MCID: HMN016
MIFTS: 39

Hemangioendothelioma malady

Categories: Rare diseases

Aliases & Classifications for Hemangioendothelioma

Aliases & Descriptions for Hemangioendothelioma:

Name: Hemangioendothelioma 50 52 69

Classifications:



Summaries for Hemangioendothelioma

NIH Rare Diseases : 50 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, lung and bone, and related phenotypes are cardiovascular system and immune system

Wikipedia : 71 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 138)
id Related Disease Score Top Affiliating Genes
1 epithelioid hemangioendothelioma 12.3
2 kaposiform hemangioendothelioma 12.0
3 retiform hemangioendothelioma 12.0
4 composite hemangioendothelioma 12.0
5 spindle cell hemangioma 11.7
6 angioma, tufted 10.8
7 hemangioma-thrombocytopenia syndrome 10.8
8 hepatitis 10.5
9 sarcoma 10.1
10 hemangioma 10.1
11 pustulosis palmaris et plantaris 10.1 KDR PECAM1
12 benign idiopathic neonatal seizures 10.1 KDR PECAM1
13 epithelioid sarcoma 10.1
14 hand, foot and mouth disease 10.0 KDR PLG
15 charcot-marie-tooth disease, axonal, type 2f 10.0 KDR PECAM1
16 gapo syndrome 10.0 FLT4 KDR
17 eosinophilic pneumonia 10.0 FLT4 KDR
18 mediastinitis 10.0
19 angiosarcoma 10.0
20 lymph node disease 10.0 FLT4 PECAM1
21 mixed connective tissue disease 10.0 PECAM1 PLG
22 benign familial neonatal epilepsy 10.0 FLT4 KDR
23 subdural empyema 10.0 KDR PLG
24 neuropathy with hearing impairment 9.9 CAMTA1 KDR PECAM1
25 endotheliitis 9.9
26 lymphoma, large-cell, immunoblastic 9.9 FLT4 KDR
27 metagonimiasis 9.9 FLT4 KDR
28 factitious disorder 9.9 CAMTA1 FLT4 KDR
29 vesiculobullous skin disease 9.9 FLT4 KDR PECAM1
30 hemolytic anemia 9.8 FLT4 KDR PECAM1
31 thyroiditis 9.8
32 cervicitis 9.8
33 vernal conjunctivitis 9.8 KDR PLG
34 hypothyroidism 9.8
35 lymphangiomatosis 9.8
36 lymphangioma 9.7
37 cavernous hemangioma 9.7
38 pericardial effusion 9.7
39 aneurysm 9.7
40 budd-chiari syndrome 9.6
41 crohn's disease 9.6
42 cerebritis 9.6
43 hepatoblastoma 9.6
44 thrombosis 9.6
45 lymphedema 9.6
46 histiocytoma 9.6
47 primary biliary cirrhosis 9.6
48 histiocytoid hemangioma 9.6
49 adenocarcinoma 9.6
50 pyogenic granuloma 9.6

Graphical network of the top 20 diseases related to Hemangioendothelioma:



Diseases related to Hemangioendothelioma

Symptoms & Phenotypes for Hemangioendothelioma

MGI Mouse Phenotypes related to Hemangioendothelioma:

44
id Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 9.65 PECAM1 PLG FLI1 FLT4 KDR
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 29)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1
Ethiodized oil Approved 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
Miconazole Approved, Investigational, Vet_approved Phase 2 22916-47-8 4189
5
Everolimus Approved Phase 2 159351-69-6 6442177
6
Benzocaine Approved Phase 2 1994-09-7, 94-09-7 2337
7
Trametinib Approved Phase 2 871700-17-3 11707110
8
Sirolimus Approved, Investigational Phase 2 53123-88-9 5284616 6436030 46835353
9
Bevacizumab Approved, Investigational Phase 2 216974-75-3
10 tannic acid Approved, Nutraceutical Phase 2
11
tremelimumab Phase 2
12 Anti-Bacterial Agents Phase 2
13 Immunoglobulins Phase 2
14 Anti-Infective Agents Phase 2
15 Antibodies, Monoclonal Phase 2
16 Immunosuppressive Agents Phase 2
17 Protein Kinase Inhibitors Phase 2
18 Antifungal Agents Phase 2
19 Antimetabolites Phase 2
20 Antibiotics, Antitubercular Phase 2
21 Antimetabolites, Antineoplastic Phase 2
22 Antibodies Phase 2
23 Antimitotic Agents Phase 2
24 Antineoplastic Agents, Phytogenic Phase 2
25 Antiviral Agents Phase 2
26 Angiogenesis Inhibitors Phase 2
27 Angiogenesis Modulating Agents Phase 2
28 Mitogens
29 Endothelial Growth Factors

