MCID: HPT022
MIFTS: 58

Hepatoblastoma

Categories: Cancer diseases, Endocrine diseases, Gastrointestinal diseases, Liver diseases, Rare diseases

Aliases & Classifications for Hepatoblastoma

MalaCards integrated aliases for Hepatoblastoma:

Name: Hepatoblastoma 12 75 53 37 29 55 6 44 15 17 72

Classifications:



External Ids:

Disease Ontology 12 DOID:687
KEGG 37 H02302
MeSH 44 D018197
NCIt 50 C3728
SNOMED-CT 68 45024009
ICD10 33 C22.2
UMLS 72 C0206624

Summaries for Hepatoblastoma

NIH Rare Diseases : 53 Hepatoblastoma is a rare malignant (cancerous) tumor of the liver that usually occurs in the first 3 years of life. In early stages of the condition, there may be no concerning signs or symptoms. As the tumor gets larger, affected children may experience a painful, abdominal lump; swelling of the abdomen; unexplained weight loss; loss of appetite; and/or nausea and vomiting. The exact underlying cause of hepatoblastoma is poorly understood. Risk factors for the tumor include prematurity with a very low birth weight, early exposure to hepatitis B infection, biliary atresia, and several different genetic conditions (i.e. Beckwith-Wiedemann syndrome, familial adenomatous polyposis, Aicardi syndrome, Glycogen storage disease, and Simpson-Golabi-Behmel syndrome). Treatment varies based on the severity of the condition but may include a combination of surgery, watchful waiting, chemotherapy, and/or radiation therapy.

MalaCards based summary : Hepatoblastoma is related to beckwith-wiedemann syndrome and familial adenomatous polyposis, and has symptoms including icterus An important gene associated with Hepatoblastoma is APC (APC Regulator Of WNT Signaling Pathway), and among its related pathways/superpathways are Wnt signaling pathway and PI3K-Akt signaling pathway. The drugs Sorafenib and Nicotine have been mentioned in the context of this disorder. Affiliated tissues include liver, fetal liver and kidney, and related phenotypes are cellular and homeostasis/metabolism

KEGG : 37
Although malignant tumors of the liver are rare during childhood, hepatoblastoma is the most common liver cancer in children, usually diagnosed during the first 3 years of life. These tumors are thought to arise from hepatic progenitors or hepatoblasts. The most frequent genetic aberrations (70-90%) in hepatoblastoma occur in genes involved in the Wnt signaling pathway. The Wnt signaling pathway is activated often by acquired activating mutations of the beta-catenin (CTNNB1) gene, and less commonly through constitutional mutations of the APC gene or somatic mutations of other genes in the pathway.

Wikipedia : 75 Hepatoblastoma is an uncommon malignant liver cancer occurring in infants and children and composed of... more...

Related Diseases for Hepatoblastoma

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

(show top 50) (show all 393)
# Related Disease Score Top Affiliating Genes
1 beckwith-wiedemann syndrome 32.2 TP53 IGF2 H19 GPC3 CDKN1C AFP
2 familial adenomatous polyposis 31.7 TP53 CTNNB1 APC
3 familial adenomatous polyposis 1 31.4 CTNNB1 APC
4 adenoma 31.3 TP53 PLAG1 CTNNB1 APC
5 desmoid disease, hereditary 31.0 CTNNB1 APC
6 rhabdomyosarcoma 30.9 TP53 PTCH1 IGF2 H19
7 embryonal sarcoma 30.9 TP53 GPC3 CTNNB1
8 embryoma 30.8 IGF2 GPC3 AFP
9 adult hepatocellular carcinoma 30.8 TP53 CTNNB1
10 teratoma 30.8 TP53 KRT7 AFP
11 colorectal adenoma 30.8 TP53 CTNNB1 APC
12 hemihyperplasia, isolated 30.6 IGF2 H19 CDKN1C
13 osteogenic sarcoma 30.4 TUG1 TP53 H19 CASP3
14 hepatic adenomas, familial 30.3 HNF1A GPC3 CTNNB1 APC AFP
15 testicular yolk sac tumor 30.1 GPC3 APOA1 AFP
16 adenocarcinoma 30.1 TP53 H19 CTNNB1 BIRC5 APC
17 intrahepatic cholangiocarcinoma 29.9 TUG1 TP53 KRT7 CTNNB1 AFP
18 umbilical hernia 29.9 IGF2 H19 CDKN1C
19 hepatocellular carcinoma 29.8 TUG1 TP53 KRT7 IGF2 HNF1A HGF
20 adrenocortical carcinoma, hereditary 29.6 TP53 IGF2 H19 CTNNB1 CDKN1C
21 medulloblastoma 28.9 TP53 PTCH1 IGF2 HGF H19 CTNNB1
22 wilms tumor 1 28.8 TP53 KRT7 IGF2 H19 GPC3 CTNNB1
23 cholangiocarcinoma 28.7 TUG1 TP53 KRT7 HGF H19 GPC3
24 lung cancer susceptibility 3 28.0 TUG1 TP53 KRT7 HGF H19 CTNNB1
25 colorectal cancer 27.5 TUG1 TP53 KRT7 IGF2 H19 CTNNB1
26 mixed hepatoblastoma 12.4
27 macrotrabecular hepatoblastoma 12.4
28 silver-russell syndrome due to 11p15 microduplication 10.8 IGF2 H19
29 silver-russell syndrome due to an imprinting defect of 11p15 10.8 IGF2 H19
30 beckwith-wiedemann syndrome due to imprinting defect of 11p15 10.8 IGF2 H19
31 calcifying epithelial odontogenic tumor 10.7 TP53 PTCH1
32 hyperplastic polyposis syndrome 10.7 TP53 APC
33 intestinal benign neoplasm 10.7 TP53 CTNNB1 APC
34 short-rib thoracic dysplasia 4 with or without polydactyly 10.6 TP53 CASP3
35 infratentorial cancer 10.6 TP53 PTCH1 CTNNB1
36 peutz-jeghers syndrome 10.6 TP53 CTNNB1 APC
37 atypical teratoid rhabdoid tumor 10.6 TP53 IGF2 CTNNB1
38 testicular malignant germ cell cancer 10.6 GPC3 AFP
39 pharynx cancer 10.6 TP53 CTNNB1 CASP3
40 nasopharyngeal disease 10.6 TP53 CTNNB1 CASP3
41 cervix disease 10.6 TP53 CTNNB1 CASP3
42 meninges sarcoma 10.6 TP53 IGF2
43 marek disease 10.6 TP53 H19
44 tongue cancer 10.5 TP53 CTNNB1 CASP3
45 colonic disease 10.5 TP53 CTNNB1 CASP3
46 male reproductive organ cancer 10.5 TP53 CTNNB1 CASP3
47 stomach disease 10.5 TP53 CTNNB1 CASP3
48 chromosomal triplication 10.5
49 intrahepatic bile duct adenoma 10.5 KRT7 AFP
50 keratocystic odontogenic tumor 10.5 TP53 PTCH1 BIRC5

