA

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

# Name GO ID Score Top Affiliating Genes
1 negative regulation of smoothened signaling pathway involved in dorsal/ventral neural tube patterning GO:1901621 8.92 PRKACB PRKACA

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

# Name GO ID Score Top Affiliating Genes
1 ubiquitin protein ligase binding GO:0031625 9.85 TP53 PRKAR2B PRKAR1A PRKACB PRKACA
2 AMP-activated protein kinase activity GO:0004679 9.62 PRKACB PRKACA
3 cAMP-dependent protein kinase regulator activity GO:0008603 9.56 PRKAR2B PRKAR1A
4 cAMP binding GO:0030552 9.35 PRKAR2B PRKAR1A LINC00473
5 cAMP-dependent protein kinase activity GO:0004691 9.1 PRKX PRKACB PRKACA
Sources

Sources for Fibrolamellar Carcinoma

About this section
2 CDC
6 CNVD
8 Cosmic
9 dbSNP
10 DGIdb
16 EFO
17 ExPASy
18 FMA
19 GARD
27 GO
28 GTR
29 HMDB
30 HPO
31 ICD10
32 ICD10 via Orphanet
33 ICD11
34 ICD9CM
35 IUPHAR
36 LifeMap
38 LOVD
40 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
52 NINDS
53 Novoseek
55 ODiseA
56 OMIM via Orphanet
57 OMIM® (Updated 08-Dec-2022)
61 PubChem
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 Tocris
71 UMLS
72 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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