MCID: HMN016
MIFTS: 38

Hemangioendothelioma

Categories: Rare diseases

Aliases & Classifications for Hemangioendothelioma

MalaCards integrated aliases 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 : 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 140)
id Related Disease Score Top Affiliating Genes
1 epithelioid hemangioendothelioma 12.2
2 kaposiform hemangioendothelioma 12.2
3 retiform hemangioendothelioma 11.9
4 malignant epithelioid hemangioendothelioma 11.9
5 composite hemangioendothelioma 11.9
6 spindle cell hemangioma 11.6
7 angioma, tufted 10.8
8 hemangioma-thrombocytopenia syndrome 10.8
9 hepatitis 10.6
10 hemangioma, capillary infantile 10.2 FLT4 KDR
11 sarcoma 10.1
12 hemangioma 10.1
13 epithelioid sarcoma 10.1
14 iliac vein thrombophlebitis 10.1 KDR PLG
15 benign familial neonatal epilepsy 10.0 FLT4 KDR
16 angiosarcoma 10.0
17 mediastinitis 10.0
18 waterhouse-friderichsen syndrome 10.0 FLT4 PECAM1
19 punctate palmoplantar keratoderma type 2 10.0 KDR PECAM1
20 segmental odontomaxillary dysplasia 9.9 KDR PECAM1
21 endotheliitis 9.9
22 myoepithelial carcinoma 9.9 KDR PECAM1
23 lymphoid leukemia 9.9 FLT4 KDR
24 placental choriocarcinoma 9.9 KDR PLG
25 hypothyroidism 9.8
26 cervicitis 9.8
27 thyroiditis 9.8
28 turner syndrome 9.8 PECAM1 PLG
29 subdural empyema 9.8 KDR PLG
30 trachea carcinoma in situ 9.8 FLT4 KDR
31 factitious disorder 9.8 CAMTA1 FLT4 KDR
32 pancreatitis 9.8
33 lymphangiomatosis 9.8
34 pericardial effusion 9.7
35 lymphangioma 9.7
36 cavernous hemangioma 9.7
37 pyogenic granuloma 9.7
38 aneurysm 9.7
39 budd-chiari syndrome 9.6
40 crohn's disease 9.6
41 lymphedema 9.6
42 primary biliary cirrhosis 9.6
43 fibroma 9.6
44 thrombosis 9.6
45 adenocarcinoma 9.6
46 cystic lymphangioma 9.6
47 teratoma 9.6
48 cerebritis 9.6
49 histiocytoma 9.6
50 histiocytoid hemangioma 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 KDR PECAM1 PLG FLI1 FLT4
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)
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
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 Phase 2 1994-09-7, 94-09-7 2337
8
Methylprednisolone Approved, Vet_approved Phase 2 83-43-2 6741
9
Trametinib Approved Phase 2 871700-17-3 11707110
10
Sirolimus Approved, Investigational Phase 2 53123-88-9 5284616 6436030 46835353
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 Anti-Bacterial Agents Phase 2
25 Anti-Infective Agents Phase 2
26 Immunoglobulins Phase 2
27 Antibodies, Monoclonal Phase 2
28 Immunosuppressive Agents Phase 2
29 Prednisolone hemisuccinate Phase 2
30 Prednisolone phosphate Phase 2
31 Anti-Inflammatory Agents Phase 2
32 Antiemetics Phase 2
33 Antifungal Agents Phase 2
34 Protective Agents Phase 2
35 Antibiotics, Antitubercular Phase 2
36 Protein Kinase Inhibitors Phase 2
37 Antimetabolites Phase 2
38 Antibodies Phase 2
39 Antimetabolites, Antineoplastic Phase 2
40 Autonomic Agents Phase 2
41 Antimitotic Agents Phase 2
42 Antineoplastic Agents, Hormonal Phase 2
43 Antineoplastic Agents, Phytogenic 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 11)

