MCID: HMN016
MIFTS: 35

Hemangioendothelioma

Categories: Cancer diseases, Cardiovascular diseases, Rare diseases

Aliases & Classifications for Hemangioendothelioma

MalaCards integrated aliases for Hemangioendothelioma:

Name: Hemangioendothelioma 20 54 70

Classifications:



External Ids:

UMLS 70 C0018915

Summaries for Hemangioendothelioma

GARD : 20 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). The drugs tannic acid and Benzocaine have been mentioned in the context of this disorder. Affiliated tissues include liver, endothelial and spleen, and related phenotypes are liver/biliary system and neoplasm

Wikipedia : 73 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 379)
# Related Disease Score Top Affiliating Genes
1 epithelioid hemangioendothelioma 32.5 WWTR1 CAMTA1
2 malignant epithelioid hemangioendothelioma 31.7 WWTR1 FOSB CAMTA1
3 kaposiform hemangioendothelioma 31.4 FLT4 FLI1 CD34
4 conventional angiosarcoma 30.2 CD34 CAMTA1
5 hemangioma 30.1 FOSB FLT4 FLI1 CD34
6 lymphangioma 30.1 FLT4 CD34
7 angiosarcoma 30.1 FLT4 FLI1 CD34 CAMTA1
8 capillary hemangioma 30.0 FLT4 CD34
9 proliferative fasciitis 29.9 FOSB CAMTA1
10 histiocytoid hemangioma 29.8 WWTR1 FOSB CD34 CAMTA1
11 breast angiosarcoma 29.3 FLT4 CD34
12 lymphangiosarcoma 29.3 FLT4 CD34
13 vascular cancer 28.8 WWTR1 PLG FLT4 FLI1 CD34 CAMTA1
14 retiform hemangioendothelioma 11.2
15 composite hemangioendothelioma 11.2
16 spindle cell hemangioma 11.1
17 angioma, tufted 11.0
18 rare tumor 10.7
19 rare vascular tumor 10.7
20 hemangioma-thrombocytopenia syndrome 10.5
21 epithelioid sarcoma 10.5
22 kaposi sarcoma 10.5
23 sarcoma 10.4
24 spindle cell sarcoma 10.4
25 thrombocytopenia 10.4
26 lymphangiomatosis 10.3
27 benign mesothelioma 10.2
28 cavernous hemangioma 10.2
29 pyogenic granuloma 10.2
30 hereditary lymphedema i 10.2
31 rhabdomyosarcoma 10.2
32 hemangiopericytoma, malignant 10.1
33 cholangiocarcinoma 10.1
34 hemopericardium 10.1
35 pericardial effusion 10.1
36 hypothyroidism 10.1
37 intrahepatic cholangiocarcinoma 10.1
38 rare lymphatic malformation 10.1
39 mesothelioma, malignant 10.1
40 goiter 10.1
41 hepatoblastoma 10.1
42 hypereosinophilic syndrome 10.1
43 rare soft tissue tumor 10.1
44 respiratory failure 10.1
45 arteriovenous malformation 10.1
46 obstructive jaundice 10.1
47 malignant hemangioma 10.1 WWTR1 CAMTA1
48 hydrops fetalis, nonimmune 10.0
49 lymphatic malformation 7 10.0
50 disseminated intravascular coagulation 10.0

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 liver/biliary system MP:0005370 9.26 FLI1 FLT4 PLG WWTR1
2 neoplasm MP:0002006 8.92 CAMTA1 CD34 FLI1 PLG

