MCID: MLG145
MIFTS: 38

Malignant Epithelioid Hemangioendothelioma

Categories: Cancer diseases, Cardiovascular diseases, Fetal diseases, Rare diseases

Aliases & Classifications for Malignant Epithelioid Hemangioendothelioma

MalaCards integrated aliases for Malignant Epithelioid Hemangioendothelioma:

Name: Malignant Epithelioid Hemangioendothelioma 12 15
Epithelioid Hemangioendothelioma, Malignant 74

Classifications:



External Ids:

Disease Ontology 12 DOID:0080190
NCIt 51 C3800
UMLS 74 C0334538

Summaries for Malignant Epithelioid Hemangioendothelioma

Disease Ontology : 12 A malignant hemangioma characterized by the presence of epithelioid endothelial cells. The neoplastic cells are arranged in cords and nests, which are embedded in a myxoid to hyalinized stroma.

MalaCards based summary : Malignant Epithelioid Hemangioendothelioma, also known as epithelioid hemangioendothelioma, malignant, is related to hemangioendothelioma and epithelioid hemangioendothelioma. An important gene associated with Malignant Epithelioid Hemangioendothelioma is CAMTA1 (Calmodulin Binding Transcription Activator 1), and among its related pathways/superpathways are Immune response IFN alpha/beta signaling pathway and MAPK Signaling: Mitogen Stimulation Pathway. The drugs Trametinib and Pembrolizumab have been mentioned in the context of this disorder. Affiliated tissues include endothelial, liver and bone, and related phenotypes are growth/size/body region and immune system

Related Diseases for Malignant Epithelioid Hemangioendothelioma

Diseases in the Epithelioid Hemangioendothelioma family:

Malignant Epithelioid Hemangioendothelioma

Diseases related to Malignant Epithelioid Hemangioendothelioma via text searches within MalaCards or GeneCards Suite gene sharing:

(show all 35)
# Related Disease Score Top Affiliating Genes
1 hemangioendothelioma 31.1 CAMTA1 FLT4 KDR
2 epithelioid hemangioendothelioma 29.9 CAMTA1 KDR TFE3 WWTR1 YAP1
3 angiosarcoma 29.9 FLT4 KDR MYC
4 conventional angiosarcoma 10.1 CAMTA1 CD34
5 nodular prostate 10.1 FOLH1 KDR
6 hobnail hemangioma 10.1 CD34 FLT4
7 hemangioma, capillary infantile 10.1 FLT4 KDR
8 interstitial emphysema 10.1 CD34 FLT4
9 lymphangioma 10.1 FLT4 KDR
10 alveoli adenoma 10.1 CD34 FLT4
11 senile angioma 10.0 CD34 KDR
12 ovary sarcoma 10.0 CD34 MYC
13 ovarian angiosarcoma 10.0 CD34 MYC
14 lipomatosis, multiple 10.0
15 pleomorphic lipoma 10.0
16 gaucher's disease 10.0
17 adenocarcinoma 10.0
18 infiltrating lipoma 10.0
19 venous hemangioma 10.0 CD34 MYC
20 fibrosarcoma of bone 10.0 KDR MYC
21 hemangioma 9.9 CD34 FLT4 KDR
22 kaposiform hemangioendothelioma 9.9 CD34 FLT4 KDR
23 lymphangiosarcoma 9.8 CD34 FLT4 MYC
24 gastrointestinal system cancer 9.8 CD34 KDR MYC
25 hematologic cancer 9.7 CD34 KDR MYC
26 vascular cancer 9.7 CAMTA1 CD34 FLT4 KDR
27 sarcomatoid squamous cell skin carcinoma 9.7 CAMTA1 CD34 TFE3
28 peripheral nervous system neoplasm 9.7 CD34 MYC
29 muscle cancer 9.7 CD34 TFE3
30 gastric adenocarcinoma 9.6 KDR MYC YAP1
31 large intestine cancer 9.6 CD34 KDR MYC
32 renal cell carcinoma, nonpapillary 9.6 FLT4 KDR TFE3
33 hepatocellular carcinoma 9.3 CAMTA1 CD34 KDR MYC YAP1
34 liver angiosarcoma 9.1 CAMTA1 CD34 TFE3 WWTR1 YAP1
35 malignant hemangioma 8.1 CAMTA1 CD34 FLT4 FOLH1 KDR MYC

Graphical network of the top 20 diseases related to Malignant Epithelioid Hemangioendothelioma:



Diseases related to Malignant Epithelioid Hemangioendothelioma

Symptoms & Phenotypes for Malignant Epithelioid Hemangioendothelioma

MGI Mouse Phenotypes related to Malignant Epithelioid Hemangioendothelioma:

