MCID: ANG020
MIFTS: 63

Angiosarcoma

Categories: Blood diseases, Cancer diseases, Cardiovascular diseases, Fetal diseases, Immune diseases, Rare diseases

Aliases & Classifications for Angiosarcoma

MalaCards integrated aliases for Angiosarcoma:

Name: Angiosarcoma 12 73 58 36 54 6 15 39 17
Hemangiosarcoma 12 44 70

Characteristics:

Orphanet epidemiological data:

58
angiosarcoma
Inheritance: Not applicable; Prevalence: <1/1000000 (Europe); Age of onset: Adult;

Classifications:

Orphanet: 58  
Rare circulatory system diseases
Developmental anomalies during embryogenesis


External Ids:

Disease Ontology 12 DOID:0001816
KEGG 36 H01666
MeSH 44 D006394
NCIt 50 C3088 C9275
SNOMED-CT 67 39000009
ICD10 via Orphanet 33 C49.9
UMLS via Orphanet 71 C0018923
Orphanet 58 ORPHA263413
UMLS 70 C0018923 C0854893

Summaries for Angiosarcoma

KEGG : 36 Angiosarcomas are rare, generally aggressive, malignant mesenchymal tumours of vascular origin and constitute less than 1% of all sarcomas. Their most frequent site of origin is the head and neck, especially the scalp, of elderly men. Angiosarcomas are likely derived from vascular endothelial cells, possibly resulting from aberrant angiogenesis. Studies have shown relatively increased expression of vascular endothelial growth factor (VEGF) and its receptors (VEGFr1-3) in human angiosarcoma. The most common gene mutation in angiosarcomas is KRAS as well as other RAS mutations. Activation of the RAS pathway is often tumorigenic. Although immortalised murine endothelial cells form benign haemangiomas in vivo, the addition of activated HRAS produces rapidly growing, poorly differentiated angiosarcomas.

MalaCards based summary : Angiosarcoma, also known as hemangiosarcoma, is related to liver angiosarcoma and skin angiosarcoma. An important gene associated with Angiosarcoma is KRAS (KRAS Proto-Oncogene, GTPase), and among its related pathways/superpathways are ERK Signaling and GPCR Pathway. The drugs Ethiodized oil and Ifosfamide have been mentioned in the context of this disorder. Affiliated tissues include breast, endothelial and spleen, and related phenotypes are Decreased cell migration and cardiovascular system

Disease Ontology : 12 A vascular cancer that derives from the cells that line the walls of blood vessels or lymphatic vessels.

Wikipedia : 73 Angiosarcoma is a cancer of the endothelial cells that line the walls of blood vessels or lymphatic... more...

Related Diseases for Angiosarcoma

Diseases related to Angiosarcoma via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 945)
# Related Disease Score Top Affiliating Genes
1 liver angiosarcoma 32.9 VIM TP53 PECAM1 KRAS
2 skin angiosarcoma 32.6 TIE1 FLT4
3 prostate angiosarcoma 32.6 VEGFA PECAM1 CD34
4 thyroid angiosarcoma 32.5 VWF PECAM1 CD34
5 breast angiosarcoma 32.4 TIE1 PECAM1 MYC KDR FLT4 CD34
6 ovarian angiosarcoma 32.4 PECAM1 CD34
7 central nervous system angiosarcoma 32.4 PECAM1 CD34
8 pediatric angiosarcoma 32.2 VWF VEGFA TP53 PECAM1 CD34
9 conventional angiosarcoma 32.1 PECAM1 CD34 CAMTA1
10 lymphangiosarcoma 32.1 VWF TIE1 PECAM1 PDPN FLT4 CD34
11 intravascular papillary endothelial hyperplasia 32.1 VIM VEGFA
12 epithelioid hemangioendothelioma 31.7 VEGFA KDR FLT1 CAMTA1 CALB2
13 kaposi sarcoma 31.1 VEGFA TP53 MYC CD34
14 hemangioendothelioma 31.1 FLT4 FLI1 CD34 CAMTA1
15 spindle cell sarcoma 30.9 VIM VEGFA MUC1 CD34
16 pneumothorax 30.8 VIM MUC1 KDR CALB2
17 heart sarcoma 30.8 PECAM1 CD34
18 fibrous histiocytoma 30.7 VIM PECAM1 CD34
19 benign mesothelioma 30.7 VIM PECAM1 PDPN CALB2
20 mesenchymal cell neoplasm 30.6 TP53 PECAM1 CD34
21 neurilemmoma 30.6 VIM MUC1 CD34 CALB2
22 malignant fibrous histiocytoma 30.6 VIM TP53 MUC1
23 cavernous hemangioma 30.6 VIM PECAM1 MUC1 KDR CD34
24 hemangiopericytoma, malignant 30.5 VIM PECAM1 MUC1 CD34
25 pyogenic granuloma 30.5 TEK KRAS ANGPT2
26 portal hypertension 30.4 VWF VEGFA PECAM1
27 histiocytoid hemangioma 30.4 PECAM1 CD34 CAMTA1
28 leiomyosarcoma 30.4 VIM VEGFA TP53 PECAM1 MYC MUC1
29 malignant peripheral nerve sheath tumor 30.4 VIM TP53 MUC1 CD34
30 congenital lymphedema 30.4 PDPN FLT4
31 rectum adenocarcinoma 30.3 TP53 MYC KRAS
32 acquired immunodeficiency syndrome 30.3 TP53 PECAM1 MYC CD34
33 squamous cell carcinoma 30.3 VEGFA TP53 PDPN MYC MUC1
34 thrombocytopenia 30.3 VWF PECAM1 KRAS FLI1 CD34 ANGPT2
35 connective tissue benign neoplasm 30.3 TP53 PECAM1 CD34 CALB2
36 varicose veins 30.3 VWF VEGFA KDR
37 cystic teratoma 30.2 TP53 KRAS CALB2
38 adenoid squamous cell carcinoma 30.2 VIM PECAM1 KRT8 CD34
39 neurofibroma 30.2 VIM VEGFA TP53 MUC1 CD34
40 suppression of tumorigenicity 12 30.2 VEGFA TP53 MYC KRT8
41 mucinous cystadenocarcinoma 30.1 VIM PDPN MUC1
42 cholangiocarcinoma 30.1 VEGFA TP53 MUC1 KRT8 KRAS
43 adenocarcinoma 30.1 VEGFA TP53 MYC MUC1 KRAS KDR
44 breast sarcoma 30.1 TP53 TIE1 PECAM1 CD34
45 arteriovenous malformation 30.1 VEGFA TIE1 TEK KRAS ANGPT2
46 ischemia 30.1 VEGFA TEK KDR FLT1
47 vascular disease 30.1 VWF VEGFA TP53 PECAM1 CD34
48 carcinosarcoma 30.1 VIM TP53 MUC1 KRAS CALB2
49 liposarcoma 30.1 VIM TP53 MYC CD34 CALB2
50 intramuscular hemangioma 30.1 TEK PECAM1 CD34