id Name Status NCT ID Phase Drugs
1 Evaluation of Lipiodol® Ultra Fluid in Association With Surgical Glues During Vascular Embolization. Not yet 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 Trametinib in Treating Patients With Epithelioid Hemangioendothelioma That Is Metastatic, Locally Advanced, or Cannot Be Removed by Surgery Recruiting NCT03148275 Phase 2 Trametinib
4 Sirolimus Versus Sirolimus Plus Prednisolone for Kaposiform Hemangioendothelioma With Kasabach-Merritt Syndrome Recruiting NCT03188068 Phase 2 Sirolimus;Prednisolone
5 A Study to Compare Vincristine to Sirolimus for Treatment of High Risk Vascular Tumors Recruiting NCT02110069 Phase 2 Vincristine;Sirolimus
6 Gemcitabine Hydrochloride With or Without Pazopanib Hydrochloride in Treating Patients With Refractory Soft Tissue Sarcoma Recruiting NCT01532687 Phase 2 Gemcitabine Hydrochloride;Pazopanib Hydrochloride
7 Multi-Arm Study to Test the Efficacy of Immunotherapeutic Agents in Multiple Sarcoma Subtypes Recruiting NCT02815995 Phase 2 Durvalumab;Tremelimumab
8 Bevacizumab in Treating Patients With Angiosarcoma Active, not recruiting NCT00288015 Phase 2
9 Safety and Efficacy Study of Sirolimus in Complicated Vascular Anomalies Active, not recruiting NCT00975819 Phase 2 sirolimus
10 Identification of Biomarkers for Patients With Vascular Anomalies Recruiting NCT03001180
11 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 932)
id Title Authors Year
1
Clinico-radiological features and next generation sequencing of pulmonary epithelioid hemangioendothelioma: A case report and review of literature. ( 28777494 )
2017
2
Successful Management of Pancreatic Kaposiform Hemangioendothelioma With Sirolimus: Case Report and Literature Review. ( 28426496 )
2017
3
Hepatic Epithelioid Hemangioendothelioma - a Rare Tumor and Diagnostic Dilemma. ( 28652454 )
2017
4
Epithelioid hemangioendothelioma of the spine: case report and review of the literature. ( 28744509 )
2017
5
Primary epithelioid hemangioendothelioma of the dorsal spine: A review. ( 28879934 )
2017
6
TFE3-rearranged hepatic epithelioid hemangioendothelioma-a case report with immunohistochemical and molecular study. ( 28585251 )
2017
7
The clinical features of epithelioid hemangioendothelioma in a Han Chinese population: A retrospective analysis. ( 28658152 )
2017
8
Intestinal obstruction due to kaposiform hemangioendothelioma in a 1-month-old infant: A case report. ( 28906349 )
2017
9
Long-term outcome for kaposiform hemangioendothelioma: A report of two cases. ( 27701822 )
2017
10
Malignant Hepatic Epithelioid Hemangioendothelioma with Villaret Syndrome. ( 28348474 )
2017
11
(18)F-FDG PET/CT in Pleural Epithelioid Hemangioendothelioma. ( 28840142 )
2017
12
Aggressive primary hepatic epithelioid hemangioendothelioma: a case report and literature review. ( 28607810 )
2017
13
New Insights about Pulmonary Epithelioid Hemangioendothelioma: Review of the Literature and Two Case Reports. ( 28884037 )
2017
14
Epithelioid hemangioendothelioma presenting as fever of unknown origin. ( 28507590 )
2017
15
68Ga-DOTA-TATE PET/CT for Molecular Imaging of Somatostatin Receptor Expression in Metastasizing Epithelioid Hemangioendothelioma: Comparison With 18F-FDG. ( 28872546 )
2017
16
Sirolimus for the treatment of progressive kaposiform hemangioendothelioma: A multicenter retrospective study. ( 28486787 )
2017
17
Integrin I^3/Akt signaling contributes to platelet-induced hemangioendothelioma growth. ( 28744026 )
2017
18
Massive retiform hemangioendothelioma that expresses D2-40. ( 28366927 )
2017
19
Composite hemangioendothelioma with neuroendocrine marker expression: an aggressive variant. ( 28731049 )
2017
20
Multicentric visceral epithelioid hemangioendothelioma, with extremity dermal deposits, unusual late recurrence on the nasal bridge and TFE3 gene rearrangement. ( 28855107 )
