MCID: HMN016
MIFTS: 43

Hemangioendothelioma

Categories: Rare diseases

Aliases & Classifications for Hemangioendothelioma

MalaCards integrated aliases for Hemangioendothelioma:

Name: Hemangioendothelioma 53 55 73

Classifications:



External Ids:

UMLS 73 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 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 MAPK Signaling: Mitogen Stimulation Pathway. The drugs Ethiodized oil and Gemcitabine 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 : 76 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 151)
# Related Disease Score Top Affiliating Genes
1 epithelioid hemangioendothelioma 32.7 CAMTA1 KDR PECAM1
2 kaposiform hemangioendothelioma 31.2 FLI1 FLT4 KDR PECAM1
3 lymphangioma 29.7 FLT4 KDR
4 capillary hemangioma 29.6 KDR PECAM1
5 hemangioma 29.4 FLT4 KDR PECAM1
6 angiosarcoma 27.8 FLI1 FLT4 KDR PECAM1
7 retiform hemangioendothelioma 12.1
8 malignant epithelioid hemangioendothelioma 12.1
9 composite hemangioendothelioma 12.1
10 spindle cell hemangioma 11.7
11 hemangioma-thrombocytopenia syndrome 11.3
12 angioma, tufted 11.3
13 hepatitis 10.7
14 sarcoma 10.2
15 epithelioid sarcoma 10.2
16 mediastinitis 10.1
17 hemangioma, capillary infantile 10.1 FLT4 KDR
18 endotheliitis 10.0
19 cervicitis 10.0
20 hypothyroidism 9.9
21 thyroiditis 9.9
22 pulmonary vein stenosis 9.9 KDR PECAM1
23 hydrocele 9.9 FLT4 PECAM1
24 breast carcinoma in situ 9.9 FLT4 KDR
25 pancreatitis 9.8
26 lymphangiomatosis 9.8
27 pyogenic granuloma 9.8
28 radiation proctitis 9.8 KDR PECAM1
29 degeneration of macula and posterior pole 9.8 KDR PLG
30 budd-chiari syndrome 9.8
31 lymphedema 9.8
32 hemopericardium 9.8
33 pericardial effusion 9.8
34 cavernous hemangioma 9.8
35 aneurysm 9.8
36 plague 9.8 PECAM1 PLG
37 corneal neovascularization 9.7 KDR PLG
38 biliary cirrhosis, primary, 1 9.7
39 hydrops fetalis, nonimmune, and/or atrial septal defect 9.7
40 crohn's disease 9.7
41 primary biliary cirrhosis 9.7
42 thrombosis 9.7
43 adenocarcinoma 9.7
44 cystic lymphangioma 9.7
45 teratoma 9.7
46 cerebritis 9.7
47 histiocytoma 9.7
48 histiocytoid hemangioma 9.7
49 pneumonia 9.7
50 hepatoblastoma 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:

46
# 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 KDR PECAM1 PLG FLI1 FLT4
3 liver/biliary system MP:0005370 9.35 PECAM1 PLG FLI1 FLT4 KDR
4 muscle MP:0005369 8.92 KDR PECAM1 FLI1 FLT4

Drugs & Therapeutics for Hemangioendothelioma

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

(show all 46)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Ethiodized oil Approved, Investigational Phase 4 8008-53-5
2
Gemcitabine Approved Phase 2 95058-81-4 60750
3
Vincristine Approved, Investigational Phase 2 2068-78-2, 57-22-7 5978
4
Miconazole Approved, Investigational, Vet_approved Phase 2 22916-47-8 4189
5
Prednisolone Approved, Vet_approved Phase 2 50-24-8 5755
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
Pembrolizumab Approved Phase 2 1374853-91-4
10
Trametinib Approved Phase 2 871700-17-3 11707110
11
Sirolimus Approved, Investigational Phase 2 53123-88-9 5284616 6436030 46835353
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 Antibiotics, Antitubercular Phase 2
26 Antibodies Phase 2
27 Immunoglobulins Phase 2
28 Antibodies, Monoclonal Phase 2
29 Immunosuppressive Agents Phase 2
30 Prednisolone hemisuccinate Phase 2
31 Prednisolone phosphate Phase 2
32 Anti-Infective Agents Phase 2
33 Antiemetics Phase 2
34 Antifungal Agents Phase 2
35 Protective Agents Phase 2
36 Protein Kinase Inhibitors Phase 2
37 Anti-Inflammatory Agents Phase 2
38 Antimetabolites Phase 2
39 Antiviral Agents Phase 2
40 Autonomic Agents Phase 2
41 Antimetabolites, Antineoplastic Phase 2
42 Antimitotic Agents Phase 2
43 Antineoplastic Agents, Hormonal Phase 2
44 Antineoplastic Agents, Phytogenic Phase 2
45 Mitogens
46 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 Sirolimus Plus Propranolol Versus Sirolimus Plus Prednisolone for Kaposiform Hemangioendothelioma Recruiting NCT03188068 Phase 2 Sirolimus;Prednisolone;Propranolol
5 Trametinib in Treating Patients With Epithelioid Hemangioendothelioma That Is Metastatic, Locally Advanced, or Cannot Be Removed by Surgery Recruiting NCT03148275 Phase 2 Trametinib
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 A Study of Epacadostat, an IDO1 Inhibitor, in Combination With Pembrolizumab in Patients With Metastatic and/or Locally Advanced Sarcoma Recruiting NCT03414229 Phase 2 Epacadostat;Pembrolizumab
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:

41
Liver, Bone, Lung, Endothelial, Skin, Lymph Node, Heart

Publications for Hemangioendothelioma

Articles related to Hemangioendothelioma:

(show top 50) (show all 973)
# Title Authors Year
1
Unicentric epithelioid hemangioendothelioma of the calcaneus: a case report and review of literature. ( 29632658 )
2018
2
Sirolimus for treatment of Kaposiform hemangioendothelioma with Kasabach-Merritt phenomenon: A retrospective cohort study. ( 29388191 )
2018
3
Giant Cervical and Mediastinal Hemangioendothelioma Arising from Subclavian Vein. ( 29503064 )
2018
4
Clinical and imagingA features ofA Kaposiform Hemangioendothelioma. ( 29536769 )
2018
5
Cytologic features, immunocytochemical findings, and DNA ploidy in four rare cases of epithelioid hemangioendothelioma involving effusions. ( 29937917 )
2018
6
Pseudomyogenic Hemangioendothelioma: Case Report and Review of the Literature. ( 29406432 )
2018
7
Impact of sirolimus treatment for refractory kaposiform hemangioendothelioma with exacerbation of the disease 10a88years after initial diagnosis. ( 29872523 )
2018
8
Composite Hemangioendothelioma Settled in the Paraspinal Region: A Rare Case Report. ( 29757449 )
2018
9
Hobnail (retiform-Dabska) hemangioendothelioma located in the jejunum. ( 29453774 )
2018
10
Diagnosing Epithelioid Hemangioendothelioma with Pericardial Involvement. ( 29689240 )
2018
11
Association between extremity kaposiform hemangioendothelioma and lymphedema. ( 29266383 )
2018
12
Atypically presenting kaposiform hemangioendothelioma of the knee: ultrasound findings. ( 29637402 )
2018
13
Diffuse hepatic epithelioid hemangioendothelioma with multiple splenic metastasis and delayed multifocal bone metastasis after liver transplantation on FDG PET/CT images: A case report. ( 29851777 )
2018
14
Telatinib Is an Effective Targeted Therapy for Pseudomyogenic Hemangioendothelioma. ( 29511030 )
2018
15
Regional Differences in Clinical Features of Kaposiform Hemangioendothelioma of the Intestinal Tract. ( 29554025 )
2018
16
Massive Epithelioid Hemangioendothelioma Causing Noncirrhotic Portal Hypertension. ( 29609064 )
2018
17
Hepatic Epithelioid Hemangioendothelioma. ( 29372848 )
2018
18
Hepatic epithelioid hemangioendothelioma: A misdiagnosed rare liver tumor. ( 29373279 )
2018
19
Vascular Tumors in Infants: Case Report and Review of Clinical, Histopathologic, and Immunohistochemical Characteristics of Infantile Hemangioma, Pyogenic Granuloma, Noninvoluting Congenital Hemangioma, Tufted Angioma, and Kaposiform Hemangioendothelioma. ( 29561329 )
2018
20
<i>Pneumocystis Jirovecii</i> Pneumonia During Sirolimus Therapy for Kaposiform Hemangioendothelioma. ( 29610164 )
2018
21
Hepatic Epithelioid Hemangioendothelioma Presenting as an Enlarging Vascular Lesion within the Spleen. ( 29850364 )
2018
22
Epithelioid Hemangioendothelioma: Update on Diagnosis and Treatment. ( 29546487 )
2018
23
Pseudomyogenic hemangioendothelioma of skin, bone and soft tissue-a clinicopathological, immunohistochemical, and fluorescence in situ hybridization study. ( 29104110 )
2018
24
Epithelioid hemangioendothelioma, an uncommon tumor of the eyelid: A case report. ( 29030282 )
2018
25
Retiform hemangioendothelioma presenting as a pedunculated nodule on the site of an inguinal pyoderma chronica. ( 29756229 )
2018
26
Composite hemangioendothelioma with neuroendocrine marker expression: an aggressive variant. ( 28972580 )
2017
27
Intravascular epithelioid hemangioendothelioma of the femoral vein diagnosed by contrast-enhanced ultrasonography: A care-compliant case report. ( 29245342 )
2017
28
18-F-FDG PET-CT in Monitoring of Chemotherapeutic Effect in a Case of Metastatic Hepatic Epithelioid Hemangioendothelioma. ( 28680215 )