Drugs & Therapeutics for Hemangioendothelioma

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

(show top 50) (show all 63)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
tannic acid Approved Phase 4 1401-55-4
2
Benzocaine Approved, Investigational Phase 4 1994-09-7, 94-09-7 2337
3
Tacrolimus Approved, Investigational Phase 4 104987-11-3 6473866 445643 439492
4
Ethiodized oil Approved, Investigational Phase 4 8008-53-5
5
Clotrimazole Approved, Vet_approved Phase 4 23593-75-1 2812
6
Miconazole Approved, Investigational, Vet_approved Phase 4 22916-47-8 4189
7
Sirolimus Approved, Investigational Phase 4 53123-88-9 6436030 5284616
8
Propranolol Approved, Investigational Phase 4 525-66-6 4946
9 Calcineurin Inhibitors Phase 4
10 Antibiotics, Antitubercular Phase 4
11 Immunosuppressive Agents Phase 4
12 Immunologic Factors Phase 4
13 Anti-Bacterial Agents Phase 4
14 Anti-Infective Agents Phase 4
15 Antifungal Agents Phase 4
16 Anti-Arrhythmia Agents Phase 4
17 Vasodilator Agents Phase 4
18 Adrenergic Antagonists Phase 4
19 Adrenergic beta-Antagonists Phase 4
20 Neurotransmitter Agents Phase 4
21 Antihypertensive Agents Phase 4
22 Adrenergic Agents Phase 4
23
Gemcitabine Approved Phase 2 95058-81-4 60750
24
Trametinib Approved Phase 2 871700-17-3 11707110
25
Methylprednisolone Approved, Vet_approved Phase 2 83-43-2 6741
26
Prednisolone Approved, Vet_approved Phase 2 50-24-8 5755
27
Methylprednisolone hemisuccinate Approved Phase 2 2921-57-5
28
Prednisolone phosphate Approved, Vet_approved Phase 2 302-25-0
29
Prednisolone acetate Approved, Vet_approved Phase 2 52-21-1
30
Vincristine Approved, Investigational Phase 2 2068-78-2, 57-22-7 5978
31
Prednisolone hemisuccinate Experimental Phase 2 2920-86-7
32 Antiviral Agents Phase 2
33 Antimetabolites Phase 2
34 Protein Kinase Inhibitors Phase 2
35 Hormones Phase 2
36 Methylprednisolone Acetate Phase 2
37 Hormone Antagonists Phase 2
38 glucocorticoids Phase 2
39 Antineoplastic Agents, Hormonal Phase 2
40 Anti-Inflammatory Agents Phase 2
41
Prednisone Approved, Vet_approved Phase 1 53-03-2 5865
42
Paclitaxel Approved, Vet_approved Phase 1 33069-62-4 36314
43
Bevacizumab Approved, Investigational Phase 1 216974-75-3
44 Tubulin Modulators Phase 1
45 Antimitotic Agents Phase 1
46 Angiogenesis Inhibitors Phase 1
47 Immunoglobulin G Phase 1
48 Immunoglobulins, Intravenous Phase 1
49 Antineoplastic Agents, Immunological Phase 1
50 Antibodies Phase 1

Interventional clinical trials:

(show all 20)
# Name Status NCT ID Phase Drugs
1 Tacrolimus for the Treatment of Superficial Kaposiform Hemangioendothelioma and Tufted Angioma: a Single Arm Prospective Study Recruiting NCT04056962 Phase 4 Tacrolimus ointment
2 Guided Discontinuation Versus Maintenance Treatment of Sirolimus in Pediatric Patients With Kaposiform Hemangioendothelioma: a Randomized Controlled Trial Recruiting NCT04448873 Phase 4 Sirolimus
3 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
4 Safety and Efficacy of Low-dose Sirolimus to Kaposiform Hemangioendothelioma:A Prospective, Randomized Open Trial Recruiting NCT04077515 Phase 4 Sirolimus(0.8mg/m2);Sirolimus(0.7mg/m2)
5 Sirolimus in the Treatment for Propranolol-resistant Infantile Hepatic Hemangioendothelioma Not yet recruiting NCT04406870 Phase 4 Sirolimus Oral Product;propranolol
6 Efficacy and Safety of Different Concentrations of Sirolimus in the Treatment of Kaposiform Hemangioendothelioma. Recruiting NCT04775173 Phase 3 Sirolimus
7 A Phase 2 Study - Clinical Trial Assessing Efficacy and Safety of the mTOR Inhibitor Sirolimus in the Treatment of Complicated Vascular Anomalies Unknown status NCT00975819 Phase 2 sirolimus
8 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
9 A Randomized, Double-Blind Phase II, Study of Gemcitabine Alone or in Combination With Pazopanib for Refractory Soft Tissue Sarcoma Completed NCT01532687 Phase 2 Gemcitabine;Gemcitabine Hydrochloride;Pazopanib;Pazopanib Hydrochloride
10 A Non-Randomized, Open-Label, Phase 2 Study of Trametinib in Patients With Unresectable or Metastatic Epithelioid Hemangioendothelioma Recruiting NCT03148275 Phase 2 Trametinib
11 A Pilot Phase 2 Study of Eribulin in Angiosarcoma and Epithelioid Hemangioendothelioma (EHE) Recruiting NCT03331250 Phase 2 Eribulin
12 Sirolimus Versus Sirolimus Plus Prednisolone for Kaposiform Hemangioendothelioma With Kasabach-Merritt Syndrome Recruiting NCT03188068 Phase 2 Sirolimus;Prednisolone
13 A Randomized Phase 2 Study of Vincristine Versus Sirolimus to Treat High Risk Kaposiform Hemangioendothelioma (KHE). Active, not recruiting NCT02110069 Phase 2 Vincristine;Sirolimus
14 A Clinical Study on the Treatment of Kaposiform Hemangioendothelioma (KHE) With Kasabach-Merritt Phenomenon (KMP) by Hormone Shock and Sirolimus Maintenance Not yet recruiting NCT04409691 Phase 1 prednison and Sirolimus;prednison
15 Targeted Complex Therapy for Advanced Melanoma, Gynecologic Cancers, and Other Malignancies: Nab-Paclitaxel (Abraxane)/Bevacizumab Complex (AB-Complex) Suspended NCT02020707 Phase 1 Nab-paclitaxel
16 Multimodal Immune Characterization of RAre Soft Tissue Sarcoma - MIRAS Project From SARRA (SARcome RAre) Project of the French Sarcoma Group Recruiting NCT03967834
17 PIVKA-II Combined With Alpha-Fetoprotein for Diagnostic and Prognostic Value of Hepatic Tumors in Infants: Protocol for a Multicenter, Observational Trialin Hepatic Tumor Recruiting NCT03645655
18 Identification of Biomarkers for Patients With Vascular Anomalies Recruiting NCT03001180
19 Lymphatic Anomalies Registry for the Assessment of Outcome Data Recruiting NCT02399527
20 Prospective Identification of Predictive Biomarkers of Trabectedin Efficacy in Non-L Soft-tissue Sarcoma Patients Recruiting NCT04008238 Trabectedin