Graphical network of the top 20 diseases related to Angiosarcoma:



Diseases related to Angiosarcoma

Symptoms & Phenotypes for Angiosarcoma

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

26
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Decreased cell migration GR00055-A-1 8.8 FLT1 TIE1 VIM

MGI Mouse Phenotypes related to Angiosarcoma:

46 (show all 18)
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 10.48 ANGPT2 FLI1 FLT1 FLT4 KDR KRAS
2 homeostasis/metabolism MP:0005376 10.41 ANGPT2 CD34 FLT1 FLT4 KDR KRAS
3 hematopoietic system MP:0005397 10.4 ANGPT2 CD34 FLI1 FLT1 KDR KRAS
4 immune system MP:0005387 10.4 ANGPT2 CD34 FLI1 FLT1 FLT4 KDR
5 cellular MP:0005384 10.35 ANGPT2 CD34 FLI1 FLT1 KDR KRAS
6 growth/size/body region MP:0005378 10.35 CAMTA1 FLI1 FLT1 FLT4 KDR KRAS
7 mortality/aging MP:0010768 10.34 ANGPT2 CAMTA1 FLI1 FLT1 FLT4 KDR
8 embryo MP:0005380 10.33 FLI1 FLT1 FLT4 KDR KRAS KRT8
9 digestive/alimentary MP:0005381 10.3 ANGPT2 FLT4 KRAS KRT8 MYC PDPN
10 integument MP:0010771 10.22 ANGPT2 CD34 FLT4 KDR KRAS MYC
11 muscle MP:0005369 10.22 FLI1 FLT1 FLT4 KDR KRAS MYC
12 nervous system MP:0003631 10.13 CALB2 CAMTA1 FLI1 FLT1 KDR KRAS
13 liver/biliary system MP:0005370 10.11 FLI1 FLT4 KDR KRAS KRT8 MYC
14 normal MP:0002873 9.93 CALB2 CAMTA1 FLT1 FLT4 KDR KRAS
15 neoplasm MP:0002006 9.91 CAMTA1 CD34 FLI1 KRAS MYC TP53
16 no phenotypic analysis MP:0003012 9.8 FLT4 KDR KRAS MYC TP53 VEGFA
17 reproductive system MP:0005389 9.65 ANGPT2 CAMTA1 FLT1 FLT4 KRAS KRT8
18 respiratory system MP:0005388 9.28 ANGPT2 KDR KRAS PDPN PECAM1 TIE1

Drugs & Therapeutics for Angiosarcoma

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

(show top 50) (show all 77)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Ethiodized oil Approved, Investigational Phase 4 8008-53-5
2
Ifosfamide Approved Phase 3 3778-73-2 3690
3 Alkylating Agents Phase 3
4
Isophosphamide mustard Phase 3 100427
5
Axitinib Approved, Investigational Phase 2 319460-85-0 6450551
6
Sorafenib Approved, Investigational Phase 2 284461-73-0 216239 406563
7
Romidepsin Approved, Investigational Phase 2 128517-07-7 5352062
8
Clotrimazole Approved, Vet_approved Phase 2 23593-75-1 2812
9
Miconazole Approved, Investigational, Vet_approved Phase 2 22916-47-8 4189
10
Sirolimus Approved, Investigational Phase 2 53123-88-9 6436030 5284616
11
Gemcitabine Approved Phase 2 95058-81-4 60750
12
Docetaxel Approved, Investigational Phase 2 114977-28-5 148124
13
Lenograstim Approved, Investigational Phase 2 135968-09-1
14
Cyclophosphamide Approved, Investigational Phase 2 50-18-0, 6055-19-2 2907
15
Propranolol Approved, Investigational Phase 2 525-66-6 4946
16
Talimogene laherparepvec Approved, Experimental, Investigational Phase 2 1187560-31-1
17
nivolumab Approved Phase 2 946414-94-4
18
Durvalumab Approved, Investigational Phase 2 1428935-60-7
19
Pembrolizumab Approved Phase 2 1374853-91-4
20
Ipilimumab Approved Phase 2 477202-00-9
21
Olaparib Approved Phase 2 763113-22-0 23725625
22
Cemiplimab Approved, Investigational Phase 2 1801342-60-8
23
Metformin Approved Phase 2 657-24-9 4091 14219
24
Adenosine Approved, Investigational Phase 2 58-61-7 60961
25
Trastuzumab Approved, Investigational Phase 2 180288-69-1 9903
26
Irinotecan Approved, Investigational Phase 2 100286-90-6, 97682-44-5 60838
27
Talazoparib Approved, Investigational Phase 2 1207456-01-6 135565082
28
Pertuzumab Approved Phase 2 380610-27-5, 145040-37-5 2540
29
Carboplatin Approved Phase 2 41575-94-4 10339178 498142 38904
30
Doxorubicin Approved, Investigational Phase 2 23214-92-8 31703
31
Dexrazoxane Approved, Withdrawn Phase 2 24584-09-6 71384
32
Paclitaxel Approved, Vet_approved Phase 2 33069-62-4 36314
33
Bevacizumab Approved, Investigational Phase 2 216974-75-3
34
Trebananib Investigational Phase 2 894356-79-7
35
Veliparib Investigational Phase 2 912444-00-9 11960529
36 Imatinib Mesylate Phase 2 220127-57-1 123596
37 Protein Kinase Inhibitors Phase 2
38 Immunologic Factors Phase 2
39 Immunosuppressive Agents Phase 2
40 Antifungal Agents Phase 2
41 Anti-Infective Agents Phase 2
42 Antiviral Agents Phase 2
43 Antimetabolites Phase 2
44 Immunoglobulins Phase 2
45 Antibodies Phase 2
46 Anti-Arrhythmia Agents Phase 2
47 Adrenergic Antagonists Phase 2
48 Vasodilator Agents Phase 2
49 Antihypertensive Agents Phase 2
50 Adrenergic beta-Antagonists Phase 2