Graphical network of the top 20 diseases related to Hepatoblastoma:



Diseases related to Hepatoblastoma

Symptoms & Phenotypes for Hepatoblastoma

UMLS symptoms related to Hepatoblastoma:


icterus

MGI Mouse Phenotypes related to Hepatoblastoma:

46 (show all 18)
# Description MGI Source Accession Score Top Affiliating Genes
1 cellular MP:0005384 10.35 APC APOA1 BIRC5 CASP3 CTNNB1 GPC3
2 homeostasis/metabolism MP:0005376 10.31 AFP APC APOA1 BIRC5 CASP3 CDO1
3 endocrine/exocrine gland MP:0005379 10.3 AFP APC APOA1 BIRC5 CASP3 CDO1
4 cardiovascular system MP:0005385 10.27 APC APOA1 CASP3 CDO1 CTNNB1 GPC3
5 embryo MP:0005380 10.19 APC BIRC5 CTNNB1 GPC3 HGF IGF2
6 craniofacial MP:0005382 10.16 APC CASP3 CDO1 CTNNB1 GPC3 IGF2
7 digestive/alimentary MP:0005381 10.15 APC CASP3 CTNNB1 GPC3 HNF1A IGF2
8 integument MP:0010771 10.13 APC APOA1 CASP3 CTNNB1 GPC3 HNF1A
9 liver/biliary system MP:0005370 10.03 AFP APC APOA1 CTNNB1 HGF HNF1A
10 limbs/digits/tail MP:0005371 10 APC CDO1 CTNNB1 GPC3 IGF2 PTCH1
11 reproductive system MP:0005389 10 AFP APC CASP3 CDO1 CTNNB1 GPC3
12 hearing/vestibular/ear MP:0005377 9.99 APC CASP3 CDO1 CTNNB1 PTCH1 TP53
13 renal/urinary system MP:0005367 9.97 APC CASP3 CTNNB1 GPC3 HNF1A IGF2
14 no phenotypic analysis MP:0003012 9.95 CASP3 CTNNB1 HGF HNF1A IGF2 PTCH1
15 pigmentation MP:0001186 9.85 APC CASP3 CTNNB1 GPC3 PTCH1 TP53
16 respiratory system MP:0005388 9.7 CASP3 CDO1 CTNNB1 GPC3 IGF2 PTCH1
17 skeleton MP:0005390 9.61 APC CASP3 CDO1 CTNNB1 GPC3 HNF1A
18 vision/eye MP:0005391 9.23 APC CASP3 CDO1 CTNNB1 IGF2 PLAG1

