MCID: HMN016
MIFTS: 41

Hemangioendothelioma

Categories: Cancer diseases, Cardiovascular diseases, Rare diseases

Aliases & Classifications for Hemangioendothelioma

MalaCards integrated aliases for Hemangioendothelioma:

Name: Hemangioendothelioma 53 55 72

Classifications:



External Ids:

UMLS 72 C0018915

Summaries for Hemangioendothelioma

NIH Rare Diseases : 53 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).

MalaCards based summary : Hemangioendothelioma is related to epithelioid hemangioendothelioma and malignant epithelioid 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 Tacrolimus 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 : 75 Hemangioendotheliomas are a family of vascular neoplasms of intermediate... more...

Related Diseases for Hemangioendothelioma

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

(show top 50) (show all 389)
# Related Disease Score Top Affiliating Genes
1 epithelioid hemangioendothelioma 34.2 KDR CAMTA1
2 malignant epithelioid hemangioendothelioma 33.2 KDR FLT4 CAMTA1
3 kaposiform hemangioendothelioma 31.9 PECAM1 KDR FLT4 FLI1
4 hemangioma 30.5 PECAM1 KDR FLT4
5 lymphangioma 30.2 KDR FLT4
6 capillary hemangioma 30.1 PECAM1 KDR
7 cavernous hemangioma 29.8 PECAM1 KDR
8 angiosarcoma 29.3 PECAM1 KDR FLT4 FLI1
9 vascular cancer 29.2 KDR FLT4 FLI1 CAMTA1
10 retinal vascular disease 28.8 PLG KDR
11 retiform hemangioendothelioma 12.4
12 composite hemangioendothelioma 12.4
13 spindle cell hemangioma 12.1
14 hemangioma-thrombocytopenia syndrome 11.6
15 angioma, tufted 11.6
16 rare tumor 10.8
17 rare vascular tumor 10.7
18 thrombocytopenia 10.5
19 kaposi sarcoma 10.5
20 epithelioid sarcoma 10.5
21 congestive heart failure 10.4
22 sarcoma 10.4
23 spindle cell sarcoma 10.4
24 lymphangiomatosis 10.3
25 histiocytoid hemangioma 10.3
26 benign mesothelioma 10.3
27 pyogenic granuloma 10.3
28 back pain 10.3
29 goiter 10.2
30 hemangiopericytoma, malignant 10.2
31 hypothyroidism 10.2
32 rhabdomyosarcoma 10.2
33 respiratory failure 10.2
34 conventional angiosarcoma 10.2
35 rare lymphatic malformation 10.2
36 lymphedema 10.2
37 cholangiocarcinoma 10.2
38 hemopericardium 10.2
39 pericardial effusion 10.2
40 intrahepatic cholangiocarcinoma 10.2
41 hepatoblastoma 10.2
42 hypereosinophilic syndrome 10.2
43 rare soft tissue tumor 10.2
44 mesothelioma, malignant 10.1
45 disseminated intravascular coagulation 10.1
46 arteriovenous malformation 10.1
47 portal hypertension 10.1
48 endemic goiter 10.1
49 mesenchymal cell neoplasm 10.1
50 fetal edema 10.1

Graphical network of the top 20 diseases related to Hemangioendothelioma:



Diseases related to Hemangioendothelioma

Symptoms & Phenotypes for Hemangioendothelioma

MGI Mouse Phenotypes related to Hemangioendothelioma:

46
# 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 top 50) (show all 71)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1 Ethiodized oil Approved, Investigational Phase 4 8008-53-5
2
Tacrolimus Approved, Investigational Phase 4 104987-11-3 445643 439492 6473866
3 Immunologic Factors Phase 4
4 Immunosuppressive Agents Phase 4
5 Calcineurin Inhibitors Phase 4
6
Sunitinib Approved, Investigational Phase 2 557795-19-4, 341031-54-7 5329102
7
Trametinib Approved Phase 2 871700-17-3 11707110
8
Prednisolone phosphate Approved, Vet_approved Phase 2 302-25-0
9
Methylprednisolone hemisuccinate Approved Phase 2 2921-57-5
10
Propranolol Approved, Investigational Phase 2 525-66-6 4946
11
Methylprednisolone Approved, Vet_approved Phase 2 83-43-2 6741
12
Prednisolone Approved, Vet_approved Phase 2 50-24-8 5755
13
tannic acid Approved Phase 2 1401-55-4
14
Vincristine Approved, Investigational Phase 2 57-22-7, 2068-78-2 5978
15
Benzocaine Approved, Investigational Phase 2 94-09-7, 1994-09-7 2337
16
Sirolimus Approved, Investigational Phase 2 53123-88-9 6436030 5284616 46835353
17
Miconazole Approved, Investigational, Vet_approved Phase 2 22916-47-8 4189
18
Everolimus Approved Phase 2 159351-69-6 6442177 70789204
19
Gemcitabine Approved Phase 2 95058-81-4 60750
20
Durvalumab Approved, Investigational Phase 2 1428935-60-7
21
Prednisolone hemisuccinate Experimental Phase 2 2920-86-7
22
Tremelimumab Investigational Phase 2 745013-59-6
23 Angiogenesis Inhibitors Phase 2
24 Angiogenesis Modulating Agents Phase 2
25 Protein Kinase Inhibitors Phase 2
26 Neurotransmitter Agents Phase 2
27 Gastrointestinal Agents Phase 2
28 Antihypertensive Agents Phase 2
29 Antiemetics Phase 2
30 Neuroprotective Agents Phase 2
31 Methylprednisolone Acetate Phase 2
32 Anti-Inflammatory Agents Phase 2
33 Adrenergic beta-Antagonists Phase 2
34 glucocorticoids Phase 2
35 Hormones Phase 2
36 Peripheral Nervous System Agents Phase 2
37 Adrenergic Antagonists Phase 2
38 Protective Agents Phase 2
39 Antineoplastic Agents, Hormonal Phase 2
40 Anti-Arrhythmia Agents Phase 2
41 Hormone Antagonists Phase 2
42 Adrenergic Agents Phase 2
43 Vasodilator Agents Phase 2
44 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 2
45 Prednisolone acetate Phase 2
46 Autonomic Agents Phase 2
47 Antineoplastic Agents, Phytogenic Phase 2
48 Tubulin Modulators Phase 2
49 Antimitotic Agents Phase 2
50 Antifungal Agents Phase 2

Interventional clinical trials:

(show all 16)
# Name Status NCT ID Phase Drugs
1 Safety and Efficacy of Lipiodol® Ultra Fluid in Association With Surgical Glues During Vascular Embolization. A Phase IV Study. Recruiting NCT02625389 Phase 4 Lipiodol® Ultra Fluid with surgical glues
2 Tacrolimus for the Treatment of Superficial Kaposiform Hemangioendothelioma and Tufted Angioma: a Single Arm Prospective Study Not yet recruiting NCT04056962 Phase 4 Tacrolimus ointment
3 A Phase II, Open-label, Non-randomized Trial of Sunitinib in Certain Subtypes of Soft Tissue Sarcomas Unknown status NCT00859456 Phase 2 Sunitinib
4 A Phase 1B/II Study of GDC-0449 (NSC 747691) in Combination With RO4929097, a Gamma-Secretase Inhibitor (GSI) in Advanced/Metastatic Sarcomas Completed NCT01154452 Phase 1, Phase 2 Gamma-Secretase Inhibitor RO4929097;Vismodegib
5 A Non-Randomized, Open-Label, Phase 2 Study of Trametinib in Patients With Unresectable or Metastatic Epithelioid Hemangioendothelioma Recruiting NCT03148275 Phase 2 Trametinib
6 A Pilot Phase 2 Study of Eribulin in Angiosarcoma and Epithelioid Hemangioendothelioma (EHE) Recruiting NCT03331250 Phase 2 Eribulin
7 Sirolimus Plus Propranolol Versus Sirolimus Plus Prednisolone for Kaposiform Hemangioendothelioma With Kasabach-Merritt Syndrome Recruiting NCT03188068 Phase 2 Sirolimus;Prednisolone;Propranolol
8 A Randomized Phase 2 Study of Vincristine Versus Sirolimus to Treat High Risk Kaposiform Hemangioendothelioma (KHE). Recruiting NCT02110069 Phase 2 Vincristine;Sirolimus
9 A Randomized, Double-Blind Phase II, Study of Gemcitabine Alone or in Combination With Pazopanib for Refractory Soft Tissue Sarcoma Active, not recruiting NCT01532687 Phase 2 Gemcitabine Hydrochloride;Pazopanib Hydrochloride
10 A Phase 2 Study - Clinical Trial Assessing Efficacy and Safety of the mTOR Inhibitor Sirolimus in the Treatment of Complicated Vascular Anomalies Active, not recruiting NCT00975819 Phase 2 sirolimus
11 A Phase II Multi-Arm Study to Test the Efficacy of Immunotherapeutic Agents in Multiple Sarcoma Subtypes Active, not recruiting NCT02815995 Phase 2 Durvalumab;Tremelimumab
12 Diagnostic and Differential Diagnostic Accuracy of PIVKA-II Combining With Alpha-Fetoprotein in Hepatic Tumor of Infant Recruiting NCT03645655
13 Identification of Biomarkers for Patients With Vascular Anomalies Recruiting NCT03001180
14 Lymphatic Anomalies Registry Recruiting NCT02399527
15 Multimodal Immune Characterization of RAre Soft Tissue Sarcoma - MIRAS Project From SARRA (SARcome RAre) Project of the French Sarcoma Group Not yet recruiting NCT03967834
16 Prospective Identification of Predictive Biomarkers of Trabectedin Efficacy in Non-L Soft-tissue Sarcoma Patients Not yet recruiting NCT04008238 Trabectedin

