MCID: HPT022
MIFTS: 56

Hepatoblastoma

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

Aliases & Classifications for Hepatoblastoma

MalaCards integrated aliases for Hepatoblastoma:

Name: Hepatoblastoma 12 74 52 36 29 54 6 43 15 17 71

Classifications:



External Ids:

Disease Ontology 12 DOID:687
KEGG 36 H02302
MeSH 43 D018197
NCIt 49 C3728
SNOMED-CT 67 109843000 45024009
ICD10 32 C22.2
UMLS 71 C0206624

Summaries for Hepatoblastoma

NIH Rare Diseases : 52 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 hepatocellular carcinoma, 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 Decreased shRNA abundance (Z-score < -2) and Decreased shRNA abundance (Z-score < -2)

KEGG : 36 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 : 74 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 471)
# Related Disease Score Top Affiliating Genes
1 beckwith-wiedemann syndrome 33.1 TP53 SLC22A18 IGF2 H19 GPC3 CTNNB1
2 hepatocellular carcinoma 32.6 TUG1 TP53 PTCH1 KRT7 IGF2 HNF1A
3 wilms tumor 5 31.3 SLC22A18 IGF2 H19 GPC3 CTNNB1
4 hemihyperplasia, isolated 31.3 IGF2 H19
5 adenoma 31.1 TP53 PLAG1 KRT7 CTNNB1 APC
6 embryonal rhabdomyosarcoma 31.0 TP53 PTCH1 IGF2 H19
7 embryonal sarcoma 30.9 TP53 GPC3 CTNNB1
8 rhabdomyosarcoma 30.9 TP53 SLC22A18 PTCH1 IGF2 H19 CTNNB1
9 hemangioma 30.9 TP53 KRT7 IGF2 CTNNB1
10 neuroblastoma 30.8 TP53 PTCH1 IGF2 H19 CTNNB1 CASP3
11 endodermal sinus tumor 30.8 KRT7 GPC3 AFP
12 adrenal cortical carcinoma 30.8 TP53 IGF2 CTNNB1
13 teratoma 30.7 TP53 KRT7 H19 GPC3 CTNNB1 AFP
14 adrenocortical carcinoma, hereditary 30.7 TP53 IGF2 H19 CTNNB1
15 colorectal adenoma 30.7 TP53 IGF2 CTNNB1 APC
16 li-fraumeni syndrome 30.7 TP53 PTCH1 IGF2 CTNNB1
17 hepatocellular adenoma 30.6 HNF1A GPC3 CTNNB1 AFP
18 osteogenic sarcoma 30.6 TUG1 TP53 IGF2 H19 CASP3
19 fibrolamellar carcinoma 30.5 KRT7 GPC3 CTNNB1 AFP
20 pleomorphic adenoma 30.5 TP53 PLAG1 KRT7 CTNNB1
21 glycogen storage disease 30.5 IGF2 HNF1A HGF CASP3 AFP
22 cystic teratoma 30.4 TP53 KRT7 AFP
23 adenocarcinoma 30.2 TP53 KRT7 HGF H19 CTNNB1 APC
24 intrahepatic cholangiocarcinoma 30.2 TUG1 TP53 KRT7 GPC3 CTNNB1 APC
25 gastrointestinal stromal tumor 30.1 TP53 IGF2 CASP3 APC
26 wilms tumor 1 30.1 TP53 KRT7 IGF2 H19 GPC3 CTNNB1
27 neural tube defects 29.9 TP53 PTCH1 IGF2 HGF H19 CTNNB1
28 medulloblastoma 29.9 TP53 PTCH1 IGF2 HGF H19 CTNNB1
29 cholangiocarcinoma 29.8 TUG1 TP53 KRT7 HGF H19 GPC3
30 lung cancer susceptibility 3 29.6 TUG1 TP53 KRT7 HGF H19 CTNNB1
31 mixed hepatoblastoma 12.5
32 macrotrabecular hepatoblastoma 12.4
33 silver-russell syndrome due to 11p15 microduplication 10.7 IGF2 H19
34 silver-russell syndrome due to an imprinting defect of 11p15 10.7 IGF2 H19
35 vulvar intraepithelial neoplasia 10.7 TP53 CTNNB1
36 calcifying epithelial odontogenic tumor 10.7 TP53 PTCH1
37 beckwith-wiedemann syndrome due to imprinting defect of 11p15 10.7 IGF2 H19
38 hyperplastic polyposis syndrome 10.7 TP53 APC
39 ovarian embryonal carcinoma 10.6 GPC3 AFP
40 marek disease 10.6 TP53 H19
41 esophagus adenocarcinoma 10.6 TP53 CTNNB1 CDO1
42 laryngeal neuroendocrine tumor 10.6 TP53 PTCH1
43 non-gestational ovarian choriocarcinoma 10.6 TP53 AFP
44 connective tissue benign neoplasm 10.6 TP53 PTCH1 CTNNB1
45 pax6-related aniridia 10.6 IGF2 H19 GPC3
46 colonic benign neoplasm 10.6 TP53 CTNNB1 APC
47 adrenal cortex disease 10.6 TP53 IGF2 CTNNB1
48 adult teratoma 10.6 TP53 AFP
49 keratoacanthoma 10.6 TP53 CTNNB1 CASP3
50 liver adenomatosis 10.6 HNF1A CTNNB1