Drugs & Therapeutics for Hepatoblastoma

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

(show top 50) (show all 120)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Sorafenib Approved, Investigational Phase 4 284461-73-0 216239 406563
2
Nicotine Approved Phase 4 54-11-5 942 89594
3 Liver Extracts Phase 4
4 Protein Kinase Inhibitors Phase 4
5
Carboplatin Approved Phase 3 41575-94-4 10339178 498142 38904
6
Oxaliplatin Approved, Investigational Phase 3 61825-94-3 5310940 9887054 43805 6857599
7
Gemcitabine Approved Phase 3 95058-81-4 60750
8
Daunorubicin Approved Phase 2, Phase 3 20830-81-3 30323
9
Irinotecan Approved, Investigational Phase 3 100286-90-6, 97682-44-5 60838
10
Cisplatin Approved Phase 3 15663-27-1 84093 441203 2767
11
Doxorubicin Approved, Investigational Phase 3 23214-92-8 31703
12
Sirolimus Approved, Investigational Phase 3 53123-88-9 6436030 5284616 46835353
13
Fluorouracil Approved Phase 3 51-21-8 3385
14
Vincristine Approved, Investigational Phase 3 57-22-7, 2068-78-2 5978
15
Everolimus Approved Phase 3 159351-69-6 6442177 70789204
16
Miconazole Approved, Investigational, Vet_approved Phase 3 22916-47-8 4189
17
Sevoflurane Approved, Vet_approved Phase 2, Phase 3 28523-86-6 5206
18
Propofol Approved, Investigational, Vet_approved Phase 2, Phase 3 2078-54-8 4943
19
Rocuronium Approved Phase 2, Phase 3 119302-91-9, 143558-00-3 441290
20
Histamine Approved, Investigational Phase 3 51-45-6 774
21
Cyproheptadine Approved Phase 3 129-03-3 2913
22
Camptothecin Experimental Phase 3 7689-03-4
23 Antidotes Phase 3
24 sodium thiosulfate Phase 3
25 Antioxidants Phase 3
26 Chelating Agents Phase 3
27 Antitubercular Agents Phase 3
28 Antiviral Agents Phase 3
29 Keratolytic Agents Phase 2, Phase 3
30 Podophyllotoxin Phase 2, Phase 3 518-28-5
31 topoisomerase I inhibitors Phase 3
32 Anti-Infective Agents Phase 3
33 Anti-Bacterial Agents Phase 3
34
Liposomal doxorubicin Phase 3 31703
35 Antibiotics, Antitubercular Phase 3
36 razoxane Phase 3
37 Cardiotonic Agents Phase 3
38 Antifungal Agents Phase 3
39 Tubulin Modulators Phase 3
40 3,6-bis(5-chloro-2-piperidyl)-2,5-piperazinedione Phase 3
41 Hematinics Phase 3
42 Antimitotic Agents Phase 3
43 Antimetabolites Phase 3
44 Antimetabolites, Antineoplastic Phase 3
45 Central Nervous System Depressants Phase 2, Phase 3
46 Anesthetics Phase 2, Phase 3
47 Bromides Phase 2, Phase 3
48 Complement C3a Phase 3
49 Dermatologic Agents Phase 3
50 Gastrointestinal Agents Phase 3

Interventional clinical trials:

