MCID: MLG145
MIFTS: 39

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 MAPK Signaling: Mitogen Stimulation Pathway and AKT Signaling Pathway. The drugs Trametinib and Durvalumab have been mentioned in the context of this disorder. Affiliated tissues include endothelial, testes and liver, and related phenotypes are immune system and integument

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 top 50) (show all 56)
# Related Disease Score Top Affiliating Genes
1 hemangioendothelioma 31.2 KDR FLT4 CAMTA1
2 epithelioid hemangioendothelioma 30.0 YAP1 WWTR1 TFE3 KDR CAMTA1
3 hemangioma of spleen 10.2 SMUG1 FLT4
4 anthracosilicosis 10.2 FOLH1 SMUG1
5 conventional angiosarcoma 10.2 CAMTA1 CD34
6 nodular prostate 10.2 FOLH1 KDR
7 hemangioma, capillary infantile 10.2 FLT4 KDR
8 hobnail hemangioma 10.2 CD34 FLT4
9 tibial adamantinoma 10.2 SMUG1 CD34
10 interstitial emphysema 10.1 CD34 FLT4
11 spleen angiosarcoma 10.1 SMUG1 CD34
12 lymphangioma 10.1 KDR FLT4
13 sternum cancer 10.1 SMUG1 CD34
14 alveoli adenoma 10.1 CD34 FLT4
15 bone epithelioid hemangioma 10.1 CD34 SMUG1
16 breast leiomyosarcoma 10.1 CD34 SMUG1
17 eccrine adenocarcinoma 10.1 CD34 SMUG1
18 breast angiosarcoma 10.1 CD34 SMUG1
19 ovarian angiosarcoma 10.1 MYC CD34
20 ovary sarcoma 10.1 MYC CD34
21 senile angioma 10.1 CD34 KDR
22 adult lymphoma 10.1 SMUG1 MYC
23 venous hemangioma 10.1 MYC CD34
24 pediatric lymphoma 10.1 CD34 SMUG1
25 lymphatic system disease 10.0 SMUG1 FLT4
26 lymphatic system cancer 10.0 MYC SMUG1
27 gaucher's disease 10.0
28 adenocarcinoma 10.0
29 infiltrating lipoma 10.0
30 fibrosarcoma of bone 10.0 KDR MYC
31 nasopharyngeal disease 10.0 SMUG1 MYC
32 hemangioma 9.9 KDR FLT4 CD34
33 angiosarcoma 9.9 MYC KDR FLT4
34 reproductive system disease 9.9 SMUG1 MYC FOLH1
35 kaposiform hemangioendothelioma 9.9 KDR FLT4 CD34
36 connective tissue cancer 9.9 SMUG1 CD34
37 xanthogranulomatous pyelonephritis 9.9 TFE3 SMUG1
38 endocrine gland cancer 9.9 SMUG1 MYC KDR
39 leukocyte disease 9.9 SMUG1 MYC CD34
40 pharynx cancer 9.9 SMUG1 MYC
41 myeloma, multiple 9.8 CD34 MYC SMUG1
42 muscle cancer 9.8 TFE3 CD34
43 vascular cancer 9.8 KDR FLT4 CD34 CAMTA1
44 sarcomatoid squamous cell skin carcinoma 9.7 TFE3 CD34 CAMTA1
45 lymphoma, non-hodgkin, familial 9.7 CD34 MYC SMUG1
46 cervix disease 9.7 SMUG1 MYC
47 hematologic cancer 9.7 CD34 KDR MYC
48 gastric adenocarcinoma 9.7 YAP1 SMUG1 MYC KDR
49 lymphangiosarcoma 9.7 SMUG1 MYC FLT4 CD34
50 muscular disease 9.6 CD34 SMUG1 TFE3

