MCID: HMN016
MIFTS: 34

Hemangioendothelioma

Categories: Cancer diseases, Cardiovascular diseases, Rare diseases

Aliases & Classifications for Hemangioendothelioma

MalaCards integrated aliases for Hemangioendothelioma:

Name: Hemangioendothelioma 52 54 71

Classifications:



External Ids:

UMLS 71 C0018915

Summaries for Hemangioendothelioma

NIH Rare Diseases : 52 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 malignant epithelioid hemangioendothelioma and kaposiform hemangioendothelioma. An important gene associated with Hemangioendothelioma is CAMTA1 (Calmodulin Binding Transcription Activator 1). The drugs Tacrolimus and Ethiodized oil have been mentioned in the context of this disorder. Affiliated tissues include liver, bone and lung, and related phenotypes are Decreased shRNA abundance (Z-score < -2) and Decreased shRNA abundance (Z-score < -2)

Wikipedia : 74 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 386)
# Related Disease Score Top Affiliating Genes
1 malignant epithelioid hemangioendothelioma 33.7 FOSB CAMTA1
2 kaposiform hemangioendothelioma 32.9 FLT4 FLI1 CD34
3 conventional angiosarcoma 30.2 CD34 CAMTA1
4 lymphangioma 30.1 FLT4 CD34
5 capillary hemangioma 30.0 FLT4 CD34
6 histiocytoid hemangioma 29.9 FOSB CD34 CAMTA1
7 proliferative fasciitis 29.9 FOSB CAMTA1
8 angiosarcoma 29.7 FLT4 FLI1 CD34 CAMTA1
9 hemangioma 29.7 FOSB FLT4 FLI1 CD34
10 hepatic veno-occlusive disease 29.5 PLG CD34
11 breast angiosarcoma 29.3 FLT4 CD34
12 lymphangiosarcoma 29.3 FLT4 CD34
13 intussusception 29.2 FLT4 CD34
14 vascular cancer 28.7 PLG FLT4 FLI1 CD34 CAMTA1
15 sarcoma, synovial 28.3 FLI1 CD34
16 epithelioid hemangioendothelioma 12.7
17 retiform hemangioendothelioma 12.4
18 composite hemangioendothelioma 12.4
19 spindle cell hemangioma 12.0
20 hemangioma-thrombocytopenia syndrome 11.6
21 angioma, tufted 11.6
22 non-involuting congenital hemangioma 11.5
23 rapidly involuting congenital hemangioma 11.5
24 rare tumor 10.8
25 rare vascular tumor 10.8
26 thrombocytopenia 10.6
27 kaposi sarcoma 10.5
28 epithelioid sarcoma 10.5
29 congestive heart failure 10.5
30 sarcoma 10.4
31 spindle cell sarcoma 10.4
32 lymphangiomatosis 10.4
33 cavernous hemangioma 10.3
34 benign mesothelioma 10.3
35 pyogenic granuloma 10.3
36 goiter 10.2
37 hypothyroidism 10.2
38 back pain 10.2
39 hemangiopericytoma, malignant 10.2
40 rhabdomyosarcoma 10.2
41 rare lymphatic malformation 10.2
42 hereditary lymphedema i 10.2
43 respiratory failure 10.2
44 cholangiocarcinoma 10.2
45 hemopericardium 10.2
46 pericardial effusion 10.2
47 intrahepatic cholangiocarcinoma 10.2
48 hepatoblastoma 10.2
49 hypereosinophilic syndrome 10.2
50 rare soft tissue tumor 10.2

Graphical network of the top 20 diseases related to Hemangioendothelioma:



Diseases related to Hemangioendothelioma

Symptoms & Phenotypes for Hemangioendothelioma

GenomeRNAi Phenotypes related to Hemangioendothelioma according to GeneCards Suite gene sharing:

26
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Decreased shRNA abundance (Z-score < -2) GR00366-A-107 9.28 FOSB
2 Decreased shRNA abundance (Z-score < -2) GR00366-A-12 9.28 CD34
3 Decreased shRNA abundance (Z-score < -2) GR00366-A-180 9.28 FOSB
4 Decreased shRNA abundance (Z-score < -2) GR00366-A-191 9.28 FOSB
5 Decreased shRNA abundance (Z-score < -2) GR00366-A-204 9.28 FOSB
6 Decreased shRNA abundance (Z-score < -2) GR00366-A-213 9.28 FOSB
7 Decreased shRNA abundance (Z-score < -2) GR00366-A-6 9.28 FOSB
8 Decreased shRNA abundance (Z-score < -2) GR00366-A-63 9.28 FOSB
9 Decreased shRNA abundance (Z-score < -2) GR00366-A-85 9.28 FOSB