Search NIH Clinical Center for Hemangioendothelioma

Genetic Tests for Hemangioendothelioma

Anatomical Context for Hemangioendothelioma

MalaCards organs/tissues related to Hemangioendothelioma:

41
Liver, Bone, Lung, Endothelial, Thyroid, Heart, Spleen

Publications for Hemangioendothelioma

Articles related to Hemangioendothelioma:

(show top 50) (show all 2578)
# Title Authors PMID Year
1
Refractory kaposiform hemangioendothelioma that expressed vascular endothelial growth factor receptor (VEGFR)-2 and VEGFR-3: a case report. 9 38
19262246 2009
2
D2-40 immunohistochemical analysis of pediatric vascular tumors reveals positivity in kaposiform hemangioendothelioma. 9 38
15920541 2005
3
Vascular endothelial growth factor receptor-3 (VEGFR-3): a marker of vascular tumors with presumed lymphatic differentiation, including Kaposi's sarcoma, kaposiform and Dabska-type hemangioendotheliomas, and a subset of angiosarcomas. 9 38
10697276 2000
4
Interferon alpha-2b at low doses as long-term antiangiogenic treatment of a metastatic intracranial hemangioendothelioma: a case report. 9 38
10601609 2000
5
Spindle cell hemangioendothelioma of the pancreas treated with interferon-alpha2a. 9 38
9510148 1998
6
Human angiostatin inhibits murine hemangioendothelioma tumor growth in vivo. 9 38
9393749 1997
7
Spindle cell (Kaposiform) hemangioendothelioma with Kasabach-Merritt syndrome in an infant: successful treatment with alpha-2A interferon. 9 38
9121401 1997
8
The human hematopoietic progenitor cell antigen (CD34) in vascular neoplasia. 9 38
1712541 1991
9
FDG PET/CT of Mediastinal Epithelioid Hemangioendothelioma. 38
31283613 2019
10
Epithelioid Hemangioendothelioma Arising Within Mediastinal Myelolipoma: A WWTR1-Driven Composite Neoplasm. 38
30942102 2019
11
Ultrasonography and magnetic resonance imaging features of kaposiform hemangioendothelioma and tufted angioma. 38
31373042 2019
12
Tacrolimus ointment for the treatment of superficial kaposiform hemangioendothelioma and tufted angioma. 38
31373046 2019
13
Low dose sirolimus treatment for refractory tufted angioma and congenital kaposiform hemangioendothelioma, both with Kasabach-Merritt phenomenon. 38
31087627 2019
14
Tips and Tricks in Contrast-Enhanced Ultrasound (CEUS) for the Characterization and Detection of Liver Malignancies. 38
31382313 2019
15
[Atypical epithelioid hemangioendothelioma: a clinicopathological analysis of eight cases]. 38
31422593 2019
16
Molecular characterization of hepatic epithelioid hemangioendothelioma reveals alterations in various genes involved in DNA repair, epigenetic regulation, signaling pathways and cell cycle control. 38
31390105 2019
17
Survival Analysis of Patients with Osseous Malignant Vascular Tumors: Results of the Surveillance, Epidemiology, and End Results (SEER) Database from 1973 to 2015. 38
31344019 2019
18
SFCE METRO-01 four-drug metronomic regimen phase II trial for pediatric extracranial tumor. 38
30920117 2019
19
Haemangioendothelioma of Oral Cavity: A Rare Entity with Unusual Location. 38
31253225 2019
20
Hepatic Sclerosing Cavernous Hemangioma Can Mimic the Nodular Elastosis Stage of Segmental Atrophy. 38
31356680 2019
21
A not so harmless mass: Kaposiform hemangioendothelioma complicated by a Kasabach-Merritt phenomenon. 38
31353149 2019
22
Kaposiform lymphangiomatosis and kaposiform hemangioendothelioma: similarities and differences. 38
31277673 2019
23
Intradural Hemangioendothelioma of Lumbar Spine: Dilemmas and differentials in diagnosis. 38
31295588 2019
24
Fusion of the Genes WWTR1 and FOSB in Pseudomyogenic Hemangioendothelioma. 38
31243110 2019
25
[Research Progress of Pulmonary Epithelioid Hemangioendothelioma]. 38
31315787 2019
26
Surgical Resection of Hepatic Epithelioid Hemangioendothelioma. 38
31405447 2019
27
A rare case of pseudomyogenic hemangioendothelioma (PHE)/epithelioid sarcoma-like hemangioendothelioma (ES-H) of the breast first misdiagnosed as metaplastic carcinoma by FNAB and review of the literature. 38
31311568 2019
28
Effective Use of Sirolimus and Zoledronic Acid for Multiosteotic Pseudomyogenic Hemangioendothelioma of the Bone in a Child: Case Report and Review of Literature. 38
31094908 2019
29
Primary pseudomyogenic hemangioendothelioma of the vulva: a rare location for a rare entity. 38
31238962 2019
30
Pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma of bone: Clinicopathologic features of 5 cases. 38
31233904 2019
31
Epithelioid Hemangioendothelioma of the Bowel in Crohn's Disease: The First Reported Case. 38
30238810 2019
32
Massive Epithelioid Hemangioendothelioma Causing Noncirrhotic Portal Hypertension. 38
29609064 2019
33
Contrast-enhanced imaging in hepatic epithelioid hemangioendothelioma: retrospective study of 10 patients. 38
30986884 2019
34
Primary vascular bone tumors in the spine: a challenge for pathologists and spine oncology surgeons. 38
30900092 2019
35
Pseudomyogenic Hemangioendothelioma. 38
30576238 2019
36
Imaging findings in epithelioid hemangioendothelioma. 38
31238187 2019
37
[Multifocal hepatic hemangioendothelioma]. 38
31344192 2019
38
Epithelioid Hemangioendothelioma: A Rare Case of an Aggressive Vascular Malignancy. 38
31209195 2019
39
Outcome of Children Treated for Infantile Hepatic Hemangioendothelioma. 38
31233466 2019
40
A report on Kaposiform hemangioendothelioma in the cervical spine. 38
30896568 2019
41
Diagnosis of known sarcoma fusions and novel fusion partners by targeted RNA sequencing with identification of a recurrent ACTB-FOSB fusion in pseudomyogenic hemangioendothelioma. 38
30459475 2019
42
Epithelioid hemangioendothelioma of the bone: A case report with findings of bone scintigraphy. 38
31083212 2019
43
Epithelioid Vascular Tumors: A Review. 38
30762622 2019
44
Activation of Hedgehog Signaling in Aggressive Hepatic Hemangioma in Newborns and Infants. 38
31092427 2019
45
Difficulty of treatment for pleural epithelioid hemangioendothelioma: a report of a case. 38
31049818 2019
46
Blockade of angiogenin by thalidomide inhibits the tumorigenesis of murine hemangioendothelioma. 38
31044460 2019
47
Hypoglycemic syndrome in hepatic epithelioid hemangioendothelioma, successful treatment - liver transplantation from a living related donor. 38
31091051 2019
48
Cinobufagin suppresses colorectal cancer angiogenesis by disrupting the endothelial mammalian target of rapamycin/hypoxia-inducible factor 1α axis. 38
30839155 2019
49
Adult-to-adult living-donor liver transplantation: The experience of the Université catholique de Louvain. 38
30850341 2019
50
Pseudomyogenic Hemangioendothelioma (Epithelioid Sarcoma-Like Hemangioendothelioma). 38
31017450 2019