Search NIH Clinical Center for Hemangioendothelioma

Genetic Tests for Hemangioendothelioma

Anatomical Context for Hemangioendothelioma

MalaCards organs/tissues related to Hemangioendothelioma:

40
Liver, Endothelial, Spleen, Lymph Node, Tongue, Small Intestine, Pancreas

Publications for Hemangioendothelioma

Articles related to Hemangioendothelioma:

(show top 50) (show all 2779)
# Title Authors PMID Year
1
Refractory kaposiform hemangioendothelioma that expressed vascular endothelial growth factor receptor (VEGFR)-2 and VEGFR-3: a case report. 54 61
19262246 2009
2
D2-40 immunohistochemical analysis of pediatric vascular tumors reveals positivity in kaposiform hemangioendothelioma. 61 54
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. 54 61
10697276 2000
4
Interferon alpha-2b at low doses as long-term antiangiogenic treatment of a metastatic intracranial hemangioendothelioma: a case report. 61 54
10601609 2000
5
Spindle cell hemangioendothelioma of the pancreas treated with interferon-alpha2a. 54 61
9510148 1998
6
Human angiostatin inhibits murine hemangioendothelioma tumor growth in vivo. 54 61
9393749 1997
7
Spindle cell (Kaposiform) hemangioendothelioma with Kasabach-Merritt syndrome in an infant: successful treatment with alpha-2A interferon. 61 54
9121401 1997
8
The human hematopoietic progenitor cell antigen (CD34) in vascular neoplasia. 54 61
1712541 1991
9
Hepatic vascular malignancies in children are associated with increased rates of surgical resection and improved overall survival compared with adults. 61
33661569 2021
10
Additive value of transarterial embolization to systemic sirolimus treatment in kaposiform hemangioendothelioma. 61
33231291 2021
11
Primary splenic epithelioid hemangioendothelioma with diffuse metastases revealed by FDG PET/CT imaging: A case report. 61
33787588 2021
12
Long-term outcomes of pediatric and young adult patients receiving radiotherapy for nonmalignant vascular anomalies. 61
33754454 2021
13
Cytomorphological features of an unusual case of a multifocal pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma in a young adult male. 61
32886445 2021
14
Vascular neoplasms and non-neoplastic vascular lesions of the spleen. 61
32674844 2021
15
Vascular Anomalies of the Head and Neck: A Pediatric Overview. 61
33723757 2021
16
Kaposiform Hemangioendothelioma: clinicopathological characteristics of 8 cases of a rare vascular tumor and review of literature. 61
33722245 2021
17
Kaposiform Hemangioendothelioma of the Oral Cavity: A Rare Tumor with an Unusual Location. 61
33715098 2021
18
WWTR1(TAZ)-CAMTA1 reprograms endothelial cells to drive epithelioid hemangioendothelioma. 61
33766984 2021
19
Epithelioid Hemangioendothelioma of the Superior Vena Cava Diagnosed With EBUS-TBNA. 61
33781573 2021
20
Post-resection prognosis of patients with hepatic epithelioid hemangioendothelioma. 61
33748027 2021
21
WWTR1(TAZ)-CAMTA1 gene fusion is sufficient to dysregulate YAP/TAZ signaling and drive epithelioid hemangioendothelioma tumorigenesis. 61
33766982 2021
22
Combined Transarterial Sclerotherapy and Embolization with Systemic Chemotherapy Followed by Everolimus in Kaposiform Hemangioendothelioma with Kasabach-Merritt Phenomenon. 61
33707126 2021
23
Pseudomyogenic hemangioendothelioma of bone with rare WWTR1-FOSB fusion gene: Case report and literature review. 61
33768875 2021
24
Pseudomyogenic Hemangioendothelioma Involving the Esophagus. 61
33767026 2021
25
Case of epithelioid hemangioendothelioma occurring in the postradiation setting for breast cancer. 61