MGI Mouse Phenotypes related to Hemangioendothelioma:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 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 60)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Tacrolimus Approved, Investigational Phase 4 104987-11-3 445643 439492 6473866
2 Ethiodized oil Approved, Investigational Phase 4 8008-53-5
3
Clotrimazole Approved, Vet_approved Phase 4 23593-75-1 2812
4
Miconazole Approved, Investigational, Vet_approved Phase 4 22916-47-8 4189
5
Sirolimus Approved, Investigational Phase 4 53123-88-9 5284616 6436030 46835353
6
Propranolol Approved, Investigational Phase 4 525-66-6 4946
7 Calcineurin Inhibitors Phase 4
8 Immunosuppressive Agents Phase 4
9 Immunologic Factors Phase 4
10 Anti-Infective Agents Phase 4
11 Anti-Bacterial Agents Phase 4
12 Antifungal Agents Phase 4
13 Antibiotics, Antitubercular Phase 4
14 Antihypertensive Agents Phase 4
15 Adrenergic Agents Phase 4
16 Adrenergic Antagonists Phase 4
17 Vasodilator Agents Phase 4
18 Anti-Arrhythmia Agents Phase 4
19 Adrenergic beta-Antagonists Phase 4
20 Neurotransmitter Agents Phase 4
21
Trametinib Approved Phase 2 871700-17-3 11707110
22
Methylprednisolone Approved, Vet_approved Phase 2 83-43-2 6741
23
Prednisolone phosphate Approved, Vet_approved Phase 2 302-25-0
24
Methylprednisolone hemisuccinate Approved Phase 2 2921-57-5
25 Prednisolone acetate Approved, Vet_approved Phase 2 52-21-1
26
Prednisolone Approved, Vet_approved Phase 2 50-24-8 5755
27
nivolumab Approved Phase 1, Phase 2 946414-94-4
28
Sunitinib Approved, Investigational Phase 1, Phase 2 557795-19-4, 341031-54-7 5329102
29
tannic acid Approved Phase 2 1401-55-4
30
Benzocaine Approved, Investigational Phase 2 94-09-7, 1994-09-7 2337
31
Vincristine Approved, Investigational Phase 2 57-22-7, 2068-78-2 5978
32
Gemcitabine Approved Phase 2 95058-81-4 60750
33
Durvalumab Approved, Investigational Phase 2 1428935-60-7
34
Prednisolone hemisuccinate Experimental Phase 2 2920-86-7
35
Tremelimumab Investigational Phase 2 745013-59-6
36 Protein Kinase Inhibitors Phase 2
37 Methylprednisolone Acetate Phase 2
38 Hormones Phase 2
39 Antineoplastic Agents, Hormonal Phase 2
40 Hormone Antagonists Phase 2
41 glucocorticoids Phase 2
42 Anti-Inflammatory Agents Phase 2
43 Pharmaceutical Solutions Phase 1, Phase 2
44 Angiogenesis Inhibitors Phase 1, Phase 2
45 Tubulin Modulators Phase 2
46 Antimitotic Agents Phase 2
47 Antiviral Agents Phase 2
48 Antimetabolites Phase 2
49 Antineoplastic Agents, Immunological Phase 2
50
Prednisone Approved, Vet_approved Phase 1 53-03-2 5865

Interventional clinical trials:

(show all 19)
# 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 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)
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 Sirolimus in the Treatment for Propranolol-resistant Infantile Hepatic Hemangioendothelioma Not yet recruiting NCT04406870 Phase 4 Sirolimus Oral Product;propranolol
5 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
6 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
7 A Non-Randomized, Open-Label, Phase 2 Study of Trametinib in Patients With Unresectable or Metastatic Epithelioid Hemangioendothelioma Recruiting NCT03148275 Phase 2 Trametinib
8 A Pilot Phase 2 Study of Eribulin in Angiosarcoma and Epithelioid Hemangioendothelioma (EHE) Recruiting NCT03331250 Phase 2 Eribulin
9 Sirolimus Plus Propranolol Versus Sirolimus Plus Prednisolone for Kaposiform Hemangioendothelioma With Kasabach-Merritt Syndrome Recruiting NCT03188068 Phase 2 Sirolimus;Prednisolone;Propranolol
10 Phase I-II Trial of Sunitinib Plus Nivolumab After Standard Treatment in Advanced Soft Tissue and Bone Sarcomas Recruiting NCT03277924 Phase 1, Phase 2 Sunitinib 37.5 MG, Sunitinib 25 MG [Sutent];Nivolumab 100 MG/10 ML [Opdivo]
11 A Randomized Phase 2 Study of Vincristine Versus Sirolimus to Treat High Risk Kaposiform Hemangioendothelioma (KHE). Active, not recruiting NCT02110069 Phase 2 Vincristine;Sirolimus
12 A Randomized, Double-Blind Phase II, Study of Gemcitabine Alone or in Combination With Pazopanib for Refractory Soft Tissue Sarcoma Active, not recruiting NCT01532687 Phase 2 Gemcitabine Hydrochloride;Pazopanib Hydrochloride
13 A Phase II Multi-Arm Study to Test the Efficacy of Immunotherapeutic Agents in Multiple Sarcoma Subtypes Active, not recruiting NCT02815995 Phase 2 Durvalumab;Tremelimumab
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 PIVKA-II Combined With Alpha-Fetoprotein for Diagnostic and Prognostic Value of Hepatic Tumors in Infants Recruiting NCT03645655
16 Identification of Biomarkers for Patients With Vascular Anomalies Recruiting NCT03001180
17 Lymphatic Anomalies Registry for the Assessment of Outcome Data Recruiting NCT02399527
18 Multimodal Immune Characterization of RAre Soft Tissue Sarcoma - MIRAS Project From SARRA (SARcome RAre) Project of the French Sarcoma Group Not yet recruiting NCT03967834
19 Prospective Identification of Predictive Biomarkers of Trabectedin Efficacy in Non-L Soft-tissue Sarcoma Patients Not yet 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, Bone, Lung, Endothelial, Thyroid, Spleen, Heart

Publications for Hemangioendothelioma

Articles related to Hemangioendothelioma:

(show top 50) (show all 2673)
# 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. 54 61
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. 61 54
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. 61 54
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
Acute disseminated encephalomyelitis associated with a novel paraneoplastic process in hepatic epithelial hemangioendothelioma: A case report. 61
32447206 2020
10
Variant WWTR1 gene fusions in epithelioid hemangioendothelioma-A genetic subset associated with cardiac involvement. 61
32170768 2020
11
Liver Transplantation for Hepatic Epithelioid Hemangioendothelioma Is Facilitated by Exception Points With Acceptable Long-term Outcomes. 61
31577674 2020
12
[Clinicopathological features of pulmonary epithelioid hemangioendothelioma: a study of 18 cases]. 61
32486531 2020
13
Intraoperative Use of Indocyanine Green Fluorescence Imaging for Detecting Superficial Liver Hemangioendothelioma. 61
32490729 2020
14
Sirolimus for Kaposiform Hemangioendothelioma With Kasabach-Merritt Phenomenon in Two Infants. 61
32176003 2020
15
Reactivation of Kasabach-Merritt phenomenon in a young adult with a congenital kaposiform hemangioendothelioma. 61
32543761 2020
16
Hepatic Epithelioid Hemangioendothelioma as a Rare Indication for Liver Transplantation. 61
32241636 2020
17
Natural history of epithelioid hemangioendothelioma that progressed over 20 years. 61
32307864 2020
18
[Composite hemangioendothelioma with neuroendocrine marker expression: report of a case]. 61
32486550 2020
19
Imaging Diagnosis of Epithelioid Hemangioendothelioma in Thoracic Vertebrae and Liver. 61
31794741 2020
20
Pseudomyogenic Hemangioendothelioma: A Rare Vascular Tumor of the Oral Cavity. 61
32118660 2020
21
Infantile hepatic hemangioendothelioma associated with pulmonary artery hypertension and cardiac insufficiency successfully treated with transcatheter arterial embolization and propranolol: A case report. 61
32541524 2020
22
Epithelioid hemangioendothelioma of the sternum. 61
32347015 2020
23
Health-related quality of life and symptom burden of epithelioid hemangioendothelioma patients: a global patient-driven Facebook study in a very rare malignancy. 61
32476528 2020
24
Analysis of mTOR pathway expression in lymphatic malformation and related diseases. 61
32067331 2020
25
Hepatic epithelioid hemangioendothelioma: An international multicenter study. 61
32535084 2020
26
Effective combined therapy for pulmonary epithelioid hemangioendothelioma: A case report. 61
32518794 2020
27
Case Report 18F-FDG PET/CT of Pseudomyogenic Hemangioendothelioma. 61
32108703 2020
28
Natural History of Epithelioid Hemangioendothelioma of the Liver: CT Findings of 15 Cases. 61
32434692 2020
29
Hepatic hemangioendothelioma: CT, MR, and FDG-PET-CT in 67 patients-a bi-institutional comprehensive cancer center review. 61
32002639 2020
30
Composite Hemangioendothelioma With Neuroendocrine Marker Expression: Report of a "Paraganglioma-Like" Paravertebral Case. 61
32418470 2020
31
Mediastinal Epithelioid Hemangioendothelioma Revealed on 68Ga-DOTATATE PET/CT. 61
32209882 2020
32
Propranolol for infantile hepatic hemangioendothelioma: Clinical evaluation of drug efficacy and safety using a single-center patient cohort. 61
32532590 2020
33
A renal cell carcinoma with EWSR1-TFE3 fusion gene. 61
31774608 2020
34
Surgical treatment of asymptomatic epithelioid hemangioendothelioma originating from the superior vena cava: A case report. 61
32312011 2020
35
Clinicopathological features of pseudomyogenic hemangioendothelioma and precision therapy based on whole exome sequencing. 61
32227592 2020
36
F18-fluorodeoxyglucose positron emission tomography/computed tomography in the evaluation of vertebral vascular tumors. 61
32353715 2020
37
Successful Excision of Epithelioid Hemangioendothelioma of the Superior Vena Cava. 61
31472133 2020
38
Multiple bilateral pulmonary epithelioid hemangioendothelioma mimicking metastatic lung cancer: case report and literature review. 61
32314617 2020
39
Prognostic stratification of clinical and molecular epithelioid hemangioendothelioma subsets. 61
31537895 2020
40
Effective surgical treatment of life-threatening huge vascular anomalies associated with thrombocytopenia and coagulopathy in infants unresponsive to drug therapy. 61
32340603 2020
41
The histopathology of congenital hemangioma and its clinical correlations: a long-term follow-up study of 55 cases. 61
32281140 2020
42
Pseudomyogenic Hemangioendothelioma (Epithelioid Sarcoma-Like Hemangioendothelioma). 61
31017450 2020
43
Primary Vascular Tumors of Bone: A Monoinstitutional Morphologic and Molecular Analysis of 427 Cases With Emphasis on Epithelioid Variants. 61
32271190 2020
44
Epithelioid Hemangioendothelioma of the Ulnar Artery Presenting with Neuropathy. 61
32205246 2020
45
TFE3 rearranged epithelioid hemangioendothelioma of bone: A clinicopathological, immunohistochemical and molecular study of two cases. 61
32151991 2020
46
Hepatic Epithelioid Hemangioendothelioma: A Rare Disease With Favorable Outcomes After Liver Transplantation. 61
32217012 2020
47
Outcome of Children Treated for Infantile Hepatic Hemangioendothelioma. 61
31233466 2020
48
Novel cavernous sinus TSC2/JAK3 mutant hemangioendothelioma in a teenager. 61
32193183 2020
49
VEGF-VEGFR pathway seems to be the best target in hepatic epithelioid hemangioendothelioma: A case series with review of the literature. 61
32201052 2020
50
Hepatic Hemangioendothelioma: An update. 61
32206176 2020

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 8.62 PLG FLI1

Molecular functions related to Hemangioendothelioma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 sequence-specific DNA binding GO:0043565 8.8 FOSB FLI1 CAMTA1

Sources for Hemangioendothelioma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
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
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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