MCID: HMN016
MIFTS: 43

Hemangioendothelioma

Categories: Rare diseases

Aliases & Classifications for Hemangioendothelioma

MalaCards integrated aliases for Hemangioendothelioma:

Name: Hemangioendothelioma 49 51 69

Classifications:



External Ids:

UMLS 69 C0018915

Summaries for Hemangioendothelioma

NIH Rare Diseases : 49 The term hemangioendothelioma describes several types of vascular neosplasms and includes both non-cancerous (benign) and cancerous (malignant) growths. The term has also been applied to those that show "borderline" behavior, intermediate between entirely benign hemangiomas and highly malignant angiosarcomas. Hemangioendotheliomas are caused by abnormal growth of blood vessel cells, although the exact underlying cause for the abnormal growth is unknown. They can also develop in an organ, such as the liver or lung. They usually grow slowly and can sometimes spread to other tissues in the body (metastasize). Examples of types of hemangioendotheliomas include spindle cell hemangioma; papillary intralymphatic (Dabska tumor); retiform; kaposiform; epithelioid; pseudomyogenic (epithelioid sarcoma-like hemangioendothelioma); and composite. Treatment depends on the type of hemangioendothelioma present but typically includes surgical excision (removal). Last updated: 7/10/2013

MalaCards based summary : Hemangioendothelioma is related to epithelioid hemangioendothelioma and kaposiform hemangioendothelioma. An important gene associated with Hemangioendothelioma is CAMTA1 (Calmodulin Binding Transcription Activator 1), and among its related pathways/superpathways are Degradation of the extracellular matrix and Signaling events mediated by VEGFR1 and VEGFR2. The drugs Ethiodized oil and Vincristine have been mentioned in the context of this disorder. Affiliated tissues include liver, bone and lung, and related phenotypes are cardiovascular system and immune system

Wikipedia : 72 Hemangioendothelioma is used to describe a group of vascular neoplasms that may be considered benign as... more...

Related Diseases for Hemangioendothelioma

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

(show top 50) (show all 154)
# Related Disease Score Top Affiliating Genes
1 epithelioid hemangioendothelioma 33.1 CAMTA1 KDR PECAM1
2 kaposiform hemangioendothelioma 32.1 FLI1 FLT4 KDR PECAM1
3 hemangioma 29.7 FLT4 KDR PECAM1
4 lymphangioma 29.7 FLT4 KDR
5 cavernous hemangioma 29.6 KDR PECAM1
6 lymphedema 29.1 FLT4 KDR
7 angiosarcoma 28.7 FLI1 FLT4 KDR PECAM1
8 retiform hemangioendothelioma 12.1
9 malignant epithelioid hemangioendothelioma 12.1
10 composite hemangioendothelioma 12.1
11 spindle cell hemangioma 11.7
12 hemangioma-thrombocytopenia syndrome 11.3
13 angioma, tufted 11.3
14 hepatitis 10.7
15 sarcoma 10.2
16 epithelioid sarcoma 10.2
17 mediastinitis 10.1
18 hemangioma, capillary infantile 10.1 FLT4 KDR
19 endotheliitis 10.0
20 kuhnt-junius degeneration 10.0 KDR PLG
21 hydrocele 10.0 FLT4 PECAM1
22 pulmonary vein stenosis 10.0 KDR PECAM1
23 radiation proctitis 9.9 KDR PECAM1
24 hypothyroidism 9.9
25 cervicitis 9.9
26 thyroiditis 9.9
27 degeneration of macula and posterior pole 9.9 KDR PLG
28 breast carcinoma in situ 9.9 FLT4 KDR
29 corneal neovascularization 9.9 KDR PLG
30 proctitis 9.9 KDR PECAM1
31 plague 9.9 PECAM1 PLG
32 episodic pain syndrome, familial, 1 9.9
33 pancreatitis 9.9
34 lymphangiomatosis 9.9
35 retinal vascular disease 9.8 KDR PLG
36 budd-chiari syndrome 9.8
37 alpha-fetoprotein deficiency 9.8
38 hemopericardium 9.8
39 pericardial effusion 9.8
40 pyogenic granuloma 9.8
41 aneurysm 9.8
42 biliary cirrhosis, primary, 1 9.7
43 blood group, i system 9.7
44 congenital heart defects, hamartomas of tongue, and polysyndactyly 9.7
45 complement hyperactivation, angiopathic thrombosis, and protein-losing enteropathy 9.7
46 hemangiopericytoma, malignant 9.7
47 hydrops fetalis, nonimmune, and/or atrial septal defect 9.7
48 crohn's disease 9.7
49 primary biliary cirrhosis 9.7
50 thrombosis 9.7