2017
29
Successful treatment of a hepatic-hemangioendothelioma infant presenting with hypothyroidism and tetralogy of Fallot. ( 28838853 )
2017
30
Primary epithelioid hemangioendothelioma of the dorsal spine: A review. ( 28879934 )
2017
31
New Insights about Pulmonary Epithelioid Hemangioendothelioma: Review of the Literature and Two Case Reports. ( 28884037 )
2017
32
Pulmonary epithelioid hemangioendothelioma imitating lung cancer. ( 29181052 )
2017
33
Successful Management of Pancreatic Kaposiform Hemangioendothelioma With Sirolimus: Case Report and Literature Review. ( 28426496 )
2017
34
Epithelioid hemangioendothelioma of the craniocervical junction; case report and review. ( 28478911 )
2017
35
Composite hemangioendothelioma with neuroendocrine marker expression: an aggressive variant. ( 28731049 )
2017
36
Malignant Hepatic Epithelioid Hemangioendothelioma with Villaret Syndrome. ( 28348474 )
2017
37
Kaposiform hemangioendothelioma in an adult with rheumatoid arthritis. ( 29154372 )
2017
38
Pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma - a rare vascular neoplasm with deceptive morphology and distinctive immunophenotype. ( 29279595 )
2017
39
Integrin I^3/Akt signaling contributes to platelet-induced hemangioendothelioma growth. ( 28744026 )
2017
40
Sirolimus therapy in the treatment of pseudomyogenic hemangioendothelioma. ( 28843050 )
2017
41
An Epithelioid Hemangioendothelioma of the Head and Neck. ( 28834832 )
2017
42
Cutaneous epithelioid hemangioendothelioma on the sole mimicking a pyogenic granuloma. ( 28664615 )
2017
43
Multicentric visceral epithelioid hemangioendothelioma, with extremity dermal deposits, unusual late recurrence on the nasal bridge and TFE3 gene rearrangement. ( 28855107 )
2017
44
Primary hepatic hemangioendothelioma in a patient with Budd-Chiari syndrome. ( 29054942 )
2017
45
Sirolimus for the treatment of progressive kaposiform hemangioendothelioma: A multicenter retrospective study. ( 28486787 )
2017
46
Identical Presentation of Scapular Osteolysis in Two Patients with Thoracic Kaposiform Hemangioendothelioma. ( 28382716 )
2017
47
Long-term outcome for kaposiform hemangioendothelioma: A report of two cases. ( 27701822 )
2017
48
Intestinal obstruction due to kaposiform hemangioendothelioma in a 1-month-old infant: A case report. ( 28906349 )
2017
49
The clinical features of epithelioid hemangioendothelioma in a Han Chinese population: A retrospective analysis. ( 28658152 )
2017
50
Pancreatic Kaposiform Hemangioendothelioma Not Responding to Sirolimus. ( 28761800 )
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 kinase activity GO:0033674 9.43 FLT4 KDR
2 vascular endothelial growth factor receptor signaling pathway GO:0048010 9.4 FLT4 KDR
3 positive regulation of endothelial cell proliferation GO:0001938 9.37 FLT4 KDR
4 vasculogenesis GO:0001570 9.32 FLT4 KDR
5 hemostasis GO:0007599 9.26 FLI1 PLG
6 positive regulation of endothelial cell migration GO:0010595 9.16 FLT4 KDR
7 cellular response to vascular endothelial growth factor stimulus GO:0035924 8.96 FLT4 KDR
8 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 MAP kinase kinase kinase activity GO:0004709 9.32 FLT4 KDR
2 mitogen-activated protein kinase kinase binding GO:0031434 9.26 FLT4 KDR
3 transmembrane receptor protein tyrosine kinase activity GO:0004714 9.16 FLT4 KDR
4 growth factor binding GO:0019838 8.96 FLT4 KDR
5 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
17 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 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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