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 immune system MP:0005387 9.91 CD34 FLT4 KDR MYC TFE3 WWTR1
2 integument MP:0010771 9.8 CD34 FLT4 KDR MYC TFE3 YAP1
3 mortality/aging MP:0010768 9.76 CAMTA1 FLT4 KDR MYC SMUG1 TFE3
4 liver/biliary system MP:0005370 9.65 FLT4 KDR MYC WWTR1 YAP1
5 muscle MP:0005369 9.35 FLT4 KDR MYC WWTR1 YAP1
6 neoplasm MP:0002006 9.02 CAMTA1 CD34 MYC SMUG1 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
Durvalumab Approved, Investigational Phase 2 1428935-60-7
3
Pembrolizumab Approved Phase 2 1374853-91-4
4
Gemcitabine Approved Phase 2 95058-81-4 60750
5
Tremelimumab Investigational Phase 2 745013-59-6
6 Protein Kinase Inhibitors Phase 2
7 Antibodies Phase 2
8 Immunologic Factors Phase 2
9 Immunoglobulins Phase 2
10 Antibodies, Monoclonal Phase 2
11 Antineoplastic Agents, Immunological Phase 2
12 Anti-Infective Agents Phase 2
13 Antimetabolites Phase 2
14 Immunosuppressive Agents Phase 2
15 Antiviral Agents Phase 2
16 Antimetabolites, Antineoplastic Phase 2

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Eribulin in Angiosarcoma and Epithelioid Hemangioendothelioma (EHE) Recruiting NCT03331250 Phase 2 Eribulin
2 Trametinib in Treating Patients With Epithelioid Hemangioendothelioma That Is Metastatic, Locally Advanced, or Cannot Be Removed by Surgery Recruiting NCT03148275 Phase 2 Trametinib
3 Multi-Arm Study to Test the Efficacy of Immunotherapeutic Agents in Multiple Sarcoma Subtypes Active, not recruiting NCT02815995 Phase 2 Durvalumab;Tremelimumab
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 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

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, Testes, Liver, Bone, Thyroid, Lung, Colon

Publications for Malignant Epithelioid Hemangioendothelioma

Articles related to Malignant Epithelioid Hemangioendothelioma:

(show all 25)
# Title Authors Year
1
Molecular imaging of glutamate-carboxypeptidase II (prostate-specific membrane antigen) in malignant epithelioid hemangioendothelioma. ( 26260651 )
2015
2
Malignant epithelioid hemangioendothelioma of the adrenal gland treated by laparoscopic excision. ( 25733308 )
2015
3
Primitive pulmonary "malignant epithelioid hemangioendothelioma" versus epithelioid angiosarcoma. A case report and review of the literature. ( 25073215 )
2014
4
Malignant epithelioid hemangioendothelioma: a case report. ( 23599886 )
2013
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 of the Lip: A Case Report and Comprehensive Literature Review. ( 24268966 )
2013
7
Malignant epithelioid hemangioendothelioma of the liver successfully treated with Sorafenib. ( 22826791 )
2012
8
Serial bone scintigraphy in a case of malignant epithelioid hemangioendothelioma. ( 22391717 )
2012
9
Malignant epithelioid hemangioendothelioma of the lower extremity: staging with F-18 FDG PET/CT. ( 21637069 )
2011
10
A case of pulmonary malignant epithelioid hemangioendothelioma misdiagnosed as adenocarcinoma by fine needle aspiration cytology. ( 21994192 )
2011
11
Malignant epithelioid hemangioendothelioma of the liver successfully treated with pegylated liposomal doxorubicin. ( 21788568 )
2011
12
Malignant epithelioid hemangioendothelioma of the bladder: the first case report in a child. ( 19573676 )
2009
13
Malignant epithelioid hemangioendothelioma with spindle phenotype: report of an unusual case diagnosed by fine needle aspiration cytology. ( 19068679 )
2008
14
Hepatic malignant epithelioid hemangioendothelioma: a case report and review of the literature. ( 18274433 )
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
11.76 FLT4 KDR MYC
2 11.65 FLT4 KDR MYC
3 11.52 MYC WWTR1 YAP1
4 11.09 FLT4 KDR WWTR1 YAP1
5 10.83 WWTR1 YAP1
6 10.77 FLT4 KDR MYC
7 10.58 WWTR1 YAP1

GO Terms for Malignant Epithelioid Hemangioendothelioma

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

(show all 13)
# 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 positive regulation of cell proliferation GO:0008284 9.55 FLT4 KDR MYC WWTR1 YAP1
3 negative regulation of gene expression GO:0010629 9.52 CD34 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 endothelium development GO:0003158 9.32 CD34 KDR
11 regulation of metanephric nephron tubule epithelial cell differentiation GO:0072307 9.26 WWTR1 YAP1
12 heart process GO:0003015 8.96 WWTR1 YAP1
13 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 growth factor binding GO:0019838 9.16 FLT4 KDR
2 activating transcription factor binding GO:0033613 8.96 MYC YAP1
3 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
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45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
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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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