Interventional clinical trials:


id Name Status NCT ID Phase
1 Evaluation of Lipiodol® Ultra Fluid in Association With Surgical Glues During Vascular Embolization. Not yet recruiting NCT02625389 Phase 4
2 Vismodegib and Gamma-Secretase/Notch Signalling Pathway Inhibitor RO4929097 in Treating Patients With Advanced or Metastatic Sarcoma Completed NCT01154452 Phase 1, Phase 2
3 Trametinib in Treating Patients With Epithelioid Hemangioendothelioma That Is Metastatic, Locally Advanced, or Cannot Be Removed by Surgery Recruiting NCT03148275 Phase 2
4 A Study to Compare Vincristine to Sirolimus for Treatment of High Risk Vascular Tumors Recruiting NCT02110069 Phase 2
5 Gemcitabine Hydrochloride With or Without Pazopanib Hydrochloride in Treating Patients With Refractory Soft Tissue Sarcoma Recruiting NCT01532687 Phase 2
6 Multi-Arm Study to Test the Efficacy of Immunotherapeutic Agents in Multiple Sarcoma Subtypes Recruiting NCT02815995 Phase 2
7 Bevacizumab in Treating Patients With Angiosarcoma Active, not recruiting NCT00288015 Phase 2
8 Safety and Efficacy Study of Sirolimus in Complicated Vascular Anomalies Active, not recruiting NCT00975819 Phase 2
9 Identification of Biomarkers for Patients With Vascular Anomalies Recruiting NCT03001180
10 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:

39
Liver, Lung, Bone, Endothelial, Skin, Lymph Node, Heart

Publications for Hemangioendothelioma

Articles related to Hemangioendothelioma:

(show top 50) (show all 896)
id Title Authors Year
1
Incidental Detection of Retiform Hemangioendothelioma by 68Ga DOTATOC PET/CT. ( 27997421 )
2017
2
Atypical retiform hemangioendothelioma arising in a patient with Milroy disease: a case report and review of the literature. ( 27730656 )
2017
3
Long-term outcome for kaposiform hemangioendothelioma: A report of two cases. ( 27701822 )
2017
4
Imaging findings of Kaposiform Hemangioendothelioma in children. ( 28027747 )
2017
5
Kaposiform Hemangioendothelioma in a 3A Months Old Infant. ( 27408442 )
2016
6
Role of Sirolimus in Advanced Kaposiform Hemangioendothelioma. ( 26864138 )
2016
7
Neonatal kaposiform hemangioendothelioma of the spleen associated with Kasabach-Merritt phenomenon. ( 27342010 )
2016
8
A Case of an Epithelioid Hemangioendothelioma Arising from the Innominate Vein Mimicking Cervical Metastatic Lymphadenopathy. ( 28058125 )
2016
9
Hypercalcemia related to PTH-rP revealing malignant hepatic epithelioid hemangioendothelioma. ( 26878613 )
2016
10
Primary Hepatic Epithelioid Hemangioendothelioma: Diagnostic Dilemmas in Cytology and Histology. ( 27072389 )
2016
11
Sirolimus for treatment of kaposiform hemangioendothelioma associated with Kasabach-Merritt phenomenon. ( 27981218 )
2016
12
Pulmonary hemangioendothelioma with osteoclast-like giant cells: A rare observation. ( 27510689 )
2016
13
P2.24: Pulmonary-Epithelioid Hemangioendothelioma: A Case ReportA of Spontaneous Regression: Track: Supportive Care and Others. ( 27676560 )
2016
14
Sorafenib Monotherapy in a Patient with Unresectable Hepatic Epithelioid Hemangioendothelioma. ( 27293400 )
2016
15
A Rare Case of Pulmonary Epithelioid Hemangioendothelioma Presenting with Skin Metastasis. ( 27218028 )
2016
16
Multifocal Polymorphous Hemangioendothelioma of the Liver: Case Report and Review of Literature. ( 27913780 )
2016
17
Straddling across the neural foramina with a leash of blood vessels: Mason's vegetant intravascular hemangioendothelioma mimicking a schwannoma. ( 26954824 )
2016
18
Composite Hemangioendothelioma: Report of a Rare Case. ( 27891348 )
2016
19
Epithelioid hemangioendothelioma of hypopharynx: A rare presentation. ( 27433056 )
2016
20
Epithelioid hemangioendothelioma in the thorax: Clinicopathologic, CT, PET, and prognostic features. ( 27472721 )
2016
21
Bullous Kaposiform Hemangioendothelioma Masquerading as Aplasia Cutis Congenita. ( 27339319 )
2016
22
Recurrence of Epithelioid Hemangioendothelioma during Pregnancy: Case Report and Systematic Review. ( 27479949 )
2016
23
A rare case of multifocal pseudomyogenic hemangioendothelioma, involving soft tissues and bone, misdiagnosed as a rhabdomyosarcoma: Diagnostic and treatment implications. ( 27510684 )
2016
24
Kaposiform Hemangioendothelioma: Multifocal Involvement, Chylothorax, and Kasabach-Merritt Phenomenon. ( 27820128 )
2016
25
Hepatic epithelioid hemangioendothelioma: a report from three university centers. ( 27818541 )
2016
26
Pseudomyogenic Hemangioendothelioma: A Vascular Tumor Previously Undescribed in the Oral Cavity. ( 27878449 )
2016
27
Sirolimus in Advanced Epithelioid Hemangioendothelioma: A Retrospective Case-Series Analysis from the Italian Rare Cancer Network Database. ( 27334221 )
2016
28
Treatment of kaposiform hemangioendothelioma and tufted angioma. ( 27252149 )
2016
29
Pleural Epithelioid Hemangioendothelioma: A Case Report and Literature Review. ( 27372473 )
2016
30
Recurrent multifocal cutaneous Kaposiform hemangioendothelioma: A rare vascular tumor of infancy and childhood. ( 27510687 )
2016
31
Primary Pseudomyogenic Hemangioendothelioma of Bone. ( 26872012 )
2016
32
Infantile Hepatic Hemangioendothelioma: An Uncommon Cause of Persistent Pulmonary Hypertension in a Newborn Infant. ( 27468364 )
2016
33
Epithelioid hemangioendotheliomas with TFE3 gene translocations are compossible with CAMTA1 gene rearrangements. ( 26840265 )
2016
34
Successful Propranolol Treatment of a Kaposiform Hemangioendothelioma Apparently Resistant to Propranolol. ( 27100060 )
2016
35
Typical CT and MRI signs of hepatic epithelioid hemangioendothelioma. ( 26998064 )
2016
36
Abdominal Kaposiform Hemangioendothelioma Associated With Lymphangiomatosis Involving Mesentery and Ileum: A Case Report of MRI, CT, and 18F-FDG PET/CT Findings. ( 26871848 )
2016
37
Interferon-alpha therapy for refractory kaposiform hemangioendothelioma: a single-center experience. ( 27796340 )
2016
38
Epithelioid Hemangioendothelioma: a Rare Primary Thyroid Tumor with Confirmation of WWTR1 and CAMTA1 Rearrangements. ( 27026033 )
2016
39
Pseudomyogenic hemangioendothelioma secondary to fibrous dysplasia of the left lower extremity in a 14-year-old female: a case report. ( 27468782 )
2016
40
Developing a Nanoparticle-Delivered High-Efficacy Treatment for Infantile Hemangiomas Using a Mouse Hemangioendothelioma Model. ( 27465164 )
2016
41
Successful Everolimus Treatment of Kaposiform Hemangioendothelioma With Kasabach-Merritt Phenomenon: Clinical Efficacy and Adverse Effects of mTOR Inhibitor Therapy. ( 26907642 )
2016
42
Epithelioid sarcoma-like hemangioendothelioma on the chest wall. ( 27493191 )
2016
43
Diagnostic utility of FOSB immunohistochemistry in pseudomyogenic hemangioendothelioma and its histological mimics. ( 27515856 )
2016
44
Radiographic and Histologic Findings of a Rare Liver Tumor-Hepatic Epithelioid Hemangioendothelioma. ( 26809872 )
2016
45
Computed tomography and magnetic resonance features of intracranial hemangioendothelioma: A study of 7 cases. ( 27123072 )
2016
46
Retiform hemangioendothelioma in the infratemporal fossa and buccal area: a case report and literature review. ( 27847741 )
2016
47
FOSB is a Useful Diagnostic Marker for Pseudomyogenic Hemangioendothelioma. ( 28009608 )
2016
48
Retiform hemangioendothelioma: A rare entity at atypical site. ( 27297277 )
2016
49
Infantile rhabdoid tumor mimicking hepatic hemangioendothelioma. ( 27072279 )
2016
50
Long term follow up after resection emphasizes the role of surgery in Primary Hepatic Epithelioid Hemangioendothelioma. ( 27594996 )
2016

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:

id 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:

id 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
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 MedGen
42 MeSH
43 MESH via Orphanet
44 MGI
46 NCI
47 NCIt
48 NDF-RT
51 NINDS
52 Novoseek
54 OMIM
55 OMIM via Orphanet
59 PubMed
60 QIAGEN
65 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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