Interventional clinical trials:

(show all 41)
# Name Status NCT ID Phase Drugs
1 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
2 Risk-Based Treatment for Non-Rhabdomyosarcoma Soft Tissue Sarcomas (NRSTS) in Patients Under 30 Years of Age Unknown status NCT00346164 Phase 3 doxorubicin hydrochloride;ifosfamide
3 A Randomized Phase 3 Trial of TRC105 and Pazopanib Versus Pazopanib Alone in Patients With Advanced Angiosarcoma (TAPPAS) Completed NCT02979899 Phase 3 Votrient
4 Dose-Finding of Propranolol in Combination With Metronomic Fixed Oral Cyclophosphamide Based on Bivariate Efficacy-tolerability Outcome in Patients With Locally Advanced or Metastatic Angiosarcoma: A Collaborative and Innovative Phase I-II Sequential Trial by the French Sarcoma Group (GSF/GETO) Unknown status NCT02732678 Phase 1, Phase 2 PROPRANOLOL
5 Phase II Multicenter Stratified Study Evaluating the Efficacy and Toxicity of Sorafenib in Treating Locally Advanced or Metastatic Angiosarcomas That Are Not Accessible to Curative Surgery Unknown status NCT00874874 Phase 2 sorafenib tosylate
6 Phase II Study of Imatinib Mesylate in Patients With Life Threatening Malignant Rare Diseases Completed NCT00154388 Phase 2 Imatinib mesylate
7 A Clinicopathological Phase II Study of Axitinib in Patients With Advanced Angiosarcoma and Other Soft Tissue Sarcomas Completed NCT01140737 Phase 2 Axitinib
8 An Open Label Multicenter Phase II Study of Bevacizumab for the Treatment of Angiosarcoma Completed NCT00288015 Phase 2
9 Phase II Study of the Angiopoietin-1 and -2 Peptibody AMG 386 for the Treatment of Angiosarcoma Completed NCT01623869 Phase 2
10 A Multicenter Phase II Study of Sorafenib (BAY43-9006) in Non-GIST Sarcomas Completed NCT00245102 Phase 2 sorafenib tosylate
11 Phase II Study, Multicenter, Randomized, Stratified, Evaluating the Efficacity of Weekly Paclitaxel, With or Without Bevacizumab in the Treatment of Metastatic or Locally Advanced Angiosarcomas Not Accessible to Surgery Treatment. Completed NCT01303497 Phase 2 Paclitaxel;Bevacizumab
12 Multicentric Phase II Study Evaluating the Efficacy and Toxicity of Weekly Paclitaxel in Locally Advanced or Metastatic Soft Tissue Angiosarcomas That Cannot be Treated by Surgery Completed NCT00217607 Phase 2 paclitaxel
13 A Phase II Study of Single Agent Depsipeptide (FK228) in Metastatic or Unresectable Soft Tissue Sarcomas Completed NCT00112463 Phase 2 romidepsin
14 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
15 A Phase II Study of Cixutumumab (IMC-A12) in Combination With Temsirolimus in Pediatric Patients With Recurrent or Refractory Solid Tumors Completed NCT01614795 Phase 2 Temsirolimus
16 Phase II Trial Of Gemcitabine and Docetaxel With Bevacizumab in Selected Sarcoma Subtypes Completed NCT00887809 Phase 2 gemcitabine;docetaxel;bevacizumab
17 A Randomized, Double-Blind Phase II, Study of Gemcitabine Alone or in Combination With Pazopanib for Refractory Soft Tissue Sarcoma Completed NCT01532687 Phase 2 Gemcitabine;Gemcitabine Hydrochloride;Pazopanib;Pazopanib Hydrochloride
18 Phase II Trial, Multicenter, First Line Paclitaxel-Avelumab Treatment for Inoperable Angiosarcoma Recruiting NCT03512834 Phase 2 Avelumab;Paclitaxel
19 A Pilot Study of Oraxol in Subjects With Cutaneous Angiosarcoma Recruiting NCT03544567 Phase 2 Oraxol
20 A Phase II Study of Talimogene Laherparepvec (T-VEC) in the Treatment of Locally Advanced Cutaneous Angiosarcoma Recruiting NCT03921073 Phase 2 T-VEC
21 Induction Paclitaxel Followed by Concurrent Paclitaxel and Radiation Therapy for Cutaneous Angiosarcoma Recruiting NCT03921008 Phase 1, Phase 2 Paclitaxel
22 A Multicenter Phase II Trial of Paclitaxel With and Without Nivolumab in Taxane Naive, and Nivolumab and Cabozantinib in Taxane Pretreated Subjects With Angiosarcoma Recruiting NCT04339738 Phase 2 Cabozantinib S-malate;Paclitaxel