Graphical network of the top 20 diseases related to Hepatoblastoma:



Diseases related to Hepatoblastoma

Symptoms & Phenotypes for Hepatoblastoma

UMLS symptoms related to Hepatoblastoma:


icterus

GenomeRNAi Phenotypes related to Hepatoblastoma according to GeneCards Suite gene sharing:

26 (show all 28)
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Decreased shRNA abundance (Z-score < -2) GR00366-A-114 9.73 AFP
2 Decreased shRNA abundance (Z-score < -2) GR00366-A-120 9.73 AFP
3 Decreased shRNA abundance (Z-score < -2) GR00366-A-177 9.73 APC
4 Decreased shRNA abundance (Z-score < -2) GR00366-A-29 9.73 AFP APC
5 Decreased shRNA abundance (Z-score < -2) GR00366-A-49 9.73 APC
6 Decreased shRNA abundance (Z-score < -2) GR00366-A-63 9.73 AFP
7 Decreased shRNA abundance (Z-score < -2) GR00366-A-65 9.73 AFP
8 Decreased shRNA abundance (Z-score < -2) GR00366-A-71 9.73 KRT7
9 Decreased shRNA abundance (Z-score < -2) GR00366-A-72 9.73 APC
10 Decreased shRNA abundance (Z-score < -2) GR00366-A-74 9.73 APC
11 Decreased shRNA abundance (Z-score < -2) GR00366-A-75 9.73 AFP
12 Increased shRNA abundance (Z-score > 2) GR00366-A-109 9.58 IGF2
13 Increased shRNA abundance (Z-score > 2) GR00366-A-115 9.58 IGF2
14 Increased shRNA abundance (Z-score > 2) GR00366-A-133 9.58 APC
15 Increased shRNA abundance (Z-score > 2) GR00366-A-134 9.58 IGF2
16 Increased shRNA abundance (Z-score > 2) GR00366-A-135 9.58 KRT7
17 Increased shRNA abundance (Z-score > 2) GR00366-A-148 9.58 IGF2
18 Increased shRNA abundance (Z-score > 2) GR00366-A-161 9.58 IGF2
19 Increased shRNA abundance (Z-score > 2) GR00366-A-192 9.58 APC KRT7
20 Increased shRNA abundance (Z-score > 2) GR00366-A-195 9.58 IGF2
21 Increased shRNA abundance (Z-score > 2) GR00366-A-202 9.58 APC
22 Increased shRNA abundance (Z-score > 2) GR00366-A-47 9.58 CTNNB1
23 Increased shRNA abundance (Z-score > 2) GR00366-A-63 9.58 IGF2
24 Increased shRNA abundance (Z-score > 2) GR00366-A-68 9.58 KRT7
25 Increased shRNA abundance (Z-score > 2) GR00366-A-72 9.58 KRT7
26 Increased shRNA abundance (Z-score > 2) GR00366-A-74 9.58 KRT7
27 Increased shRNA abundance (Z-score > 2) GR00366-A-87 9.58 IGF2
28 Increased shRNA abundance (Z-score > 2) GR00366-A-91 9.58 KRT7

MGI Mouse Phenotypes related to Hepatoblastoma:

45 (show all 16)
# Description MGI Source Accession Score Top Affiliating Genes
1 cellular MP:0005384 10.33 APC APOA1 CASP3 CTNNB1 GPC3 HGF
2 cardiovascular system MP:0005385 10.3 APC APOA1 CASP3 CDO1 CTNNB1 GPC3
3 growth/size/body region MP:0005378 10.27 APC CASP3 CDO1 CTNNB1 GPC3 HGF
4 homeostasis/metabolism MP:0005376 10.27 AFP APC APOA1 CASP3 CDO1 CTNNB1
5 endocrine/exocrine gland MP:0005379 10.25 AFP APC APOA1 CASP3 CDO1 CTNNB1
6 craniofacial MP:0005382 10.19 APC CASP3 CDO1 CTNNB1 GPC3 IGF2
7 digestive/alimentary MP:0005381 10.13 APC CASP3 CTNNB1 GPC3 HNF1A IGF2
8 integument MP:0010771 10.13 APC APOA1 CASP3 CTNNB1 GPC3 HNF1A
9 hearing/vestibular/ear MP:0005377 10.02 APC CASP3 CDO1 CTNNB1 PLAG1 PTCH1
10 liver/biliary system MP:0005370 10.02 AFP APC APOA1 CTNNB1 HGF HNF1A
11 limbs/digits/tail MP:0005371 9.95 APC CDO1 CTNNB1 GPC3 IGF2 PTCH1
12 muscle MP:0005369 9.92 APC CASP3 CTNNB1 HNF1A IGF2 PPARA
13 no phenotypic analysis MP:0003012 9.8 CASP3 CTNNB1 HGF HNF1A IGF2 PTCH1
14 renal/urinary system MP:0005367 9.65 APC CASP3 CTNNB1 GPC3 HNF1A IGF2
15 pigmentation MP:0001186 9.63 APC CASP3 CTNNB1 GPC3 PTCH1 TP53
16 reproductive system MP:0005389 9.36 AFP APC CASP3 CDO1 CTNNB1 GPC3

Drugs & Therapeutics for Hepatoblastoma

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

(show top 50) (show all 118)
# 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
Irinotecan Approved, Investigational Phase 3 97682-44-5, 100286-90-6 60838
6
Carboplatin Approved Phase 3 41575-94-4 10339178 38904 498142
7
Gemcitabine Approved Phase 3 95058-81-4 60750
8
Oxaliplatin Approved, Investigational Phase 3 61825-94-3 43805 6857599 5310940 9887054
9
Daunorubicin Approved Phase 2, Phase 3 20830-81-3 30323
10
Cisplatin Approved Phase 3 15663-27-1 2767 441203 84093
11
Vincristine Approved, Investigational Phase 3 57-22-7, 2068-78-2 5978
12
Doxorubicin Approved, Investigational Phase 3 23214-92-8 31703
13
Fluorouracil Approved Phase 3 51-21-8 3385
14
Clotrimazole Approved, Vet_approved Phase 3 23593-75-1 2812
15
Miconazole Approved, Investigational, Vet_approved Phase 3 22916-47-8 4189
16
Sirolimus Approved, Investigational Phase 3 53123-88-9 5284616 6436030 46835353
17
Propofol Approved, Investigational, Vet_approved Phase 2, Phase 3 2078-54-8 4943
18
Rocuronium Approved Phase 2, Phase 3 119302-91-9, 143558-00-3 441290
19
Sevoflurane Approved, Vet_approved Phase 2, Phase 3 28523-86-6 5206
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 Antioxidants Phase 3
25 Antitubercular Agents Phase 3
26 Chelating Agents Phase 3
27 sodium thiosulfate Phase 3
28 Antiviral Agents Phase 3
29 Podophyllotoxin Phase 2, Phase 3 518-28-5
30 Keratolytic Agents Phase 2, Phase 3
31 Anti-Infective Agents Phase 3
32 Anti-Bacterial Agents Phase 3
33 topoisomerase I inhibitors Phase 3
34 Cardiotonic Agents Phase 3
35 3,6-bis(5-chloro-2-piperidyl)-2,5-piperazinedione Phase 3
36
Liposomal doxorubicin Phase 3 31703
37 razoxane Phase 3
38 Hematinics Phase 3
39 Antifungal Agents Phase 3
40 Tubulin Modulators Phase 3
41 Antibiotics, Antitubercular Phase 3
42 Antimetabolites Phase 3
43 Antimitotic Agents Phase 3
44 Anesthetics Phase 2, Phase 3
45 Bromides Phase 2, Phase 3
46 Dermatologic Agents Phase 3
47 Neurotransmitter Agents Phase 3
48 Histamine Antagonists Phase 3
49 Anti-Allergic Agents Phase 3
50
Histamine Phosphate Phase 3 51-74-1 65513