Variations for Hemangioendothelioma

Expression for Hemangioendothelioma

Search GEO for disease gene expression data for Hemangioendothelioma.

Pathways for Hemangioendothelioma

Pathways related to Hemangioendothelioma according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Show member pathways
12.09 PLG PECAM1 KDR
2
Show member pathways
11.33 KDR FLT4
3
Show member pathways
11.24 KDR FLT4
4 10.85 PLG KDR
5 10.75 PECAM1 KDR FLT4
6 10.59 KDR FLT4

GO Terms for Hemangioendothelioma

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

# Name GO ID Score Top Affiliating Genes
1 transmembrane receptor protein tyrosine kinase signaling pathway GO:0007169 9.43 KDR FLT4
2 positive regulation of endothelial cell proliferation GO:0001938 9.4 KDR FLT4
3 vascular endothelial growth factor receptor signaling pathway GO:0048010 9.37 KDR FLT4
4 hemostasis GO:0007599 9.32 PLG FLI1
5 positive regulation of endothelial cell migration GO:0010595 9.26 KDR FLT4
6 cellular response to vascular endothelial growth factor stimulus GO:0035924 9.16 KDR FLT4
7 angiogenesis GO:0001525 9.13 PECAM1 KDR FLT4
8 vascular endothelial growth factor signaling pathway GO:0038084 8.62 KDR FLT4

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 KDR FLT4
2 growth factor binding GO:0019838 8.96 KDR FLT4
3 vascular endothelial growth factor-activated receptor activity GO:0005021 8.62 KDR FLT4

Sources for Hemangioendothelioma

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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