23 A Pilot Phase 2 Study of Eribulin in Angiosarcoma and Epithelioid Hemangioendothelioma (EHE) Recruiting NCT03331250 Phase 2 Eribulin
24 A Phase 2 Study Examining AGEN2034 as a Single-Agent and in Combination With AGEN1884 in Patients With Recurrent, Inoperable Angiosarcoma Recruiting NCT04607200 Phase 2 AGEN2034;AGEN1884
25 Neoadjuvant Trial on the Efficacy of Propranolol Monotherapy in Cutaneous Angiosarcoma Recruiting NCT04518124 Phase 2 Propranolol
26 A Phase II Multi-Arm Study to Test the Efficacy of Oleclumab and Durvalumab in Multiple Sarcoma Subtypes Recruiting NCT04668300 Phase 2
27 A Phase II Study of Talimogene Laherparepvec (T-VEC) Administered Concurrently With the Anti-PD1 Monoclonal Antibody Pembrolizumab in Patients With Metastatic and/or Locally Advanced Sarcoma Recruiting NCT03069378 Phase 2 Talimogene Laherparepvec (T-VEC);Pembrolizumab
28 DART: Dual Anti-CTLA-4 and Anti-PD-1 Blockade in Rare Tumors Recruiting NCT02834013 Phase 2
29 I-SPY Trial (Investigation of Serial Studies to Predict Your Therapeutic Response With Imaging And moLecular Analysis 2) Recruiting NCT01042379 Phase 2 Standard Therapy;AMG 386 with or without Trastuzumab;AMG 479 (Ganitumab) plus Metformin;MK-2206 with or without Trastuzumab;AMG 386 and Trastuzumab;T-DM1 and Pertuzumab;Pertuzumab and Trastuzumab;Ganetespib;ABT-888;Neratinib;PLX3397;Pembrolizumab - 4 cycle;Talazoparib plus Irinotecan;Patritumab and Trastuzumab;Pembrolizumab - 8 cycle;SGN-LIV1A;Durvalumab plus Olaparib;SD-101 + Pembrolizumab;Tucatinib plus trastuzumab and pertuzumab;Cemiplimab;Cemiplimab plus REGN3767;Trilaciclib with or without trastuzumab + pertuzumab;SYD985 ([vic-]trastuzumab duocarmazine);Oral Paclitaxel + Encequidar + Dostarlimab (TSR-042) + Carboplatin with or without trastuzumab;Oral Paclitaxel + Encequidar + Dostarlimab (TSR-042) with or without trastuzumab
30 Phase II Study Evaluating the Role of Pazopanib in Angiosarcoma Active, not recruiting NCT01462630 Phase 2 pazopanib hydrochloride
31 Multicenter, Open-Label Phase II Study of Daily Oral Regorafenib for Chemotherapy-Refractory, Metastatic and Locally Advanced Angiosarcoma Active, not recruiting NCT02048722 Phase 2 regorafenib
32 A Non-Inferiority Study of Doxorubicin With Upfront Dexrazoxane for the Treatment of Advanced or Metastatic Soft Tissue Sarcoma Active, not recruiting NCT02584309 Phase 2 Dexrazoxane;Doxorubicin
33 Single-arm, Multicenter, Open Label Phase II Trial to Evaluate the Efficacy of Pazopanib in Combination With Paclitaxel in Advanced and Relapsed Angiosarcoma Terminated NCT02212015 Phase 2 Pazopanib + Paclitaxel
34 Paclitaxel in Combination With Bevacizumab (Avastin) for the Treatment of Metastatic or Unresectable Angiosarcoma Terminated NCT01055028 Phase 2 Bevacizumab;Paclitaxel
35 A Phase 1 Study of Doxorubicin and A12 in Advanced Soft Tissue Sarcoma Completed NCT00720174 Phase 1 Doxorubicin Hydrochloride
36 A Phase 1B Study of Ribociclib in Combination With Doxorubicin in Advanced Soft Tissue Sarcomas Active, not recruiting NCT03009201 Phase 1 Doxorubicin Hydrochloride;Ribociclib
37 Targeted Complex Therapy for Advanced Melanoma, Gynecologic Cancers, and Other Malignancies: Nab-Paclitaxel (Abraxane)/Bevacizumab Complex (AB-Complex) Suspended NCT02020707 Phase 1 Nab-paclitaxel
38 Identification, Molecular Epidemiology Angiosarcoma of the Liver France Unknown status NCT01786889
39 Cytological Diagnosis of Hepatic Angiosarcoma and Its Histopathologic Correlation Completed NCT03025438
40 Observational - Potentially Actionable Mutations in Archived Non-Rhabdomyosarcoma Soft Tissue Sarcomas (NRSTS) Completed NCT01567046
41 Phase 1 Clinical Trial of Boron Neutron Capture Therapy (BNCT) Using CICS-1 and SPM-011 for Malignant Melanoma and Angiosarcoma Recruiting NCT04293289