Interventional clinical trials:

(show top 50) (show all 68)
# 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 Liver Tumour Studies - Hepatoblastoma and Hepatocellular Carcinoma Completed NCT00003912 Phase 3 carboplatin;cisplatin;doxorubicin hydrochloride
4 A Randomized Phase III Study of Sodium Thiosulfate for the Prevention of Cisplatin-Induced Ototoxicity in Children Completed NCT00716976 Phase 3 sodium thiosulfate
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 Pazopanib GW786034, NSC# 737754 in Children, Adolescents and Young Adults With Refractory Solid Tumors Completed NCT01956669 Phase 2 Pazopanib GW786034
21 Phase II Study of Arsenic Trioxide in Neuroblastoma and Other Pediatric Solid Tumors Completed NCT00024258 Phase 2 arsenic trioxide
22 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
23 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
24 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)
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 Olaparib in Patients With Tumors Harboring Defects in DNA Damage Repair Genes Recruiting NCT03233204 Phase 2 Olaparib
28 NCI-COG Pediatric MATCH (Molecular Analysis for Therapy Choice) - Phase 2 Subprotocol of AG-120 (Ivosidenib) in Patients With Tumors Harboring IDH1 Mutations Recruiting NCT04195555 Phase 2 Ivosidenib
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 Ensartinib in Patients With Tumors Harboring ALK or ROS1 Genomic Alterations Recruiting NCT03213652 Phase 2 Ensartinib
32 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
33 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
34 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
35 NCI-COG Pediatric MATCH (Molecular Analysis For Therapy Choice)- Phase 2 Subprotocol of LY3023414 in Patients With Solid Tumors Recruiting NCT03213678 Phase 2 Samotolisib
36 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
37 NCI-COG Pediatric MATCH (Molecular Analysis for Therapy Choice) Screening Protocol Recruiting NCT03155620 Phase 2 Ensartinib;Erdafitinib;Larotrectinib;Olaparib;Palbociclib;Samotolisib;Selpercatinib;Selumetinib Sulfate;Tazemetostat;Tipifarnib;Ulixertinib;Vemurafenib
38 NCI-COG Pediatric MATCH (Molecular Analysis for Therapy Choice) - Phase 2 Subprotocol of LOXO-292 in Patients With Tumors Harboring RET Gene Alterations Not yet recruiting NCT04320888 Phase 2 Selpercatinib
39 NCI-COG Pediatric MATCH (Molecular Analysis for Therapy Choice)- Phase 2 Subprotocol of Tipifarnib in Patients With Tumors Harboring HRAS Genomic Alterations Not yet recruiting NCT04284774 Phase 2 Tipifarnib
40 A Phase II Study of Imetelstat (GRN163L, NSC# 754228) in Children With Relapsed or Refractory Solid Tumors Withdrawn NCT02011126 Phase 2 Imetelstat Sodium
41 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
42 A Phase 1 Study Using Simvastatin in Combination With Topotecan and Cyclophosphamide in Relapsed and/or Refractory Pediatric Solid and CNS Tumors Completed NCT02390843 Phase 1 Simvastatin;Cyclophosphamide;Topotecan;Myeloid growth factor
43 Sirolimus in Combination With Metronomic Therapy in Children With Recurrent and Refractory Solid Tumors: A Phase I Study Completed NCT01331135 Phase 1 sirolimus
44 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
45 A Phase I Study of Vorinostat in Combination With Vincristine, Irinotecan, and Temozolomide in Children, Adolescents, and Young Adults With Relapsed or Refractory Solid Tumors and CNS Malignancies Recruiting NCT04308330 Phase 1 Vorinostat
46 Phase I Study of EGFR806 CAR T Cell Immunotherapy for Recurrent/Refractory Solid Tumors in Children and Young Adults Recruiting NCT03618381 Phase 1
47 A Phase I Study of Cytoreductive Surgery and Hyperthermic Intrathoracic Pleural Chemotherapy (HITC) With Escalating Doses for Children and Adolescents With Unilateral Pleural Malignancy Recruiting NCT01998529 Phase 1 Cisplatin;Amifostine
48 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
49 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®)
50 Pilot Pharmacokinetic Study of VAL-413 in Patients With Recurrent Pediatric Solid Tumors Not yet recruiting NCT04337177 Phase 1 VAL-413;Temozolomide