33125182 2021
26
Severe adverse events during sirolimus "off-label" therapy for vascular anomalies. 61
33580918 2021
27
Comparison of efficacy and safety of corticosteroid and vincristine in treating kaposiform hemangioendothelioma and tufted angioma: A multicenter prospective randomized controlled clinical trial. 61
33608936 2021
28
Laparoscopic resection of liver tumors in children. 61
33046224 2021
29
"Bull's eye" appearance of hepatocellular adenomas in patients with glycogen storage disease type I - atypical magnetic resonance imaging findings: Two case reports. 61
33585634 2021
30
Retiform hemangioendothelioma: a case series and review of the literature. 61
33593408 2021
31
Sirolimus for patients with progressive epithelioid hemangioendothelioma. 61
33107994 2021
32
Novel EGFL7-FOSB fusion in Pseudomyogenic Hemangioendothelioma with Widely Metastatic Disease. 61
33550637 2021
33
[A case of neonatal mesenteric Kaposiform hemangioendothelioma with Kasabach-Merritt phenomenon]. 61
33548963 2021
34
Primary Bone Marrow Epithelioid Hemangioendothelioma Treated With Gemcitabine and Docetaxel. 61
32991259 2021
35
Femoral epithelioid hemangioendothelioma detected with magnetic resonance imaging and positron emission tomography/computed tomography: A case report. 61
33644202 2021
36
Activity of sirolimus in patients with progressive epithelioid hemangioendothelioma: A case-series analysis within the Italian Rare Cancer Network. 61
33107985 2021
37
Epithelioid hemangioendothelioma (EHE) involving pleural effusion. 61
32749764 2021
38
Contemporary Management of Vascular Anomalies of the Head and Neck-Part 1: Vascular Malformations: A Review. 61
33237296 2021
39
Liver Transplantation in a Patient With Hepatic Angiosarcoma. 61
33585099 2021
40
Epithelioid hemangioendothelioma: a rare cause of a liver tumor. 61
33207899 2021
41
Hepatic Epithelioid Hemangioendothelioma Presenting Synchronously with Hepatocellular Carcinoma. 61
33790724 2021
42
Case Report: The Complete Remission of a Mixed Germ Cell Tumor With Somatic Type Malignancy of Sarcoma Type With a GCT-Oriented Therapy: Clinical Findings and Genomic Profiling. 61
33796464 2021
43
Hepatic hemangioendothelioma of infancy: clinical features of a large cohort of patients and proposed management. 61
33515109 2021
44
Epithelioid hemangioma of bone harboring FOS and FOSB gene rearrangements: A clinicopathologic and molecular study. 61
33034932 2021
45
A rare cardiac tumor presenting with myxoma: Primary cardiac hemangioendothelioma. 61
33768989 2021
46
The 2020 WHO Classification: What's New in Soft Tissue Tumor Pathology? 61
32796172 2021
47
Use of Sirolimus in a Premature Neonate With Kaposiform Hemangioedema. 61
33603586 2021
48
Epithelioid Hemangioendothelioma and Epithelioid Hemangioma: Pazopanib as a Potential Salvage Therapy. 61
33776722 2021
49
Clinical Characteristics and Prognostic Factors in Pediatric Vascular Tumors. 61
33623471 2021
50
Mitochondria as Target for Tumor Management of Hemangioendothelioma. 61
32597200 2021

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 hemostasis GO:0007599 8.96 PLG FLI1
2 tissue homeostasis GO:0001894 8.62 WWTR1 CD34

Sources for Hemangioendothelioma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
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
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Apr-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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