Search NIH Clinical Center for Angiosarcoma

Cochrane evidence based reviews: hemangiosarcoma

Genetic Tests for Angiosarcoma

Anatomical Context for Angiosarcoma

MalaCards organs/tissues related to Angiosarcoma:

40
Breast, Endothelial, Spleen, Skin, Liver, Thyroid, Kidney

Publications for Angiosarcoma

Articles related to Angiosarcoma:

(show top 50) (show all 6592)
# Title Authors PMID Year
1
Malignant transformation of infantile hemangioma to angiosarcoma: response to chemotherapy with bevacizumab. 6 61
24740626 2014
2
Prospective enterprise-level molecular genotyping of a cohort of cancer patients. 6
25157968 2014
3
Autocrine and paracrine roles of VEGF/VEGFR-2 and VEGF-C/VEGFR-3 signaling in angiosarcomas of the scalp and face. 61 54
19913279 2010
4
Sporadic cutaneous angiosarcomas generally lack hypoxia-inducible factor 1alpha: a histologic and immunohistochemical study of 45 cases. 61 54
20123452 2010
5
Can lymphangiosarcoma be resurrected? A clinicopathological and immunohistochemical study of lymphatic differentiation in 49 angiosarcomas. 61 54
20459536 2010
6
Dramatic and durable efficacy of imatinib in an advanced angiosarcoma without detectable KIT and PDGFRA mutations. 54 61
19182535 2009
7
Oral acantholytic squamous cell carcinoma shares clinical and histological features with angiosarcoma. 54 61
18671846 2008
8
[Retroperitoneal giant mixed sarcoma. Case report]. 61 54
18507961 2008
9
Podoplanin is a useful diagnostic marker for epithelioid hemangioendothelioma of the liver. 61 54
18084256 2008
10
Detection and characterization of vascular endothelial growth factors and their receptors in a series of angiosarcomas. 61 54
18041747 2008
11
Cytokeratin and epithelial membrane antigen expression in angiosarcomas: an immunohistochemical study of 33 cases. 54 61
17284115 2007
12
Bilateral massive bloody pleurisy complicated by angiosarcoma. 61 54
17268128 2007
13
Intracranial angiosarcoma arising from a schwannoma. 61 54
16995966 2007
14
Observations on angiopoietin 2 in patients with angiosarcoma. 61 54
15149523 2004
15
[Oral adenoid squemous carcinoma. Tumor markers and prognosis]. 54 61
15045535 2004
16
Angiosarcoma of the larynx. Case report and review of the literature. 61 54
14677313 2003
17
Expression and function of the non-neuronal cholinergic system in endothelial cells. 61 54
12628465 2003
18
Actin-binding protein fascin expression in skin neoplasia. 54 61
12139639 2002
19
Epithelioid angiosarcoma of the pleura: a case report. 54 61
11748365 2001
20
Expression of vascular endothelial growth factor in a human hemangiosarcoma cell line (ISO-HAS). 54 61
11480589 2001
21
Angiosarcoma of the scalp: absence of vascular endothelial cadherin in primary and metastatic lesions. 54 61
11251578 2001
22
The non-neuronal cholinergic system in the endothelium: evidence and possible pathobiological significance. 54 61
11243570 2001
23
Expression of Tie1, Tie2, and angiopoietins 1, 2, and 4 in Kaposi's sarcoma and cutaneous angiosarcoma. 61 54
10854238 2000
24
[Podoplanin--a specific marker for lymphatic endothelium expressed in angiosarcoma]. 54 61
10714221 1999
25
[Primary renal epithelioid angiosarcoma with transitional cell carcinoma in renal pelvis]. 54 61
10374567 1997
26
Pseudomesotheliomatous angiosarcoma: a pleuropulmonary lesion simulating malignant pleural mesothelioma. 54 61
9181362 1997
27
Strong expression of kinase insert domain-containing receptor, a vascular permeability factor/vascular endothelial growth factor receptor in AIDS-associated Kaposi's sarcoma and cutaneous angiosarcoma. 61 54
8644848 1996
28
Multicentric epithelioid angiosarcoma of the bone. Pitfalls in clinical and morphological diagnosis. 54 61
9075571 1996
29
[A case of angiosarcoma with renal failure caused by recombinant interleukin-2 (rIL-2) and non-steroid anti-inflammatory drug (NSAID)]. 54 61
7776536 1995
30
Expression of VE (vascular endothelial)-cadherin and other endothelial-specific markers in haemangiomas. 54 61
7891227 1995
31
Pseudovascular adenoid squamous cell carcinoma of the skin. A neoplasm that may be mistaken for angiosarcoma. 61 54
1599022 1992
32
Immunoquantitation of von Willebrand factor (factor VIII-related antigen) in vinyl chloride exposed workers. 61 54
1739944 1992
33
[Soft tissue angiosarcoma in a child. Immunohistochemical and ultrastructural features]. 54 61
1580939 1992
34
The human hematopoietic progenitor cell antigen (CD34) in vascular neoplasia. 61 54
1712541 1991
35
An immunohistochemical study of sarcomatoid liver carcinomas. 61 54
1710948 1991
36
Primary retroperitoneal angiosarcoma with eosinophilic globules. A combined light-microscopic, immunohistochemical, and ultrastructural study. 61 54
1693269 1990
37
Hepatic vascular malignancies in children are associated with increased rates of surgical resection and improved overall survival compared with adults. 61
33661569 2021
38
Angiosarcoma of the vulva following radiation for colorectal cancer. 61
33598522 2021
39
Potential application of genomic profiling for the diagnosis and treatment of patients with sarcoma. 61
33747210 2021
40
Vaginal epithelioid angiosarcoma: A literature review of a rare entity in an unusual site. 61
33604443 2021
41
Dermatologic sequelae of breast cancer: From disease, surgery, and radiation. 61
33226140 2021
42
Angiosarcoma associated with radiation therapy after treatment of breast cancer. Retrospective study on ten years. 61
33487559 2021
43
Epizootic neoplasia in a managed population of Atlantic bumper fish, Chloroscombrus chrysurus (Osteichthyes: Carangidae), at a public aquarium. 61
33348452 2021
44
Genomic mutational profiling of circulating tumour DNA in metastatic angiosarcoma. 61
33230874 2021
45
MYC gene amplification by fluorescence in situ hybridization and MYC protein expression by immunohistochemistry in the diagnosis of cutaneous angiosarcoma: Systematic review and appropriate use criteria. 61
33128474 2021
46
Cutaneous Sarcomas. 61
33602520 2021
47
Vasculogenic Mesenchymal Tumor: A Clinicopathologic and Molecular Study of 55 Cases of a Distinctive Neoplasm Originating From Mediastinal Yolk Sac Tumor and an Occasional Precursor to Angiosarcoma. 61
33136584 2021
48
A rare case of primary angiosarcoma of the anterior mediastinum. 61
33222090 2021
49
Right atrial cardiac angiosarcoma treated with concurrent proton beam therapy and paclitaxel: A novel approach to a rare disease. 61
33605065 2021
50
Angiosarcoma of the Abdominal Aorta After Endovascular Aneurysm Repair: 18F-FDG PET/CT Imaging Findings. 61
33156052 2021

Variations for Angiosarcoma

ClinVar genetic disease variations for Angiosarcoma:

6
# Gene Name Type Significance ClinVarId dbSNP ID Position
1 MED12 NM_005120.3(MED12):c.130G>A (p.Gly44Ser) SNV other 92217 rs199469669 GRCh37: X:70339253-70339253
GRCh38: X:71119403-71119403
2 KRAS NM_033360.4(KRAS):c.57G>T (p.Leu19Phe) SNV Likely pathogenic 376034 rs121913538 GRCh37: 12:25398262-25398262
GRCh38: 12:25245328-25245328
3 KRAS NM_033360.4(KRAS):c.57G>C (p.Leu19Phe) SNV Likely pathogenic 217822 rs121913538 GRCh37: 12:25398262-25398262
GRCh38: 12:25245328-25245328