Search NIH Clinical Center for Hepatoblastoma

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:

40
Liver, Fetal Liver, Kidney, Lung, Brain, T Cells, Bone

Publications for Hepatoblastoma

Articles related to Hepatoblastoma:

(show top 50) (show all 3299)
# Title Authors PMID Year
1
Hepatocyte growth factor protects hepatoblastoma cells from chemotherapy-induced apoptosis by AKT activation. 61 54
20372801 2010
2
Involvement of mTOR and survivin inhibition in tamoxifen-induced apoptosis in human hepatoblastoma cell line HepG2. 61 54
19931998 2010
3
Establishment and characterization of a cancer cell line derived from an aggressive childhood liver tumor. 61 54
19637320 2009
4
Predictive power of pretreatment prognostic factors in children with hepatoblastoma: a report from the Children's Oncology Group. 61 54
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. 54 61
20210251 2009
6
Regulation of the human SLC25A20 expression by peroxisome proliferator-activated receptor alpha in human hepatoblastoma cells. 61 54
19748481 2009
7
Regulation of expression of apolipoprotein A-I by selenium status in human liver hepatoblastoma cells. 54 61
19294445 2009
8
Thyroid hormones act indirectly to increase sex hormone-binding globulin production by liver via hepatocyte nuclear factor-4alpha. 54 61
19336534 2009
9
Histologic subtypes of hepatoblastoma are characterized by differential canonical Wnt and Notch pathway activation in DLK+ precursors. 61 54
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. 61 54
19228886 2009
11
Peroxisome-proliferator receptor gamma represses hepatic sex hormone-binding globulin expression. 54 61
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. 54 61
18602814 2009
13
[Successful treatment of second hepatoblastoma relapse--case report]. 54 61
19780340 2009
14
Hepatic stem-like phenotype and interplay of Wnt/beta-catenin and Myc signaling in aggressive childhood liver cancer. 54 61
19061838 2008
15
Loss of imprinting of IGF2 correlates with hypermethylation of the H19 differentially methylated region in hepatoblastoma. 61 54
19034281 2008
16
Rosuvastatin selectively stimulates apolipoprotein A-I but not apolipoprotein A-II synthesis in Hep G2 cells. 61 54
18555840 2008
17
Mesenchymal hamartoma of the liver mimicking hepatoblastoma. 54 61
18525464 2008
18
Diagnostic value of lectin reactive alpha-fetoprotein for neoinfantile hepatic tumors and malignant germ cell tumors: preliminary study. 54 61
18525461 2008
19
The expression of Bcl-XL, Bcl-XS and p27Kip1 in topotecan-induced apoptosis in hepatoblastoma HepG2 cell line. 54 61
18568767 2008
20
Hepatoblastoma with a low serum alpha-fetoprotein level at diagnosis: the SIOPEL group experience. 61 54
18166449 2008
21
Variable antigen expression in hepatoblastomas. 61 54
18227729 2008
22
Combining intracellular and secreted bioluminescent reporter proteins for multicolor cell-based assays. 54 61
18264589 2008
23
Expression of glypican 3 in hepatoblastoma: an immunohistochemical study of 65 cases. 54 61
17949790 2008
24
Sporadic childhood hepatoblastomas show activation of beta-catenin, mismatch repair defects and p53 mutations. 61 54
17962810 2008
25
Monosaccharide-induced lipogenesis regulates the human hepatic sex hormone-binding globulin gene. 54 61
17992261 2007
26
Pioglitazone stimulates apolipoprotein A-I production without affecting HDL removal in HepG2 cells: involvement of PPAR-alpha. 54 61
17872455 2007
27
Erythropoietin receptor presence in hepatoblastoma: a possible link to increased incidence of hepatoblastoma in very low birthweight infants. 54 61
16937361 2007
28
Promoter hypermethylation of the RASSF1A gene predicts the poor outcome of patients with hepatoblastoma. 54 61
16937357 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. 61 54
17372916 2007
30
[Application of the human hepatoblastoma cell lines inducibly expressing peroxisome proliferator-activated receptors (PPARs)]. 54 61
17666873 2007
31
A novel function of capsaicin-sensitive TRPV1 channels: involvement in cell migration. 61 54
17184838 2007
32
Further delineation of the phenotype resulting from BRAF or MEK1 germline mutations helps differentiate cardio-facio-cutaneous syndrome from Costello syndrome. 54 61
17551924 2007
33
Mesenchymal hamartoma of the liver associated with features of Beckwith-Wiedemann syndrome and high serum alpha-fetoprotein levels. 54 61
17535089 2007
34
siRNA-mediated Bcl-2 and Bcl-xl gene silencing sensitizes human hepatoblastoma cells to chemotherapeutic drugs. 61 54
17439414 2007
35
PLAG1, the prototype of the PLAG gene family: versatility in tumour development (review). 54 61
17332914 2007
36
Ornithine transcarbamylase and arginase I deficiency are responsible for diminished urea cycle function in the human hepatoblastoma cell line HepG2. 54 61
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? 54 61
16317745 2006
38
Signaling pathway of insulin-like growth factor-II as a target of molecular therapy for hepatoblastoma. 54 61
17072986 2006
39
Effective treatment of tumors with strong beta-catenin/T-cell factor activity by transcriptionally targeted oncolytic herpes simplex virus vector. 61 54
17047077 2006
40
Identification of human low-density lipoprotein receptor as a novel target gene regulated by liver X receptor alpha. 61 54
16920108 2006
41
The hook effect: a need for constant vigilance. 61 54
16824284 2006
42
In vitro RNA interference against beta-catenin inhibits the proliferation of pediatric hepatic tumors. 61 54
16465377 2006
43
Integrating the diagnosis of childhood malignancies. 54 61
17163162 2006
44
Thrombopoietin stimulates migration and activates multiple signaling pathways in hepatoblastoma cells. 54 61
16150872 2006
45
The glypican 3 oncofetal protein is a promising diagnostic marker for hepatocellular carcinoma. 61 54
15920546 2005
46
Hepatoblastoma in a child with progressive familial intrahepatic cholestasis. 61 54
16269056 2005
47
Iron and insulin resistance. 54 61
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. 61 54
16253722 2005
49
Second case of hepatoblastoma in a young patient with Simpson-Golabi-Behmel syndrome. 61 54
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. 54 61
16197558 2005