(show top 50) (show all 58)
# Name Status NCT ID Phase Drugs
1 G-CSF Alone or Combination With GM-CSF on Prevention and Treatment of Infection in Children With Malignant Tumor: a Prospective, Multicentre, Randomised Controlled Trial Recruiting NCT02933333 Phase 4
2 Mechanism of Sorafenib Resistance in Patients With Advanced Hepatocellular Carcinoma Recruiting NCT02733809 Phase 4 Sorafenib
3 A Randomized Phase III Study of Sodium Thiosulfate for the Prevention of Cisplatin-Induced Ototoxicity in Children Completed NCT00716976 Phase 3 sodium thiosulfate
4 Liver Tumour Studies - Hepatoblastoma and Hepatocellular Carcinoma Completed NCT00003912 Phase 3 carboplatin;cisplatin;doxorubicin hydrochloride
5 A Multi-centre Open-label Randomised Phase III Trial of the Efficacy of Sodium Thiosulphate in Reducing Ototoxicity in Patients Receiving Cisplatin Chemotherapy for Standard Risk Hepatoblastoma Completed NCT00652132 Phase 3 cisplatin;sodium thiosulfate
6 Intergroup Protocol for Treatment of Children With Hepatoblastoma Completed NCT00003994 Phase 3 cisplatin;vincristine sulfate;fluorouracil;amifostine trihydrate;carboplatin
7 Paediatric Hepatic International Tumour Trial Recruiting NCT03017326 Phase 3 Cisplatin;Doxorubicin;Carboplatin;5Fluorouracil;Vincristine;Etoposide;Irinotecan;Gemcitabine;Oxaliplatin;Sorafenib
8 Pediatric Hepatic Malignancy International Therapeutic Trial (PHITT) Recruiting NCT03533582 Phase 2, Phase 3 Carboplatin;Cisplatin;Doxorubicin;Etoposide;Fluorouracil;Gemcitabine;Irinotecan;Oxaliplatin;Sorafenib;Vincristine Sulfate
9 Treatment of Children With All Stages of Hepatoblastoma With Temsirolimus (NSC#683864) Added to High Risk Stratum Treatment Active, not recruiting NCT00980460 Phase 3 Cisplatin;Dexrazoxane;Doxorubicin Hydrochloride;Fluorouracil;Irinotecan Hydrochloride;Temsirolimus;Vincristine Sulfate
10 Efficacy and Safety Study of Sugammadex in Children 0-2 Years Old Not yet recruiting NCT03728543 Phase 2, Phase 3 Sugammadex
11 A Randomized, Open-Label, Multicenter, Controlled Study to Assess Safety and Efficacy of ELAD® in Subjects With Severe Acute Alcoholic Hepatitis (sAAH) and Lille Score Failure Terminated NCT01829347 Phase 3
12 Comparative Evaluation of MRI and MDCT for the Detection of Metastic Pulmonary Nodules Terminated NCT00751920 Phase 3
13 Prevention of Cancer/Treatment-Related Weight Loss in Children at High Nutritional Risk Terminated NCT01132547 Phase 3 cyproheptadine hydrochloride
14 Irinotecan Single-Drug Treatment For Children With Refractory or Recurrent Hepatoblastoma Unknown status NCT00287976 Phase 2 irinotecan hydrochloride
15 Intensified Pre-Operative Chemotherapy And Radical Surgery For High Risk Hepatoblastoma Unknown status NCT00077389 Phase 2 carboplatin;cisplatin;doxorubicin hydrochloride
16 High-Dose Chemotherapy With Tandem Peripheral Blood Stem Cell (PBSC) Rescue for the Treatment of High-Risk Pediatric Solid Tumors. Unknown status NCT00179816 Phase 1, Phase 2 High-Dose Chemotherapy with Tandem PBSC Rescue.
17 A Phase I/II Study of LDE225 in Pediatric Patients With Recurrent or Refractory Medulloblastoma or Other Tumors Potentially Dependent on the Hedgehog-signaling Pathway and Adult Patients With Recurrent or Refractory Medulloblastoma Completed NCT01125800 Phase 1, Phase 2 LDE225
18 A Phase II Study of MLN8237, a Selective Aurora A Kinase Inhibitor in Children With Recurrent/Refractory Solid Tumors and Leukemias Completed NCT01154816 Phase 2 Alisertib
19 A Phase II Study of Oxaliplatin in Children With Recurrent Solid Tumors Completed NCT00091182 Phase 2 oxaliplatin
20 A Phase II Study of IMC-A12 (Anti-IGF-I Receptor Monoclonal Antibody, NSC #742460) in Children With Relapsed/Refractory Solid Tumors Completed NCT00831844 Phase 2
21 A Phase II Study of Hyperthermic Peritoneal Perfusion (HIPEC) for Adolescent and Young Adults With Desmoplastic Small Round Cell Tumor and Other Non-Carcinomas Completed NCT01277744 Phase 2 Cisplatin
22 Phase 2 Single- Arm Studies of Temozolomide in Combination With Topotecan in Refractory and Relapsed Neuroblastoma and Other Paediatric Solid Tumours Completed NCT00918320 Phase 2 Temozolomide/Hycamtin (Topotecan)
23 A Phase II Study of Pazopanib GW786034, NSC# 737754 in Children, Adolescents and Young Adults With Refractory Solid Tumors Recruiting NCT01956669 Phase 2 Pazopanib GW786034
24 NCI-COG Pediatric MATCH (Molecular Analysis for Therapy Choice)- Phase 2 Subprotocol of Olaparib in Patients With Tumors Harboring Defects in DNA Damage Repair Genes Recruiting NCT03233204 Phase 2 Olaparib
25 NCI-COG Pediatric MATCH (Molecular Analysis for Therapy Choice)- Phase 2 Subprotocol of Vemurafenib in Patients With Tumors Harboring Braf V600 Mutations Recruiting NCT03220035 Phase 2 Vemurafenib
26 NCI-COG Pediatric MATCH (Molecular Analysis for Therapy Choice) - Phase 2 Subprotocol of Palbociclib in Patients With Tumors Harboring Activating Alterations in Cell Cycle Genes Recruiting NCT03526250 Phase 2 Palbociclib