Cosmic variations for Angiosarcoma:

9 (show top 50) (show all 1392)
# Cosmic Mut ID Gene Symbol COSMIC Disease Classification
(Primary site, Site subtype, Primary histology, Histology subtype)
Mutation CDS Mutation AA GRCh38 Location Conf
1 COSM94290791 XIAP soft tissue,blood vessel,angiosarcoma,NS c.73G>C p.E25Q 23:123885735-123885735 3
2 COSM92181036 XIAP soft tissue,blood vessel,angiosarcoma,NS c.73G>C p.E25Q 23:123885735-123885735 3
3 COSM108084128 XIAP soft tissue,blood vessel,angiosarcoma,NS c.73G>C p.E25Q 23:123885735-123885735 3
4 COSM91383022 WT1 soft tissue,blood vessel,angiosarcoma,NS c.983G>A p.W328* 11:32416508-32416508 3
5 COSM148593299 WT1 soft tissue,blood vessel,angiosarcoma,NS c.983G>A p.W328* 11:32416508-32416508 3
6 COSM100781781 WT1 soft tissue,blood vessel,angiosarcoma,NS c.347G>A p.W116* 11:32416508-32416508 3
7 COSM149739973 WT1 soft tissue,blood vessel,angiosarcoma,NS c.983G>A p.W328* 11:32416508-32416508 3
8 COSM130518667 WT1 soft tissue,blood vessel,angiosarcoma,NS c.314+1069G>A p.? 11:32416508-32416508 3
9 COSM113485009 WT1 soft tissue,blood vessel,angiosarcoma,NS c.983G>A p.W328* 11:32416508-32416508 3
10 COSM111539054 WT1 soft tissue,blood vessel,angiosarcoma,NS c.950+1069G>A p.? 11:32416508-32416508 3
11 COSM147426307 WT1 soft tissue,blood vessel,angiosarcoma,NS c.950+1069G>A p.? 11:32416508-32416508 3
12 COSM149814991 TSC2 soft tissue,blood vessel,angiosarcoma,NS c.1073G>A p.W358* 16:2060767-2060767 3
13 COSM151514404 TSC2 soft tissue,blood vessel,angiosarcoma,NS c.4510C>T p.R1504W 16:2085245-2085245 3
14 COSM150365953 TSC2 soft tissue,blood vessel,angiosarcoma,NS c.1073G>A p.W358* 16:2060767-2060767 3
15 COSM151378978 TSC2 soft tissue,blood vessel,angiosarcoma,NS c.2494C>G p.H832D 16:2074338-2074338 3
16 COSM151755621 TSC2 soft tissue,blood vessel,angiosarcoma,NS c.2494C>G p.H832D 16:2074338-2074338 3
17 COSM150324884 TSC2 soft tissue,blood vessel,angiosarcoma,NS c.1073G>A p.W358* 16:2060767-2060767 3
18 COSM150315618 TSC2 soft tissue,blood vessel,angiosarcoma,NS c.4384C>T p.R1462W 16:2085245-2085245 3
19 COSM102634843 TSC2 soft tissue,blood vessel,angiosarcoma,NS c.2494C>G p.H832D 16:2074338-2074338 3
20 COSM148983289 TSC2 soft tissue,blood vessel,angiosarcoma,NS c.4387C>T p.R1463W 16:2085245-2085245 3
21 COSM101196039 TSC2 soft tissue,blood vessel,angiosarcoma,NS c.2347C>G p.H783D 16:2074338-2074338 3
22 COSM136629025 TSC2 soft tissue,blood vessel,angiosarcoma,NS c.1106G>A p.W369* 16:2060767-2060767 3
23 COSM87041278 TSC2 soft tissue,blood vessel,angiosarcoma,NS c.1073G>A p.W358* 16:2060767-2060767 3
24 COSM148996186 TSC2 soft tissue,blood vessel,angiosarcoma,NS c.2494C>G p.H832D 16:2074338-2074338 3
25 COSM150586941 TSC2 soft tissue,blood vessel,angiosarcoma,NS c.2494C>G p.H832D 16:2074338-2074338 3
26 COSM147712334 TSC2 soft tissue,blood vessel,angiosarcoma,NS c.2494C>G p.H832D 16:2074338-2074338 3
27 COSM110114858 TSC2 soft tissue,blood vessel,angiosarcoma,NS c.962G>A p.W321* 16:2060767-2060767 3
28 COSM136619132 TSC2 soft tissue,blood vessel,angiosarcoma,NS c.4417C>T p.R1473W 16:2085245-2085245 3
29 COSM90415849 TSC2 soft tissue,blood vessel,angiosarcoma,NS c.1073G>A p.W358* 16:2060767-2060767 3
30 COSM151518304 TSC2 soft tissue,blood vessel,angiosarcoma,NS c.1073G>A p.W358* 16:2060767-2060767 3
31 COSM110120269 TSC2 soft tissue,blood vessel,angiosarcoma,NS c.2383C>G p.H795D 16:2074338-2074338 3
32 COSM149803740 TSC2 soft tissue,blood vessel,angiosarcoma,NS c.4453C>T p.R1485W 16:2085245-2085245 3
33 COSM150369517 TSC2 soft tissue,blood vessel,angiosarcoma,NS c.2494C>G p.H832D 16:2074338-2074338 3
34 COSM150584116 TSC2 soft tissue,blood vessel,angiosarcoma,NS c.1073G>A p.W358* 16:2060767-2060767 3
35 COSM110105462 TSC2 soft tissue,blood vessel,angiosarcoma,NS c.4276C>T p.R1426W 16:2085245-2085245 3
36 COSM90421077 TSC2 soft tissue,blood vessel,angiosarcoma,NS c.2494C>G p.H832D 16:2074338-2074338 3
37 COSM151371641 TSC2 soft tissue,blood vessel,angiosarcoma,NS c.4579C>T p.R1527W 16:2085245-2085245 3
38 COSM151519993 TSC2 soft tissue,blood vessel,angiosarcoma,NS c.2494C>G p.H832D 16:2074338-2074338 3
39 COSM149820498 TSC2 soft tissue,blood vessel,angiosarcoma,NS c.2494C>G p.H832D 16:2074338-2074338 3
40 COSM87029815 TSC2 soft tissue,blood vessel,angiosarcoma,NS c.4585C>T p.R1529W 16:2085245-2085245 3
41 COSM151748696 TSC2 soft tissue,blood vessel,angiosarcoma,NS c.4456C>T p.R1486W 16:2085245-2085245 3
42 COSM101192082 TSC2 soft tissue,blood vessel,angiosarcoma,NS c.926G>A p.W309* 16:2060767-2060767 3
43 COSM102630453 TSC2 soft tissue,blood vessel,angiosarcoma,NS c.1073G>A p.W358* 16:2060767-2060767 3
44 COSM148992188 TSC2 soft tissue,blood vessel,angiosarcoma,NS c.1073G>A p.W358* 16:2060767-2060767 3
45 COSM147707387 TSC2 soft tissue,blood vessel,angiosarcoma,NS c.1073G>A p.W358* 16:2060767-2060767 3
46 COSM151376817 TSC2 soft tissue,blood vessel,angiosarcoma,NS c.1073G>A p.W358* 16:2060767-2060767 3
47 COSM87046601 TSC2 soft tissue,blood vessel,angiosarcoma,NS c.2494C>G p.H832D 16:2074338-2074338 3
48 COSM147694585 TSC2 soft tissue,blood vessel,angiosarcoma,NS c.4456C>T p.R1486W 16:2085245-2085245 3
49 COSM151753419 TSC2 soft tissue,blood vessel,angiosarcoma,NS c.1073G>A p.W358* 16:2060767-2060767 3
50 COSM101182298 TSC2 soft tissue,blood vessel,angiosarcoma,NS c.4240C>T p.R1414W 16:2085245-2085245 3

