MCID: HMN016
MIFTS: 44

Hemangioendothelioma

Categories: Cancer diseases, Cardiovascular diseases, 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 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 Prednisolone phosphate 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 149)
# Related Disease Score Top Affiliating Genes
1 epithelioid hemangioendothelioma 33.9 CAMTA1 KDR
2 malignant epithelioid hemangioendothelioma 33.2 CAMTA1 FLT4 KDR
3 kaposiform hemangioendothelioma 33.0 FLI1 FLT4 KDR PECAM1
4 hemangioma 30.2 FLT4 KDR PECAM1
5 lymphangioma 29.9 FLT4 KDR
6 capillary hemangioma 29.8 KDR PECAM1
7 angiosarcoma 29.7 FLI1 FLT4 KDR PECAM1
8 cavernous hemangioma 29.5 KDR PECAM1
9 retiform hemangioendothelioma 12.2
10 composite hemangioendothelioma 12.2
11 hemangioma-thrombocytopenia syndrome 11.4
12 angioma, tufted 11.4
13 spindle cell hemangioma 11.4
14 sarcoma 10.4
15 epithelioid sarcoma 10.3
16 hypothyroidism 10.2
17 hydrops fetalis, nonimmune, and/or atrial septal defect 10.1
18 fetal edema 10.1
19 hydrops fetalis 10.1
20 hepatoblastoma 10.0
21 lymphangiomatosis 10.0
22 pyogenic granuloma 10.0
23 hemangioma, capillary infantile 10.0 FLT4 KDR
24 pulmonary vein stenosis 10.0 KDR PECAM1
25 breast carcinoma in situ 10.0 FLT4 KDR
26 radiation proctitis 9.9 KDR PECAM1
27 budd-chiari syndrome 9.9
28 crohn's disease 9.9
29 lymphedema 9.9
30 hemopericardium 9.9
31 pericardial effusion 9.9
32 teratoma 9.9
33 plague 9.9 PECAM1 PLG
34 corneal neovascularization 9.9 KDR PLG
35 malignant hemangioma 9.9 CAMTA1 FLT4 KDR
36 biliary cirrhosis, primary, 1 9.9
37 lung cancer 9.9
38 hemangiopericytoma, malignant 9.9
39 multiple enchondromatosis, maffucci type 9.9
40 congestive heart failure 9.9
41 primary biliary cirrhosis 9.9
42 colitis 9.9
43 thrombosis 9.9
44 arteriovenous malformation 9.9
45 adenocarcinoma 9.9
46 cystic lymphangioma 9.9
47 histiocytoma 9.9
48 spindle cell sarcoma 9.9
49 histiocytoid hemangioma 9.9
50 pneumonia 9.9

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 61)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Ethiodized oil Approved, Investigational Phase 4 8008-53-5
2
Prednisolone phosphate Approved, Vet_approved Phase 2 302-25-0
3
Methylprednisolone Approved, Vet_approved Phase 2 83-43-2 6741
4
Prednisolone Approved, Vet_approved Phase 2 50-24-8 5755
5
Methylprednisolone hemisuccinate Approved Phase 2 2921-57-5
6
Everolimus Approved Phase 2 159351-69-6 6442177
7
Sirolimus Approved, Investigational Phase 2 53123-88-9 46835353 6436030 5284616
8
Miconazole Approved, Investigational, Vet_approved Phase 2 22916-47-8 4189
9
Propranolol Approved, Investigational Phase 2 525-66-6 4946
10
Trametinib Approved Phase 2 871700-17-3 11707110
11
Vincristine Approved, Investigational Phase 2 57-22-7, 2068-78-2 5978
12
Benzocaine Approved, Investigational Phase 2 94-09-7, 1994-09-7 2337
13 tannic acid Approved Phase 2
14
Gemcitabine Approved Phase 2 95058-81-4 60750
15
Pembrolizumab Approved Phase 2 1374853-91-4
16
Prednisolone hemisuccinate Experimental Phase 2 2920-86-7
17
Tremelimumab Investigational Phase 2 745013-59-6
18 Autonomic Agents Phase 2
19 Hormones Phase 2
20 Antihypertensive Agents Phase 2
21 Hormone Antagonists Phase 2
22 Anti-Arrhythmia Agents Phase 2
23 Immunosuppressive Agents Phase 2
24 Antineoplastic Agents, Hormonal Phase 2
25 Adrenergic Antagonists Phase 2
26 Anti-Infective Agents Phase 2
27 Adrenergic beta-Antagonists Phase 2
28 Immunologic Factors Phase 2
29 Antiemetics Phase 2
30 Adrenergic Agents Phase 2
31 Protective Agents Phase 2
32 Prednisolone acetate Phase 2
33 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 2
34 Anti-Inflammatory Agents Phase 2
35 Antibiotics, Antitubercular Phase 2
36 Neurotransmitter Agents Phase 2
37 Neuroprotective Agents Phase 2
38 Antifungal Agents Phase 2
39 Anti-Bacterial Agents Phase 2
40 glucocorticoids Phase 2
41 Vasodilator Agents Phase 2
42 Peripheral Nervous System Agents Phase 2
43 Gastrointestinal Agents Phase 2
44 Methylprednisolone acetate Phase 2
45 Protein Kinase Inhibitors Phase 2
46 Antineoplastic Agents, Phytogenic Phase 2
47 Antimitotic Agents Phase 2
48 Antimetabolites Phase 2
49 Antiviral Agents Phase 2
50 Antimetabolites, Antineoplastic Phase 2