27 NCI-COG Pediatric MATCH (Molecular Analysis for Therapy Choice) - Phase 2 Subprotocol of BVD-523FB (Ulixertinib) in Patients With Tumors Harboring Activating MAPK Pathway Mutations Recruiting NCT03698994 Phase 2 Ulixertinib
28 Phase 2 Trial of XL184 (Cabozantinib) an Oral Small-Molecule Inhibitor of Multiple Kinases, in Children and Young Adults With Refractory Sarcomas, Wilms Tumor, and Other Rare Tumors Recruiting NCT02867592 Phase 2 Cabozantinib;Cabozantinib S-malate
29 NCI-COG Pediatric MATCH (Molecular Analysis for Therapy Choice) - Phase 2 Subprotocol of LOXO-101 (Larotrectinib) in Patients With Tumors Harboring Actionable NTRK Fusions Recruiting NCT03213704 Phase 2 Larotrectinib;Larotrectinib Sulfate
30 NCI-COG Pediatric MATCH (Molecular Analysis for Therapy Choice)- Phase 2 Subprotocol of Tazemetostat in Patients With Tumors Harboring Alterations in EZH2 or Members of the SWI/SNF Complex Recruiting NCT03213665 Phase 2 Tazemetostat
31 NCI-COG Pediatric MATCH (Molecular Analysis for Therapy Choice) - Phase 2 Subprotocol of JNJ-42756493 (Erdafitinib) in Patients With Tumors Harboring FGFR1/2/3/4 Alterations Recruiting NCT03210714 Phase 2 Erdafitinib
32 NCI-COG Pediatric MATCH (Molecular Analysis For Therapy Choice)- Phase 2 Subprotocol of Ly3023414 in Patients With Solid Tumors Recruiting NCT03213678 Phase 2 Samotolisib
33 A Phase II Trial of Reduced Intensity Conditioning and Partially HLA-mismatched (HLA-haploidentical) Related Donor Bone Marrow Transplantation for High-risk Solid Tumors Recruiting NCT01804634 Phase 2 Cyclophosphamide;Fludarabine;Melphalan;Tacrolimus
34 NCI-COG Pediatric MATCH (Molecular Analysis for Therapy Choice) Screening Protocol Recruiting NCT03155620 Phase 2 Ensartinib;Erdafitinib;Larotrectinib;Olaparib;Palbociclib;Samotolisib;Selumetinib Sulfate;Tazemetostat;Ulixertinib;Vemurafenib
35 A Phase II Study of Imetelstat (GRN163L, NSC# 754228) in Children With Relapsed or Refractory Solid Tumors Withdrawn NCT02011126 Phase 2 Imetelstat Sodium
36 TREATMENT OF CHILDREN AND YOUNG ADULTS WITH RECURRENT/REFRACTORY SOLID TUMORS WITH HIGH DOSE ETOPOSIDE AND CARBOPLATIN PLUS ESCALATING DOSE CYCLOPHOSPHAMIDE, FOLLOWED BY HEMATOPOIETIC RESCUE USING AUTOLOGOUS CD34+ SELECTED BLOOD STEM CELLS: A PILOT STUDY Completed NCT00007813 Phase 1 carboplatin;cyclophosphamide;etoposide
37 A Phase I Trial With Escalating Doses of Oxaliplatin and Hyperthermic Intraperitoneal Chemotherapy (HIPEC) in Children and Adolescents With Extensive Colon Carcinoma Completed NCT02062749 Phase 1 Oxaliplatin
38 Phase I Study of EGFR806 CAR T Cell Immunotherapy for Recurrent/Refractory Solid Tumors in Children and Young Adults Recruiting NCT03618381 Phase 1
39 A Phase 1 Study Using Simvastatin in Combination With Topotecan and Cyclophosphamide in Relapsed and/or Refractory Pediatric Solid and CNS Tumors Recruiting NCT02390843 Phase 1 Simvastatin;Cyclophosphamide;Topotecan;Myeloid growth factor
40 AflacST1603: A Phase 1 Study Using Nab-paclitaxel (Abraxane®) in Combination With Gemcitabine for Pediatric Relapsed and Refractory Solid Tumors Recruiting NCT03507491 Phase 1 Gemcitabine;Nab-paclitaxel
41 A Multi-center, Open-label, Non-randomized, Phase I Dose Escalation Study of Regorafenib (BAY 73-4506) in Pediatric Subjects With Solid Malignant Tumors That Are Recurrent or Refractory to Standard Therapy. Active, not recruiting NCT02085148 Phase 1 Regorafenib (BAY73-4506);Vincristine (Cellcristin®);Irinotecan (Irinotecan Cell pharm®)
42 Sirolimus in Combination With Metronomic Therapy in Children With Recurrent and Refractory Solid Tumors: A Phase I Study Active, not recruiting NCT01331135 Phase 1 sirolimus
43 A Phase I Study of Cytoreductive Surgery and Hyperthermic Intrathoracic Pleural Chemotherapy (HITC) With Escalating Doses for Children and Adolescents With Unilateral Pleural Malignancy Active, not recruiting NCT01998529 Phase 1 Cisplatin;Amifostine
44 A Phase I Study of the Chemoprotectant Amifostine With Autologous Stem Cell Transplantation for High Risk or Relapsed Pediatric Solid Tumors and Brain Tumors Terminated NCT00003926 Phase 1 amifostine trihydrate;busulfan;filgrastim;melphalan;thiotepa
45 Dose Escalation of Total Marrow Irradiation Added to an Alkylator-Intense Conditioning Regimen for Patients With High Risk or Relapsed Solid Tumors Terminated NCT00623077 Phase 1 busulfan;etoposide;ifosfamide;melphalan;thiotepa;Mesna
46 Neutrophil Extracellular Traps (NETs) Formation Following Chemotherapy for Pediatric Hematological and Solid Tumors, and Its Relation to Other Neutrophil Functions and the Role of NETs in Antitumor Activity Unknown status NCT01533779
47 Cancer and Deafness Associated With the Use of Platinum Derivatives in Children Unknown status NCT02425397
48 Genetics and Biology of Liver Tumorigenesis in Children Completed NCT01336881
49 Investigation of DICER1 in Cystic Nephroma and Cystic Partially Differentiated Nephroblastoma Completed NCT01353300
50 Establishing Continuous Cell Lines and Xenografts From Pediatric Cancers for Biological and Pre-Clinical Therapeutic Studies Completed NCT00898755