Expression for Angiosarcoma

Search GEO for disease gene expression data for Angiosarcoma.

Pathways for Angiosarcoma

Pathways related to Angiosarcoma according to GeneCards Suite gene sharing:

(show top 50) (show all 52)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.73 VIM VEGFA TP53 TIE1 TEK MYC
2
Show member pathways
13.71 VEGFA TP53 TIE1 TEK MYC KRAS
3
Show member pathways
13.69 TP53 TIE1 TEK MYC KRAS KDR
4
Show member pathways
13.49 VEGFA TP53 TIE1 TEK MYC KRAS
5
Show member pathways
13.42 TP53 TIE1 TEK KRAS KDR FLT4
6
Show member pathways
13.34 VWF VIM VEGFA TP53 TEK MYC
7
Show member pathways
13.31 TIE1 TEK KRAS KDR FLT4 FLT1
8
Show member pathways
13.29 VWF VEGFA TP53 TEK PECAM1 PDPN
9
Show member pathways
13.25 VIM VEGFA TIE1 TEK KRAS KDR
10
Show member pathways
12.94 TIE1 TEK KDR FLT4 FLT1
11
Show member pathways
12.91 VWF VEGFA KRAS KDR FLT4 FLT1
12 12.83 VEGFA TP53 MYC KRAS FLT4
13
Show member pathways
12.7 VEGFA TEK KRAS KDR FLT4 FLT1
14
Show member pathways
12.69 VWF VEGFA TP53 TEK MYC KRAS
15
Show member pathways
12.63 VEGFA TP53 MYC KRAS KDR
16 12.58 VEGFA TP53 TEK MYC KRAS KDR
17
Show member pathways
12.55 TP53 TIE1 TEK MYC KRAS ANGPT2
18 12.54 VIM VEGFA TP53 MYC KRAS
19
Show member pathways
12.49 TP53 KRAS KDR FLT4 FLT1
20
Show member pathways
12.44 TP53 KRAS KDR FLT4 FLT1
21
Show member pathways
12.44 VEGFA TP53 MYC MUC1 KRAS KDR
22
Show member pathways
12.42 VEGFA KRAS KDR FLT4 FLT1
23
Show member pathways
12.39 VEGFA KDR FLT4 FLT1
24 12.39 VEGFA KDR FLT4 FLT1
25
Show member pathways
12.38 TIE1 TEK KRAS KDR FLT4 FLT1
26 12.29 VEGFA TP53 MYC KRAS KDR
27 12.28 TP53 MYC FLT1 FLI1
28
Show member pathways
12.24 TEK PECAM1 KRAS ANGPT2
29
Show member pathways
12.23 TP53 MYC KRAS KDR FLT4 FLT1
30 12.2 VEGFA TP53 MYC KRAS
31 12.16 VEGFA TP53 MYC KRAS
32 12.11 VEGFA TP53 PECAM1 KDR
33
Show member pathways
12.11 TP53 TIE1 TEK MYC KRAS KDR
34 12.08 VIM VEGFA MYC KDR
35 12.05 TIE1 TEK KDR FLT4 FLT1 ANGPT2
36 11.96 VEGFA TEK FLT1 ANGPT2
37 11.95 VIM VEGFA TP53 MYC MUC1
38
Show member pathways
11.86 VEGFA KDR FLT4 FLT1
39
Show member pathways
11.8 KRAS KDR FLT4 FLT1
40 11.73 VEGFA TP53 MYC KRAS
41 11.72 TP53 MYC KRAS
42 11.72 VEGFA TP53 KDR FLT1
43 11.57 VEGFA TP53 MYC
44 11.54 VEGFA MYC KRAS
45 11.4 VEGFA TP53 ANGPT2
46
Show member pathways
11.35 KDR FLT4 FLT1
47 11.35 VEGFA KDR FLT1
48 11.17 VWF TEK PECAM1 PDPN KDR FLT4
49 11.14 VEGFA TEK KDR FLT1
50 11.07 VEGFA MYC KRAS KDR FLT4 FLT1