2017
21
Epithelioid hemangioendothelioma of the craniocervical junction; case report and review. ( 28478911 )
2017
22
Cutaneous epithelioid hemangioendothelioma on the sole mimicking a pyogenic granuloma. ( 28664615 )
2017
23
MicroRNA profile of hepatic epithelioid hemangioendothelioma: A case report. ( 28454305 )
2017
24
Successful treatment of a hepatic-hemangioendothelioma infant presenting with hypothyroidism and tetralogy of Fallot. ( 28838853 )
2017
25
Kaposiform hemangioendothelioma with Kasabach-Merritt syndrome: successful treatment with sirolimus. ( 28220608 )
2017
26
Imaging findings of Kaposiform Hemangioendothelioma in children. ( 28027747 )
2017
27
18-F-FDG PET-CT in Monitoring of Chemotherapeutic Effect in a Case of Metastatic Hepatic Epithelioid Hemangioendothelioma. ( 28680215 )
2017
28
Magnetic resonance findings of hepatic epithelioid hemangioendothelioma: emphasis on hepatobiliary phase using Gd-EOB-DTPA. ( 28365784 )
2017
29
Atypical retiform hemangioendothelioma arising in a patient with Milroy disease: a case report and review of the literature. ( 27730656 )
2017
30
Sirolimus therapy in the treatment of pseudomyogenic hemangioendothelioma. ( 28843050 )
2017
31
Pancreatic Kaposiform Hemangioendothelioma Not Responding to Sirolimus. ( 28761800 )
2017
32
An Epithelioid Hemangioendothelioma of the Head and Neck. ( 28834832 )
2017
33
Incidental Detection of Retiform Hemangioendothelioma by 68Ga DOTATOC PET/CT. ( 27997421 )
2017
34
Hemangioendothelioma of the Bladder: The Youngest Case Report in A Child. ( 28752069 )
2017
35
Identical Presentation of Scapular Osteolysis in Two Patients with Thoracic Kaposiform Hemangioendothelioma. ( 28382716 )
2017
36
Kaposiform Hemangioendothelioma Presenting as Hydrops Fetalis. ( 28318049 )
2017
37
Beta blocker and steroid therapy in the treatment of infantile hepatic hemangioendothelioma. ( 28652510 )
2017
38
Pseudomyogenic hemangioendothelioma secondary to fibrous dysplasia of the left lower extremity in a 14-year-old female: a case report. ( 27468782 )
2016
39
Mediastinal Epithelioid Hemangioendothelioma. ( 26649628 )
2016
40
Infantile Hepatic Hemangioendothelioma: An Uncommon Cause of Persistent Pulmonary Hypertension in a Newborn Infant. ( 27468364 )
2016
41
Interferon-alpha therapy for refractory kaposiform hemangioendothelioma: a single-center experience. ( 27796340 )
2016
42
Clinicopathological Features and Prognosis of Hepatic Epithelioid Hemangioendothelioma After Liver Resection and Transplantation. ( 28031549 )
2016
43
A Case of an Epithelioid Hemangioendothelioma Arising from the Innominate Vein Mimicking Cervical Metastatic Lymphadenopathy. ( 28058125 )
2016
44
Sirolimus in Advanced Epithelioid Hemangioendothelioma: A Retrospective Case-Series Analysis from the Italian Rare Cancer Network Database. ( 27334221 )
2016
45
Sorafenib Monotherapy in a Patient with Unresectable Hepatic Epithelioid Hemangioendothelioma. ( 27293400 )
2016
46
Ambiguous presentations of pulmonary epithelioid hemangioendothelioma: Two case reports of a rare pulmonary malignancy. ( 27489719 )
2016
47
Neonatal kaposiform hemangioendothelioma of the spleen associated with Kasabach-Merritt phenomenon. ( 27342010 )
2016
48
Treatment of kaposiform hemangioendothelioma and tufted angioma. ( 27252149 )
2016
49
Recurrent multifocal cutaneous Kaposiform hemangioendothelioma: A rare vascular tumor of infancy and childhood. ( 27510687 )
2016
50
Retiform hemangioendothelioma in the infratemporal fossa and buccal area: a case report and literature review. ( 27847741 )
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 HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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