Interventional clinical trials:

(show all 13)
# 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
13 Diagnostic Accuracy of PIVKA-II Combining With Alpha-Fetoprotein in Hepatic Tumor Not yet recruiting NCT03645655

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, Heart, Spleen, Thyroid

Publications for Hemangioendothelioma

Articles related to Hemangioendothelioma:

(show top 50) (show all 1147)
# 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
Hepatic Hemangioendothelioma: A Rare Cause of Congenital Hypothyroidism. ( 30370481 )
2018
26
Epithelioid Hemangioendothelioma of the Bowel in Crohn's Disease: The First Reported Case. ( 30238810 )
2018
27
Epithelioid Hemangioendothelioma as a Model of YAP/TAZ-Driven Cancer: Insights from a Rare Fusion Sarcoma. ( 29996478 )
2018
28
A Case of Large Meningeal Epithelioid Hemangioendothelioma With WWTR1-CAMTA1 Gene Rearrangement and Slow Growth Over 15 Years. ( 30085199 )
2018
29
CT-guided Biopsy for the Diagnosis of Pulmonary Epithelioid Hemangioendothelioma Mimicking Metastatic Lung Cancer: A Case Report. ( 30101918 )
2018
30
The Feature of Solitary Small Nodular Type of Hepatic Epithelioid Hemangioendothelioma. ( 30186092 )
2018
31
A Case of Vanishing Metastatic Mass: Right Atrial Mass in the Setting of Primary Epithelioid Hemangioendothelioma of the Spine. ( 30186136 )
2018
32
Primary Epithelioid Hemangioendothelioma of the Heart. ( 30219135 )
2018
33
Cutaneous epithelioid hemangioendothelioma: A rare presentation. ( 30289116 )
2018
34
Complete metabolic response to therapy of hepatic epithelioid hemangioendothelioma evaluated with 18F-fluorodeoxyglucose positron emission tomography/contrast-enhanced computed tomography: A CARE case report. ( 30334971 )
2018
35
Clinical characteristics of Japanese patients with epithelioid hemangioendothelioma: a multicenter retrospective study. ( 30340559 )
2018
36
Hepatic epithelioid hemangioendothelioma: A great mimicker. ( 30366173 )
2018
37
The "Target sign" and the "Lollipop sign" in hepatic epithelioid hemangioendothelioma. ( 30367214 )
2018
38
Hepatic epithelioid hemangioendothelioma: Challenges in the preoperative diagnosis. ( 30392574 )
2018
39
Primary Epithelioid Hemangioendothelioma of the Heart. ( 30454535 )
2018
40
18F-FDG PET and Bone Scintigraphy of Epithelioid Hemangioendothelioma. ( 30516676 )
2018
41
Expanding the Spectrum of Genetic Alterations in Pseudomyogenic Hemangioendothelioma With Recurrent Novel ACTB-FOSB Gene Fusions. ( 30256258 )
2018
42
Kaposiform hemangioendothelioma without cutaneous involvement. ( 30293120 )
2018
43
Comparison between infantile hepatic hemangioendothelioma and hepatoblastoma in pediatric patients: clinical manifestations and contrast-enhanced computed tomography features. ( 30302991 )
2018
44
Comparison of Corticosteroid and Vincristine in Treating Kaposiform Hemangioendothelioma and Tufted Angioma: A Systematic Review and Meta-Analysis. ( 30372769 )
2018
45
Diagnosis of known sarcoma fusions and novel fusion partners by targeted RNA sequencing with identification of a recurrent ACTB-FOSB fusion in pseudomyogenic hemangioendothelioma. ( 30459475 )
2018
46
Adult-onset kaposiform hemangioendothelioma with neurofibromatosis type 1: A case report and literature review. ( 30466240 )
2018
47
Primary pseudomyogenic hemangioendothelioma of bone: case report and review of the literature. ( 30546145 )
2018
48
Retiform hemangioendothelioma presenting as a pedunculated nodule on the site of an inguinal pyoderma chronica. ( 29756229 )
2018
49
Sirolimus as initial therapy for kaposiform hemangioendothelioma and tufted angioma. ( 29999213 )
2018
50
Successful Management of Kaposiform Hemangioendothelioma with Long-Term Sirolimus Treatment: a Case Report and Review of the Literature. ( 30002799 )
2018

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 negative regulation of signal transduction GO:0009968 9.46 FLT4 KDR
2 transmembrane receptor protein tyrosine kinase signaling pathway GO:0007169 9.43 FLT4 KDR
3 positive regulation of endothelial cell proliferation GO:0001938 9.4 FLT4 KDR
4 vascular endothelial growth factor receptor signaling pathway GO:0048010 9.37 FLT4 KDR
5 hemostasis GO:0007599 9.32 FLI1 PLG
6 positive regulation of endothelial cell migration GO:0010595 9.26 FLT4 KDR
7 cellular response to vascular endothelial growth factor stimulus GO:0035924 9.16 FLT4 KDR
8 angiogenesis GO:0001525 9.13 FLT4 KDR PECAM1
9 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 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
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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