Variations for Hepatoblastoma

ClinVar genetic disease variations for Hepatoblastoma:

6 (show all 13) ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎
# Gene Name Type Significance ClinVarId dbSNP ID GRCh37 Pos GRCh38 Pos
1 CTNNB1 NM_001904.4(CTNNB1):c.84_95del (p.Gln28_Asp32delinsHis)deletion other 438756 rs1553630102 3:41266086-41266097 3:41224595-41224606
2 ARID1A NM_006015.6(ARID1A):c.6259G>A (p.Gly2087Arg)SNV other 438750 rs1553153748 1:27106648-27106648 1:26780157-26780157
3 MN1 NM_002430.3(MN1):c.912_914GCA[5] (p.Gln309del)short repeat other 438776 rs747503495 22:28195603-28195605 22:27799615-27799617
4 APC NM_000038.6(APC):c.4183A>T (p.Ser1395Cys)SNV Pathogenic 817 rs137854578 5:112175474-112175474 5:112839777-112839777
5 CTNNB1 NM_001904.4(CTNNB1):c.94G>T (p.Asp32Tyr)SNV Pathogenic/Likely pathogenic 17581 rs28931588 3:41266097-41266097 3:41224606-41224606
6 TP53 NM_001126112.2(TP53):c.722C>T (p.Ser241Phe)SNV Likely pathogenic 12359 rs28934573 17:7577559-7577559 17:7674241-7674241
7 CTNNB1 NM_001904.4(CTNNB1):c.121A>G (p.Thr41Ala)SNV Conflicting interpretations of pathogenicity, other 17580 rs121913412 3:41266124-41266124 3:41224633-41224633
8 CTNNB1 NM_001904.4(CTNNB1):c.101G>T (p.Gly34Val)SNV Conflicting interpretations of pathogenicity 17582 rs28931589 3:41266104-41266104 3:41224613-41224613
9 CDK4 NM_000075.4(CDK4):c.625C>T (p.Arg209Cys)SNV Conflicting interpretations of pathogenicity 135825 rs140644696 12:58144446-58144446 12:57750663-57750663
10 MSH6 NM_000179.2(MSH6):c.942C>G (p.Ser314Arg)SNV Conflicting interpretations of pathogenicity 229741 rs150440246 2:48026064-48026064 2:47798925-47798925
11 PTCH1 NM_000264.5(PTCH1):c.2270T>C (p.Phe757Ser)SNV Conflicting interpretations of pathogenicity 237468 rs547954117 9:98229688-98229688 9:95467406-95467406
12 RAF1 NM_001354689.3(RAF1):c.226A>G (p.Met76Val)SNV Uncertain significance 181507 rs730880999 3:12653543-12653543 3:12612044-12612044
13 STK11 NM_000455.4(STK11):c.397G>A (p.Val133Met)SNV Uncertain significance 182906 rs567769257 19:1219345-1219345 19:1219346-1219346