GO Terms for Angiosarcoma

Cellular components related to Angiosarcoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular region GO:0005576 9.91 VWF VEGFA TEK MUC1 KDR FLT4
2 membrane raft GO:0045121 9.55 TEK PECAM1 PDPN KRAS KDR
3 receptor complex GO:0043235 9.35 TIE1 TEK KDR FLT4 FLT1
4 integral component of plasma membrane GO:0005887 9.28 TIE1 TEK PECAM1 PDPN MUC1 KDR

Biological processes related to Angiosarcoma according to GeneCards Suite gene sharing:

(show all 42)
# Name GO ID Score Top Affiliating Genes
1 multicellular organism development GO:0007275 10.19 VEGFA TIE1 TEK PDPN KDR FLT4
2 positive regulation of cell proliferation GO:0008284 10.1 VEGFA MYC KRAS KDR FLT4 FLT1
3 positive regulation of gene expression GO:0010628 10.08 VIM VEGFA TP53 MYC KRAS CD34
4 negative regulation of apoptotic process GO:0043066 10.05 VEGFA TP53 TEK PDPN MYC KDR
5 positive regulation of cell migration GO:0030335 9.99 VEGFA PECAM1 PDPN KDR FLT1
6 positive regulation of ERK1 and ERK2 cascade GO:0070374 9.97 VEGFA TEK KDR FLT4
7 leukocyte migration GO:0050900 9.97 TEK PECAM1 CD34 ANGPT2
8 cytokine-mediated signaling pathway GO:0019221 9.97 VIM VEGFA TP53 MYC MUC1 KRAS
9 protein autophosphorylation GO:0046777 9.95 TEK KDR FLT4 FLT1
10 peptidyl-tyrosine phosphorylation GO:0018108 9.88 TIE1 TEK KDR FLT4 FLT1
11 positive regulation of phosphatidylinositol 3-kinase signaling GO:0014068 9.87 VEGFA TEK KDR FLT1
12 positive regulation of endothelial cell proliferation GO:0001938 9.86 VEGFA TEK KDR FLT4
13 vasculogenesis GO:0001570 9.85 VEGFA TIE1 KDR
14 positive regulation of endothelial cell migration GO:0010595 9.85 VEGFA TEK KDR FLT4
15 transmembrane receptor protein tyrosine kinase signaling pathway GO:0007169 9.85 TIE1 TEK KDR FLT4 FLT1
16 vascular endothelial growth factor receptor signaling pathway GO:0048010 9.84 VEGFA KDR FLT4 FLT1
17 positive regulation of MAP kinase activity GO:0043406 9.83 VEGFA KRAS FLT1
18 hematopoietic progenitor cell differentiation GO:0002244 9.83 TP53 KDR FLT4 FLT1
19 positive regulation of MAPK cascade GO:0043410 9.8 VEGFA TEK KDR FLT4 FLT1
20 positive regulation of protein phosphorylation GO:0001934 9.8 VEGFA TEK PECAM1 KRAS KDR FLT4
21 sprouting angiogenesis GO:0002040 9.79 VEGFA TEK FLT4
22 blood vessel morphogenesis GO:0048514 9.77 FLT4 FLT1 ANGPT2
23 positive regulation of focal adhesion assembly GO:0051894 9.75 VEGFA TEK KDR
24 positive regulation of vasculogenesis GO:2001214 9.69 KDR CD34
25 positive regulation of positive chemotaxis GO:0050927 9.68 VEGFA KDR
26 lymphangiogenesis GO:0001946 9.68 PDPN FLT4
27 positive regulation of protein kinase C signaling GO:0090037 9.68 VEGFA FLT4
28 positive regulation of cellular component movement GO:0051272 9.68 VEGFA PDPN
29 cellular response to vascular endothelial growth factor stimulus GO:0035924 9.67 VEGFA KDR FLT4 FLT1
30 positive regulation of transcription from RNA polymerase II promoter in response to stress GO:0036003 9.66 TP53 MUC1
31 positive regulation of transcription from RNA polymerase II promoter in response to hypoxia GO:0061419 9.65 VEGFA TP53
32 positive regulation of endothelial cell chemotaxis by VEGF-activated vascular endothelial growth factor receptor signaling pathway GO:0038033 9.65 VEGFA KDR
33 positive regulation of kinase activity GO:0033674 9.65 TIE1 TEK KDR FLT4 FLT1
34 vascular endothelial growth factor receptor-2 signaling pathway GO:0036324 9.64 VEGFA KDR
35 endothelium development GO:0003158 9.64 KDR CD34
36 glomerulus vasculature development GO:0072012 9.63 TEK ANGPT2
37 Tie signaling pathway GO:0048014 9.63 TEK ANGPT2
38 glomerular endothelium development GO:0072011 9.62 PECAM1 CD34
39 vascular wound healing GO:0061042 9.61 VEGFA KDR CD34
40 angiogenesis GO:0001525 9.5 VEGFA TIE1 TEK KDR FLT4 FLT1
41 vascular endothelial growth factor signaling pathway GO:0038084 9.46 VEGFA KDR FLT4 FLT1
42 positive regulation of angiogenesis GO:0045766 9.17 VEGFA TIE1 TEK KDR FLT1 CD34

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

# Name GO ID Score Top Affiliating Genes
1 protein binding GO:0005515 10.35 VWF VIM VEGFA TP53 TIE1 TEK
2 protein kinase activity GO:0004672 9.72 TIE1 TEK KDR FLT4 FLT1
3 growth factor binding GO:0019838 9.56 TEK KDR FLT4 FLT1
4 protein tyrosine kinase activity GO:0004713 9.55 TIE1 TEK KDR FLT4 FLT1
5 vascular endothelial growth factor-activated receptor activity GO:0005021 9.13 KDR FLT4 FLT1
6 transmembrane receptor protein tyrosine kinase activity GO:0004714 9.02 TIE1 TEK KDR FLT4 FLT1

Sources for Angiosarcoma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
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
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Apr-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
Content
Loading form....