Graphical network of the top 20 diseases related to Hemangioendothelioma:



Diseases related to Hemangioendothelioma

Symptoms & Phenotypes for Hemangioendothelioma

MGI Mouse Phenotypes related to Hemangioendothelioma:

43
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 9.65 FLI1 FLT4 KDR PECAM1 PLG
2 immune system MP:0005387 9.55 FLI1 FLT4 KDR PECAM1 PLG
3 liver/biliary system MP:0005370 9.35 FLI1 FLT4 KDR PECAM1 PLG
4 muscle MP:0005369 8.92 FLI1 FLT4 KDR PECAM1

Drugs & Therapeutics for Hemangioendothelioma

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

(show all 48)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Ethiodized oil Approved, Investigational Phase 4 8008-53-5
2
Vincristine Approved, Investigational Phase 2 2068-78-2, 57-22-7 5978
3
Gemcitabine Approved Phase 2 95058-81-4 60750
4
Prednisolone Approved, Vet_approved Phase 2 50-24-8 5755
5
Miconazole Approved, Investigational, Vet_approved Phase 2 22916-47-8 4189
6
Everolimus Approved Phase 2 159351-69-6 6442177
7
Benzocaine Approved, Investigational Phase 2 1994-09-7, 94-09-7 2337
8
Methylprednisolone Approved, Vet_approved Phase 2 83-43-2 6741
9
Sirolimus Approved, Investigational Phase 2 53123-88-9 46835353 6436030 5284616
10
Trametinib Approved Phase 2 871700-17-3 11707110
11
Bevacizumab Approved, Investigational Phase 2 216974-75-3
12 tannic acid Approved, Nutraceutical Phase 2
13
Tremelimumab Investigational Phase 2 745013-59-6
14 Prednisolone acetate Phase 2
15 glucocorticoids Phase 2
16 Methylprednisolone acetate Phase 2
17 Methylprednisolone Hemisuccinate Phase 2
18 Gastrointestinal Agents Phase 2
19 Neuroprotective Agents Phase 2
20 Hormone Antagonists Phase 2
21 Hormones Phase 2
22 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 2
23 Peripheral Nervous System Agents Phase 2
24 Antibodies, Monoclonal Phase 2
25 Anti-Bacterial Agents Phase 2
26 Antiemetics Phase 2
27 Anti-Infective Agents Phase 2
28 Antifungal Agents Phase 2
29 Immunoglobulins Phase 2
30 Prednisolone hemisuccinate Phase 2
31 Immunosuppressive Agents Phase 2
32 Prednisolone phosphate Phase 2
33 Antimetabolites Phase 2
34 Anti-Inflammatory Agents Phase 2
35 Antimetabolites, Antineoplastic Phase 2
36 Protective Agents Phase 2
37 Antimitotic Agents Phase 2
38 Protein Kinase Inhibitors Phase 2
39 Antibiotics, Antitubercular Phase 2
40 Antineoplastic Agents, Hormonal Phase 2
41 Antibodies Phase 2
42 Antineoplastic Agents, Phytogenic Phase 2
43 Autonomic Agents Phase 2
44 Antiviral Agents Phase 2
45 Angiogenesis Inhibitors Phase 2
46 Angiogenesis Modulating Agents Phase 2
47 Mitogens
48 Endothelial Growth Factors

Interventional clinical trials:

(show all 12)

# Name Status NCT ID Phase Drugs
1 Evaluation of Lipiodol® Ultra Fluid in Association With Surgical Glues During Vascular Embolization. Recruiting NCT02625389 Phase 4 Lipiodol® Ultra Fluid with surgical glues
2 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
3 Eribulin in Angiosarcoma and Epithelioid Hemangioendothelioma (EHE) Recruiting NCT03331250 Phase 2 Eribulin
4 Trametinib in Treating Patients With Epithelioid Hemangioendothelioma That Is Metastatic, Locally Advanced, or Cannot Be Removed by Surgery Recruiting NCT03148275 Phase 2 Trametinib
5 Sirolimus Versus Sirolimus Plus Prednisolone for Kaposiform Hemangioendothelioma With Kasabach-Merritt Syndrome Recruiting NCT03188068 Phase 2 Sirolimus;Prednisolone
6 A Study to Compare Vincristine to Sirolimus for Treatment of High Risk Vascular Tumors Recruiting NCT02110069 Phase 2 Vincristine;Sirolimus
7 Gemcitabine Hydrochloride With or Without Pazopanib Hydrochloride in Treating Patients With Refractory Soft Tissue Sarcoma Recruiting NCT01532687 Phase 2 Gemcitabine Hydrochloride;Pazopanib Hydrochloride
8 Multi-Arm Study to Test the Efficacy of Immunotherapeutic Agents in Multiple Sarcoma Subtypes Recruiting NCT02815995 Phase 2 Durvalumab;Tremelimumab
9 Bevacizumab in Treating Patients With Angiosarcoma Active, not recruiting NCT00288015 Phase 2
10 Safety and Efficacy Study of Sirolimus in Complicated Vascular Anomalies Active, not recruiting NCT00975819 Phase 2 sirolimus
11 Identification of Biomarkers for Patients With Vascular Anomalies Recruiting NCT03001180
12 Lymphatic Anomalies Registry Recruiting NCT02399527