Copy number variations for Hepatoblastoma from CNVD:

7
# CNVD ID Chromosome 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:

36
# Name Kegg Source Accession
1 Wnt signaling pathway hsa04310

Pathways related to Hepatoblastoma according to GeneCards Suite gene sharing:

(show all 18)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
12.79 TP53 IGF2 HGF CTNNB1 CASP3 APC
2
Show member pathways
12.62 TP53 IGF2 HGF CTNNB1 APC
3
Show member pathways
12.55 TP53 HGF CTNNB1 CASP3 APC
4
Show member pathways
12.31 TP53 GPC3 CTNNB1 APC
5 12.27 TP53 HNF1A CTNNB1 APC
6 12.22 TP53 PTCH1 IGF2 HGF CTNNB1 CASP3
7
Show member pathways
12.16 TP53 PTCH1 CTNNB1 APC
8 12.07 PTCH1 CTNNB1 APC AFP
9 11.95 TP53 CTNNB1 CASP3 APC
10 11.74 TP53 HGF APC AFP
11 11.5 TP53 IGF2 CTNNB1 APC
12 11.48 TP53 PTCH1 IGF2 HGF GPC3 CTNNB1
13 11.4 TP53 CASP3 APC
14 11.34 TP53 HNF1A CASP3
15 11.24 HNF1A CTNNB1 APC
16 11.19 HNF1A APOA1 AFP
17 10.91 HGF CTNNB1 APC
18 10.45 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 11)
# Name GO ID Score Top Affiliating Genes
1 negative regulation of transcription by RNA polymerase II GO:0000122 9.93 TP53 PTCH1 PPARA IGF2 HNF1A CTNNB1
2 positive regulation of transcription by RNA polymerase II GO:0045944 9.87 TP53 PPARA PLAG1 IGF2 HNF1A HGF
3 heart development GO:0007507 9.78 TP53 PPARA CTNNB1 CASP3
4 negative regulation of cell proliferation GO:0008285 9.77 TP53 PTCH1 GPC3 CTNNB1 APC
5 in utero embryonic development GO:0001701 9.73 TP53 PTCH1 IGF2 CTNNB1
6 cellular protein metabolic process GO:0044267 9.71 IGF2 GPC3 APOA1 AFP
7 positive regulation of peptidyl-tyrosine phosphorylation GO:0050731 9.67 TP53 IGF2 HGF
8 positive regulation of neuron apoptotic process GO:0043525 9.43 TP53 CTNNB1 CASP3
9 response to drug GO:0042493 9.35 TP53 PTCH1 CTNNB1 CASP3 APOA1
10 branching involved in ureteric bud morphogenesis GO:0001658 9.33 PTCH1 GPC3 CTNNB1
11 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 MDM2/MDM4 family protein binding GO:0097371 8.62 TP53 PPARA

Sources for Hepatoblastoma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
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
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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