Search NIH Clinical Center for Hepatoblastoma

Inferred drug relations via UMLS 72 / NDF-RT 51 :


Cisplatin
Vincristine

Cochrane evidence based reviews: hepatoblastoma

Genetic Tests for Hepatoblastoma

Genetic tests related to Hepatoblastoma:

# Genetic test Affiliating Genes
1 Hepatoblastoma 29

Anatomical Context for Hepatoblastoma

MalaCards organs/tissues related to Hepatoblastoma:

41
Liver, Fetal Liver, Kidney, Brain, Lung, Bone, Colon

Publications for Hepatoblastoma

Articles related to Hepatoblastoma:

(show top 50) (show all 3149)
# Title Authors PMID Year
1
Hepatocyte growth factor protects hepatoblastoma cells from chemotherapy-induced apoptosis by AKT activation. 9 38
20372801 2010
2
Involvement of mTOR and survivin inhibition in tamoxifen-induced apoptosis in human hepatoblastoma cell line HepG2. 9 38
19931998 2010
3
Establishment and characterization of a cancer cell line derived from an aggressive childhood liver tumor. 9 38
19637320 2009
4
Predictive power of pretreatment prognostic factors in children with hepatoblastoma: a report from the Children's Oncology Group. 9 38
19588519 2009
5
Could APC gene screening be useful in children with hepatoblastoma? Early onset of adenocarcinoma in a child with familial adenomatous polyposis and hepatoblastoma. 9 38
20210251 2009
6
Regulation of the human SLC25A20 expression by peroxisome proliferator-activated receptor alpha in human hepatoblastoma cells. 9 38
19748481 2009
7
Regulation of expression of apolipoprotein A-I by selenium status in human liver hepatoblastoma cells. 9 38
19294445 2009
8
Thyroid hormones act indirectly to increase sex hormone-binding globulin production by liver via hepatocyte nuclear factor-4alpha. 9 38
19336534 2009
9
Histologic subtypes of hepatoblastoma are characterized by differential canonical Wnt and Notch pathway activation in DLK+ precursors. 9 38
19200579 2009
10
Knockdown of ATP8B1 expression leads to specific downregulation of the bile acid sensor FXR in HepG2 cells: effect of the FXR agonist GW4064. 9 38
19228886 2009
11
Peroxisome-proliferator receptor gamma represses hepatic sex hormone-binding globulin expression. 9 38
19179433 2009
12
Effects of niacin and chromium on the expression of ATP-binding cassette transporter A1 and apolipoprotein A-1 genes in HepG2 cells. 9 38
18602814 2009
13
[Successful treatment of second hepatoblastoma relapse--case report]. 9 38
19780340 2009
14
Hepatic stem-like phenotype and interplay of Wnt/beta-catenin and Myc signaling in aggressive childhood liver cancer. 9 38
19061838 2008
15
Loss of imprinting of IGF2 correlates with hypermethylation of the H19 differentially methylated region in hepatoblastoma. 9 38
19034281 2008
16
Rosuvastatin selectively stimulates apolipoprotein A-I but not apolipoprotein A-II synthesis in Hep G2 cells. 9 38
18555840 2008
17
Mesenchymal hamartoma of the liver mimicking hepatoblastoma. 9 38
18525464 2008
18
Diagnostic value of lectin reactive alpha-fetoprotein for neoinfantile hepatic tumors and malignant germ cell tumors: preliminary study. 9 38
18525461 2008
19
The expression of Bcl-XL, Bcl-XS and p27Kip1 in topotecan-induced apoptosis in hepatoblastoma HepG2 cell line. 9 38
18568767 2008
20
Hepatoblastoma with a low serum alpha-fetoprotein level at diagnosis: the SIOPEL group experience. 9 38
18166449 2008
21
Variable antigen expression in hepatoblastomas. 9 38
18227729 2008
22
Expression of glypican 3 in hepatoblastoma: an immunohistochemical study of 65 cases. 9 38
17949790 2008
23
Combining intracellular and secreted bioluminescent reporter proteins for multicolor cell-based assays. 9 38
18264589 2008
24
Sporadic childhood hepatoblastomas show activation of beta-catenin, mismatch repair defects and p53 mutations. 9 38
17962810 2008
25
Monosaccharide-induced lipogenesis regulates the human hepatic sex hormone-binding globulin gene. 9 38
17992261 2007
26
Pioglitazone stimulates apolipoprotein A-I production without affecting HDL removal in HepG2 cells: involvement of PPAR-alpha. 9 38
17872455 2007
27
Promoter hypermethylation of the RASSF1A gene predicts the poor outcome of patients with hepatoblastoma. 9 38
16937357 2007
28
Erythropoietin receptor presence in hepatoblastoma: a possible link to increased incidence of hepatoblastoma in very low birthweight infants. 9 38
16937361 2007
29
Insulin-like growth factor (IGF)-II regulates CCAAT/enhancer binding protein alpha expression via phosphatidyl-inositol 3 kinase in human hepatoblastoma cell lines. 9 38
17372916 2007
30
[Application of the human hepatoblastoma cell lines inducibly expressing peroxisome proliferator-activated receptors (PPARs)]. 9 38
17666873 2007
31
Further delineation of the phenotype resulting from BRAF or MEK1 germline mutations helps differentiate cardio-facio-cutaneous syndrome from Costello syndrome. 9 38
17551924 2007
32
A novel function of capsaicin-sensitive TRPV1 channels: involvement in cell migration. 9 38
17184838 2007
33
Mesenchymal hamartoma of the liver associated with features of Beckwith-Wiedemann syndrome and high serum alpha-fetoprotein levels. 9 38
17535089 2007
34
siRNA-mediated Bcl-2 and Bcl-xl gene silencing sensitizes human hepatoblastoma cells to chemotherapeutic drugs. 9 38
17439414 2007
35
PLAG1, the prototype of the PLAG gene family: versatility in tumour development (review). 9 38
17332914 2007
36
Ornithine transcarbamylase and arginase I deficiency are responsible for diminished urea cycle function in the human hepatoblastoma cell line HepG2. 9 38
17098461 2007
37
Should children at risk for familial adenomatous polyposis be screened for hepatoblastoma and children with apparently sporadic hepatoblastoma be screened for APC germline mutations? 9 38
16317745 2006
38
Signaling pathway of insulin-like growth factor-II as a target of molecular therapy for hepatoblastoma. 9 38
17072986 2006
39
Effective treatment of tumors with strong beta-catenin/T-cell factor activity by transcriptionally targeted oncolytic herpes simplex virus vector. 9 38
17047077 2006
40
Identification of human low-density lipoprotein receptor as a novel target gene regulated by liver X receptor alpha. 9 38
16920108 2006
41
The hook effect: a need for constant vigilance. 9 38
16824284 2006
42
In vitro RNA interference against beta-catenin inhibits the proliferation of pediatric hepatic tumors. 9 38
16465377 2006
43
Thrombopoietin stimulates migration and activates multiple signaling pathways in hepatoblastoma cells. 9 38
16150872 2006
44
Integrating the diagnosis of childhood malignancies. 9 38
17163162 2006
45
The glypican 3 oncofetal protein is a promising diagnostic marker for hepatocellular carcinoma. 9 38
15920546 2005
46
Hepatoblastoma in a child with progressive familial intrahepatic cholestasis. 9 38
16269056 2005
47
Iron and insulin resistance. 9 38
16225476 2005
48
Kidney volume and plasma hepatocyte growth factor-transforming growth factor beta1 ratio among children with biliary atresia before and after liver transplantation: the reversibility of nephromegaly. 9 38
16253722 2005
49
Second case of hepatoblastoma in a young patient with Simpson-Golabi-Behmel syndrome. 9 38
16166055 2005
50
Gene expression profiling of potential peroxisome proliferator-activated receptor (PPAR) target genes in human hepatoblastoma cell lines inducibly expressing different PPAR isoforms. 9 38
16197558 2005