Search NIH Clinical Center for Hemangioendothelioma

Genetic Tests for Hemangioendothelioma

Anatomical Context for Hemangioendothelioma

MalaCards organs/tissues related to Hemangioendothelioma:

38
Liver, Bone, Lung, Endothelial, Skin, Lymph Node, Heart

Publications for Hemangioendothelioma

Articles related to Hemangioendothelioma:

(show top 50) (show all 953)
# Title Authors Year
1
Pseudomyogenic Hemangioendothelioma: Case Report and Review of the Literature. ( 29406432 )
2018
2
Epithelioid hemangioendothelioma, an uncommon tumor of the eyelid: A case report. ( 29030282 )
2018
3
Association between extremity kaposiform hemangioendothelioma and lymphedema. ( 29266383 )
2018
4
Pseudomyogenic hemangioendothelioma of skin, bone and soft tissue-a clinicopathological, immunohistochemical, and fluorescence in situ hybridization study. ( 29104110 )
2018
5
Hepatic Epithelioid Hemangioendothelioma. ( 29372848 )
2018
6
Sirolimus for treatment of Kaposiform hemangioendothelioma with Kasabach-Merritt phenomenon: A retrospective cohort study. ( 29388191 )
2018
7
Hepatic epithelioid hemangioendothelioma: A misdiagnosed rare liver tumor. ( 29373279 )
2018
8
Hemangioendothelioma of palate: A case report with review of literature. ( 29391717 )
2017
9
Successful Management of Pancreatic Kaposiform Hemangioendothelioma With Sirolimus: Case Report and Literature Review. ( 28426496 )
2017
10
Multicentric visceral epithelioid hemangioendothelioma, with extremity dermal deposits, unusual late recurrence on the nasal bridge and TFE3 gene rearrangement. ( 28855107 )
2017
11
(18)F-FDG PET/CT in Pleural Epithelioid Hemangioendothelioma. ( 28840142 )
2017
12
Kaposiform hemangioendothelioma with Kasabach-Merritt syndrome: successful treatment with sirolimus. ( 28220608 )
2017
13
Sirolimus therapy in the treatment of pseudomyogenic hemangioendothelioma. ( 28843050 )
2017
14
Epithelioid hemangioendothelioma of the spine: case report and review of the literature. ( 28744509 )
2017
15
Kaposiform hemangioendothelioma in an adult with rheumatoid arthritis. ( 29154372 )
2017
16
Management of Refractory Pediatric Kaposiform Hemangioendothelioma With Sirolimus and Aspirin. ( 29240034 )
2017
17
Aggressive primary hepatic epithelioid hemangioendothelioma: a case report and literature review. ( 28607810 )
2017
18
Pulmonary epithelioid hemangioendothelioma imitating lung cancer. ( 29181052 )
2017
19
Incidental Detection of Retiform Hemangioendothelioma by 68Ga DOTATOC PET/CT. ( 27997421 )
2017
20
Clinico-radiological features and next generation sequencing of pulmonary epithelioid hemangioendothelioma: A case report and review of literature. ( 28777494 )
2017
21
The clinical features of epithelioid hemangioendothelioma in a Han Chinese population: A retrospective analysis. ( 28658152 )
2017
22
Primary epithelioid hemangioendothelioma of the dorsal spine: A review. ( 28879934 )
2017
23
Cutaneous epithelioid hemangioendothelioma on the sole mimicking a pyogenic granuloma. ( 28664615 )
2017
24
Intestinal obstruction due to kaposiform hemangioendothelioma in a 1-month-old infant: A case report. ( 28906349 )
2017
25
Epithelioid hemangioendothelioma of the craniocervical junction; case report and review. ( 28478911 )
2017
26
Pseudomyogenic hemangioendothelioma. ( 29360904 )
2017
27
Epithelioid hemangioendothelioma presenting as fever of unknown origin. ( 28507590 )
2017
28
Composite hemangioendothelioma with neuroendocrine marker expression: an aggressive variant. ( 28731049 )
2017
29
Long-term outcome for kaposiform hemangioendothelioma: A report of two cases. ( 27701822 )
2017
30
Hepatic epithelioid hemangioendothelioma with metastasis to the mesentery of the small intestine: A case report. ( 29142751 )