47
# Description MGI Source Accession Score Top Affiliating Genes
1 growth/size/body region MP:0005378 9.87 CAMTA1 FLT4 KDR MYC TFE3 WWTR1
2 immune system MP:0005387 9.8 CD34 FLT4 KDR MYC TFE3 WWTR1
3 integument MP:0010771 9.63 CD34 FLT4 KDR MYC TFE3 YAP1
4 liver/biliary system MP:0005370 9.35 FLT4 KDR MYC WWTR1 YAP1
5 muscle MP:0005369 9.02 FLT4 KDR MYC WWTR1 YAP1

Drugs & Therapeutics for Malignant Epithelioid Hemangioendothelioma

Drugs for Malignant Epithelioid Hemangioendothelioma (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 16)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Trametinib Approved Phase 2 871700-17-3 11707110
2
Pembrolizumab Approved Phase 2 1374853-91-4
3
Gemcitabine Approved Phase 2 95058-81-4 60750
4
Durvalumab Approved, Investigational Phase 2 1428935-60-7
5
Tremelimumab Investigational Phase 2 745013-59-6
6 Protein Kinase Inhibitors Phase 2
7 Antineoplastic Agents, Immunological Phase 2
8 Anti-Infective Agents Phase 2
9 Immunologic Factors Phase 2
10 Antiviral Agents Phase 2
11 Immunosuppressive Agents Phase 2
12 Antimetabolites, Antineoplastic Phase 2
13 Antimetabolites Phase 2
14 Immunoglobulins Phase 2
15 Antibodies Phase 2
16 Antibodies, Monoclonal Phase 2

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Vismodegib and Gamma-Secretase/Notch Signalling Pathway Inhibitor RO4929097 in Treating Patients With Advanced or Metastatic Sarcoma Completed NCT01154452 Phase 1, Phase 2 Gamma-Secretase Inhibitor RO4929097;Vismodegib
2 Eribulin in Angiosarcoma and Epithelioid Hemangioendothelioma (EHE) Recruiting NCT03331250 Phase 2 Eribulin
3 Trametinib in Treating Patients With Epithelioid Hemangioendothelioma That Is Metastatic, Locally Advanced, or Cannot Be Removed by Surgery Recruiting NCT03148275 Phase 2 Trametinib
4 A Study of Epacadostat, an IDO1 Inhibitor, in Combination With Pembrolizumab in Patients With Metastatic and/or Locally Advanced Sarcoma Recruiting NCT03414229 Phase 2 Epacadostat;Pembrolizumab
5 Gemcitabine Hydrochloride With or Without Pazopanib Hydrochloride in Treating Patients With Refractory Soft Tissue Sarcoma Recruiting NCT01532687 Phase 2 Gemcitabine Hydrochloride;Pazopanib Hydrochloride
6 Multi-Arm Study to Test the Efficacy of Immunotherapeutic Agents in Multiple Sarcoma Subtypes Active, not recruiting NCT02815995 Phase 2 Durvalumab;Tremelimumab

Search NIH Clinical Center for Malignant Epithelioid Hemangioendothelioma

Genetic Tests for Malignant Epithelioid Hemangioendothelioma

Anatomical Context for Malignant Epithelioid Hemangioendothelioma

MalaCards organs/tissues related to Malignant Epithelioid Hemangioendothelioma:

42
Endothelial, Liver, Bone, Testes, Thyroid, Lung, Colon

Publications for Malignant Epithelioid Hemangioendothelioma

Articles related to Malignant Epithelioid Hemangioendothelioma:

(show all 25)
# Title Authors Year
1
Malignant epithelioid hemangioendothelioma of the adrenal gland treated by laparoscopic excision. ( 25733308 )
2015
2
Molecular imaging of glutamate-carboxypeptidase II (prostate-specific membrane antigen) in malignant epithelioid hemangioendothelioma. ( 26260651 )
2015
3
Primitive pulmonary "malignant epithelioid hemangioendothelioma" versus epithelioid angiosarcoma. A case report and review of the literature. ( 25073215 )
2014
4
Malignant epithelioid hemangioendothelioma of the lip: a case report and comprehensive literature review. ( 24268966 )
2014
5
Malignant epithelioid hemangioendothelioma progressing after chemotherapy and Interferon treatment: a case presentation and a brief review of the literature. ( 23575093 )
2013
6
Malignant epithelioid hemangioendothelioma: a case report. ( 23599886 )
2013
7
Serial bone scintigraphy in a case of malignant epithelioid hemangioendothelioma. ( 22391717 )
2012
8
Malignant epithelioid hemangioendothelioma of the liver successfully treated with Sorafenib. ( 22826791 )
2012
9
Malignant epithelioid hemangioendothelioma of the lower extremity: staging with F-18 FDG PET/CT. ( 21637069 )
2011
10
Malignant epithelioid hemangioendothelioma of the liver successfully treated with pegylated liposomal doxorubicin. ( 21788568 )
2011
11
A case of pulmonary malignant epithelioid hemangioendothelioma misdiagnosed as adenocarcinoma by fine needle aspiration cytology. ( 21994192 )
2011
12
Malignant epithelioid hemangioendothelioma of the bladder: the first case report in a child. ( 19573676 )
2009
13
Hepatic malignant epithelioid hemangioendothelioma: a case report and review of the literature. ( 18274433 )
2008
14
Malignant epithelioid hemangioendothelioma with spindle phenotype: report of an unusual case diagnosed by fine needle aspiration cytology. ( 19068679 )
2008
15
An authentic malignant epithelioid hemangioendothelioma of the thyroid: a case report and review of the literature. ( 16405412 )
2005
16
Malignant epithelioid hemangioendothelioma presenting as an aneurysm of the superficial palmar arch: a case report. ( 12132094 )
2002
17
Establishment and characterization of a malignant epithelioid hemangioendothelioma from mouse thyroid tumor. ( 7670562 )
1995
18
Malignant epithelioid hemangioendothelioma of the tibia associated with a bone infarct in a patient who had Gaucher disease. A case report. ( 7744904 )
1995
19
Malignant epithelioid hemangioendothelioma arising in an intramuscular lipoma. ( 8431858 )
1993
20
Scintigraphic features of malignant epithelioid hemangioendothelioma. ( 2766632 )
1989
21
Malignant epithelioid hemangioendothelioma at the site of a hip prosthesis. ( 3381668 )
1988
22
Malignant epithelioid hemangioendothelioma of the colon. Report of a case. ( 3622180 )
1987
23
Lung metastasis of malignant epithelioid hemangioendothelioma mimicking a primary intravascular bronchioalveolar tumor. A histologic, ultrastructural, and immunohistochemical study. ( 2579721 )
1985
24
Malignant epithelioid hemangioendothelioma of the liver in young women. Relationship to oral contraceptive use. ( 3904492 )
1985
25
Malignant epithelioid hemangioendothelioma of the liver, spreading through the hepatic veins. ( 6437065 )
1984

Variations for Malignant Epithelioid Hemangioendothelioma

Expression for Malignant Epithelioid Hemangioendothelioma

Search GEO for disease gene expression data for Malignant Epithelioid Hemangioendothelioma.

Pathways for Malignant Epithelioid Hemangioendothelioma

Pathways related to Malignant Epithelioid Hemangioendothelioma according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Show member pathways
11.82 FLT4 KDR MYC
2
Show member pathways
11.76 FLT4 KDR MYC
3 11.65 FLT4 KDR MYC
4 11.52 MYC WWTR1 YAP1
5 11.09 FLT4 KDR WWTR1 YAP1
6 10.83 WWTR1 YAP1
7 10.77 FLT4 KDR MYC
8 10.58 WWTR1 YAP1

GO Terms for Malignant Epithelioid Hemangioendothelioma

Cellular components related to Malignant Epithelioid Hemangioendothelioma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 cytoplasm GO:0005737 9.23 CAMTA1 CD34 FLT4 FOLH1 KDR MYC

Biological processes related to Malignant Epithelioid Hemangioendothelioma according to GeneCards Suite gene sharing:

(show all 14)
# Name GO ID Score Top Affiliating Genes
1 positive regulation of transcription by RNA polymerase II GO:0045944 9.83 CAMTA1 MYC TFE3 WWTR1 YAP1
2 negative regulation of gene expression GO:0010629 9.7 CD34 KDR MYC
3 positive regulation of endothelial cell migration GO:0010595 9.54 FLT4 KDR
4 cellular response to vascular endothelial growth factor stimulus GO:0035924 9.51 FLT4 KDR
5 regulation of canonical Wnt signaling pathway GO:0060828 9.49 WWTR1 YAP1
6 ERK1 and ERK2 cascade GO:0070371 9.48 KDR MYC
7 hippo signaling GO:0035329 9.46 WWTR1 YAP1
8 vascular endothelial growth factor signaling pathway GO:0038084 9.4 FLT4 KDR
9 positive regulation of vasculogenesis GO:2001214 9.37 CD34 KDR
10 positive regulation of cell proliferation GO:0008284 9.35 FLT4 KDR MYC WWTR1 YAP1
11 endothelium development GO:0003158 9.32 CD34 KDR
12 regulation of metanephric nephron tubule epithelial cell differentiation GO:0072307 9.26 WWTR1 YAP1
13 heart process GO:0003015 9.16 WWTR1 YAP1
14 tissue homeostasis GO:0001894 8.8 CD34 WWTR1 YAP1

Molecular functions related to Malignant Epithelioid Hemangioendothelioma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 transmembrane receptor protein tyrosine kinase activity GO:0004714 9.26 FLT4 KDR
2 growth factor binding GO:0019838 9.16 FLT4 KDR
3 activating transcription factor binding GO:0033613 8.96 MYC YAP1
4 vascular endothelial growth factor-activated receptor activity GO:0005021 8.62 FLT4 KDR

Sources for Malignant Epithelioid Hemangioendothelioma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
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