Variations for Hepatoblastoma

ClinVar genetic disease variations for Hepatoblastoma:

6 (show all 13)
# Gene Variation Type Significance SNP ID GRCh37 Pos GRCh38 Pos
1 CTNNB1 NM_001904.4(CTNNB1): c.84_95del (p.Gln28_Asp32delinsHis) deletion other rs1553630102 3:41266086-41266097 3:41224596-41224607
2 MN1 NM_002430.3(MN1): c.912_914GCA[5] (p.Gln309del) short repeat other rs747503495 22:28195603-28195605 22:27799615-27799617
3 ARID1A NM_006015.6(ARID1A): c.6259G> A (p.Gly2087Arg) single nucleotide variant other rs1553153748 1:27106648-27106648 1:26780157-26780157
4 APC NM_000038.6(APC): c.4183A> T (p.Ser1395Cys) single nucleotide variant Pathogenic rs137854578 5:112175474-112175474 5:112839777-112839777
5 CTNNB1 NM_001904.4(CTNNB1): c.94G> T (p.Asp32Tyr) single nucleotide variant Pathogenic/Likely pathogenic rs28931588 3:41266097-41266097 3:41224606-41224606
6 TP53 NM_000546.5(TP53): c.722C> T (p.Ser241Phe) single nucleotide variant Likely pathogenic rs28934573 17:7577559-7577559 17:7674241-7674241
7 CTNNB1 NM_001904.4(CTNNB1): c.121A> G (p.Thr41Ala) single nucleotide variant Conflicting interpretations of pathogenicity, other rs121913412 3:41266124-41266124 3:41224633-41224633
8 CDK4 NM_000075.4(CDK4): c.625C> T (p.Arg209Cys) single nucleotide variant Conflicting interpretations of pathogenicity rs140644696 12:58144446-58144446 12:57750663-57750663
9 PTCH1 NM_001083602.2(PTCH1): c.2072T> C (p.Phe691Ser) single nucleotide variant Conflicting interpretations of pathogenicity rs547954117 9:98229688-98229688 9:95467406-95467406
10 MSH6 NM_000179.2(MSH6): c.942C> G (p.Ser314Arg) single nucleotide variant Conflicting interpretations of pathogenicity rs150440246 2:48026064-48026064 2:47798925-47798925
11 CTNNB1 NM_001904.4(CTNNB1): c.101G> T (p.Gly34Val) single nucleotide variant Conflicting interpretations of pathogenicity rs28931589 3:41266104-41266104 3:41224613-41224613
12 STK11 NM_000455.4(STK11): c.397G> A (p.Val133Met) single nucleotide variant Uncertain significance rs567769257 19:1219345-1219345 19:1219346-1219346
13 RAF1 NM_002880.3(RAF1): c.226A> G (p.Met76Val) single nucleotide variant Uncertain significance rs730880999 3:12653543-12653543 3:12612044-12612044