2017
31
New Insights about Pulmonary Epithelioid Hemangioendothelioma: Review of the Literature and Two Case Reports. ( 28884037 )
2017
32
Malignant Hepatic Epithelioid Hemangioendothelioma with Villaret Syndrome. ( 28348474 )
2017
33
Apatinib for the treatment of pulmonary epithelioid hemangioendothelioma: A case report and literature review. ( 29137048 )
2017
34
Kaposiform Hemangioendothelioma Presenting as Hydrops Fetalis. ( 28318049 )
2017
35
Hepatic Epithelioid Hemangioendothelioma - a Rare Tumor and Diagnostic Dilemma. ( 28652454 )
2017
36
An Epithelioid Hemangioendothelioma of the Head and Neck. ( 28834832 )
2017
37
18-F-FDG PET-CT in Monitoring of Chemotherapeutic Effect in a Case of Metastatic Hepatic Epithelioid Hemangioendothelioma. ( 28680215 )
2017
38
Imaging findings of Kaposiform Hemangioendothelioma in children. ( 28027747 )
2017
39
Integrin I^3/Akt signaling contributes to platelet-induced hemangioendothelioma growth. ( 28744026 )
2017
40
Massive retiform hemangioendothelioma that expresses D2-40. ( 28366927 )
2017
41
Artesunate inhibits proliferation and invasion of mouse hemangioendothelioma cells<i>in vitro</i>and of tumor growth<i>in vivo</i>. ( 29113263 )
2017
42
Restricted Range of Motion and a Cold Upper Extremity in a Two-Year-Old Boy: Kaposiform Hemangioendothelioma of the Bone and the Brachial Plexus: A Case Report. ( 29286963 )
2017
43
Identical Presentation of Scapular Osteolysis in Two Patients with Thoracic Kaposiform Hemangioendothelioma. ( 28382716 )
2017
44
Composite hemangioendothelioma of the forehead and right eye; a case report. ( 29233122 )
2017
45
Sirolimus for the treatment of progressive kaposiform hemangioendothelioma: A multicenter retrospective study. ( 28486787 )
2017
46
Magnetic resonance findings of hepatic epithelioid hemangioendothelioma: emphasis on hepatobiliary phase using Gd-EOB-DTPA. ( 28365784 )
2017
47
Hemangioendothelioma of the Bladder: The Youngest Case Report in A Child. ( 28752069 )
2017
48
Pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma - a rare vascular neoplasm with deceptive morphology and distinctive immunophenotype. ( 29279595 )
2017
49
TFE3-rearranged hepatic epithelioid hemangioendothelioma-a case report with immunohistochemical and molecular study. ( 28585251 )
2017
50
Primary hepatic hemangioendothelioma in a patient with Budd-Chiari syndrome. ( 29054942 )
2017

Variations for Hemangioendothelioma

Expression for Hemangioendothelioma

Search GEO for disease gene expression data for Hemangioendothelioma.

Pathways for Hemangioendothelioma

GO Terms for Hemangioendothelioma

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

# Name GO ID Score Top Affiliating Genes
1 positive regulation of MAPK cascade GO:0043410 9.4 FLT4 KDR
2 vascular endothelial growth factor receptor signaling pathway GO:0048010 9.37 FLT4 KDR
3 positive regulation of endothelial cell proliferation GO:0001938 9.32 FLT4 KDR
4 hemostasis GO:0007599 9.26 FLI1 PLG
5 positive regulation of endothelial cell migration GO:0010595 9.16 FLT4 KDR
6 cellular response to vascular endothelial growth factor stimulus GO:0035924 8.96 FLT4 KDR
7 vascular endothelial growth factor signaling pathway GO:0038084 8.62 FLT4 KDR

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

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

Sources for Hemangioendothelioma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
27 GO
28 GTR
29 HGMD
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 MedGen
41 MeSH
42 MESH via Orphanet
43 MGI
45 NCI
46 NCIt
47 NDF-RT
50 NINDS
51 Novoseek
53 OMIM
54 OMIM via Orphanet
58 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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