Copy number variations for Hepatoblastoma from CNVD:

7
# CNVD ID Chromosom Start End Type Gene Symbol CNVD Disease
1 48501 11 1 52900000 Methylation H19 Hepatoblastoma
2 48504 11 1 52900000 Methylation IGF2 Hepatoblastoma

Expression for Hepatoblastoma

Search GEO for disease gene expression data for Hepatoblastoma.

Pathways for Hepatoblastoma

Pathways related to Hepatoblastoma according to KEGG:

37
# Name Kegg Source Accession
1 Wnt signaling pathway hsa04310

Pathways related to Hepatoblastoma according to GeneCards Suite gene sharing:

(show all 22)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
12.86 TP53 IGF2 HGF CTNNB1 CASP3 APC
2
Show member pathways
12.75 TP53 CTNNB1 CASP3 BIRC5 APC
3
Show member pathways
12.66 TP53 IGF2 HGF CTNNB1 APC
4
Show member pathways
12.6 TP53 HGF CTNNB1 CASP3 APC
5 12.28 TP53 PTCH1 IGF2 HGF CTNNB1 CASP3
6 12.23 TP53 HNF1A CTNNB1 BIRC5 APC
7
Show member pathways
12.15 TP53 PTCH1 GPC3 CTNNB1 APC
8 12.12 PTCH1 CTNNB1 APC AFP
9 12 CTNNB1 BIRC5 APC AFP
10 11.94 TP53 CTNNB1 CASP3 BIRC5 APC
11 11.88 TP53 HGF APC AFP
12
Show member pathways
11.8 TP53 HNF1A CTNNB1 BIRC5 APC
13 11.71 TP53 IGF2 CTNNB1 APC
14 11.64 TP53 CASP3 BIRC5
15 11.56 PTCH1 IGF2 CDKN1C
16 11.48 TP53 PTCH1 IGF2 HGF GPC3 CTNNB1
17 11.44 TP53 CASP3 APC
18 11.36 HNF1A CTNNB1 APC
19 11.31 HNF1A APOA1 AFP
20 11.16 TP53 HNF1A CASP3 BIRC5
21 11.06 HGF CTNNB1 APC
22 10.49 PTCH1 GPC3

GO Terms for Hepatoblastoma

Cellular components related to Hepatoblastoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 beta-catenin destruction complex GO:0030877 8.96 CTNNB1 APC
2 Wnt signalosome GO:1990909 8.62 CTNNB1 APC

Biological processes related to Hepatoblastoma according to GeneCards Suite gene sharing:

(show all 14)
# Name GO ID Score Top Affiliating Genes
1 negative regulation of apoptotic process GO:0043066 9.93 TP53 HGF CTNNB1 CASP3 BIRC5
2 negative regulation of transcription, DNA-templated GO:0045892 9.88 TP53 PTCH1 CTNNB1 CDKN1C BIRC5
3 cytokine-mediated signaling pathway GO:0019221 9.83 TP53 HGF CASP3 BIRC5
4 negative regulation of cell proliferation GO:0008285 9.8 TP53 PTCH1 GPC3 CTNNB1 APC
5 cellular protein metabolic process GO:0044267 9.73 IGF2 GPC3 APOA1 AFP
6 positive regulation of peptidyl-tyrosine phosphorylation GO:0050731 9.63 TP53 IGF2 HGF
7 negative regulation of epithelial cell proliferation GO:0050680 9.54 PTCH1 GPC3 CDKN1C
8 striated muscle cell differentiation GO:0051146 9.51 IGF2 CASP3
9 anterior/posterior axis specification GO:0009948 9.49 GPC3 CTNNB1
10 branching involved in ureteric bud morphogenesis GO:0001658 9.43 PTCH1 GPC3 CTNNB1
11 regulation of mitotic cell cycle GO:0007346 9.33 PTCH1 CDKN1C BIRC5
12 embryonic placenta morphogenesis GO:0060669 9.32 IGF2 CDKN1C
13 negative regulation of cyclin-dependent protein serine/threonine kinase activity GO:0045736 9.13 CDKN1C CASP3 APC
14 cell proliferation involved in metanephros development GO:0072203 8.62 PTCH1 GPC3

Molecular functions related to Hepatoblastoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 cyclin-dependent protein serine/threonine kinase inhibitor activity GO:0004861 8.62 CDKN1C CASP3

Sources for Hepatoblastoma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 TGDB
71 Tocris
72 UMLS
73 UMLS via Orphanet
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