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MCID: LYM008
MIFTS: 53

Lymphangiosarcoma

Categories: Cancer diseases, Immune diseases, Rare diseases, Skin diseases
Genes Tissues Related diseases Publications Pathways Drugs
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Aliases & Classifications for Lymphangiosarcoma

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MalaCards integrated aliases for Lymphangiosarcoma:

Name: Lymphangiosarcoma 12 44 15 73
Stewart Treves Syndrome 53 44
Skin Lymphangiosarcoma 12 73
Angiosarcoma Associated with Chronic Lymphedema 53
Lymphangiosarcoma of Stewart and Treves 12
Lymphangiosarcoma Following Mastectomy 53
Postmastectomy Extremity Angiosarcoma 53
Malignant Lymphangioendothelioma 12
Stewart-Treves Syndrome 12

Classifications:

MalaCards categories:
Global: Rare diseases Cancer diseases
Anatomical: Skin diseases Immune diseases
See all MalaCards categories (disease lists)


External Ids:

Disease Ontology 12 DOID:2689
NCIt 50 C3205 C4490
SNOMED-CT 68 62497000 63373002
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Summaries for Lymphangiosarcoma

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NIH Rare Diseases : 53 Stewart Treves syndrome is a rare angiosarcoma that develops in people with long-standing lymphedema. Most affected people have a history of breast cancer that was treated with a radical mastectomy. Early signs of the condition vary and may include purplish patches that develop into skin nodules, a mass under the skin, or a poorly healing eschar with recurrent bleeding and oozing. The exact underlying cause of the condition is poorly understood. Although Stewart Treves syndrome is always associated with long-standing lymphedema, other unknown factors also appear to contribute to the development of the condition. Early diagnosis and aggressive surgical treatment may improve the long-term outlook of the condition. Chemotherapy, radiation therapy, and immunotherapy continue to be studied as possible treatment options and may be recommended in some cases.

MalaCards based summary : Lymphangiosarcoma, also known as stewart treves syndrome, is related to cervical cancer and filariasis. An important gene associated with Lymphangiosarcoma is PROX1 (Prospero Homeobox 1), and among its related pathways/superpathways are TGF-Beta Pathway and Response to elevated platelet cytosolic Ca2+. The drugs Paclitaxel and Doxorubicin have been mentioned in the context of this disorder. Affiliated tissues include skin, endothelial and breast, and related phenotypes are cardiovascular system and cellular

Disease Ontology : 12 A lymphatic system cancer that has material basis in endothelial cells located in lymphatic vessels.

Wikipedia : 76 Lymphangiosarcoma is a rare cancer which occurs in long-standing cases of primary or secondary... more...

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Related Diseases for Lymphangiosarcoma

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

(show top 50) (show all 121)
# Related Disease Score Top Affiliating Genes
1 cervical cancer 30.5 FLT4 MYC TYMP VEGFA VEGFC
2 filariasis 30.4 FLT4 IL2 TNF
3 congenital lymphedema 30.3 FLT4 PDPN VEGFC
4 angiosarcoma 30.3 FLT4 MYC PROX1 VEGFA VWF
5 lymphangioma 30.0 FLT4 HBA1 PDPN PROX1 VEGFA VEGFC
6 lymphedema 10.7
7 angiokeratoma of fordyce 10.3 HBA1 PROX1
8 pulmonary artery leiomyosarcoma 10.3 HBA1 SMUG1
9 histiocytoid hemangioma 10.3 F8 HBA1
10 lymphatic malformations 10.3 FLT4 PROX1
11 human herpesvirus 8 10.3
12 spinal cord injury 10.3
13 lymphoma 10.3
14 lymphosarcoma 10.3
15 intravascular papillary endothelial hyperplasia 10.3
16 hemangioma of spleen 10.3 FLT4 SMUG1
17 oral tuberculosis 10.3 IL2 TNF
18 fainting 10.2 F8 VWF
19 indeterminate leprosy 10.2 F8 HBA1 TNF
20 gorham's disease 10.2 FLT4 VEGFC
21 brain sarcoma 10.2 HBA1 SMUG1
22 cerebral arteritis 10.2 F8 VWF
23 pericardium cancer 10.2 F8 HBA1 SMUG1
24 splenic tuberculosis 10.2 IL2 SMUG1
25 hemifacial spasm 10.2 FLT4 VEGFA
26 breast cancer 10.2
27 elephantiasis 10.2
28 orbital lymphangioma 10.2 FLT4 PDPN
29 uveoparotid fever 10.2 LYZ TNF
30 mucinous cystadenocarcinoma of the pancreas 10.2 PDPN VEGFC
31 skin hemangioma 10.2 HBA1 PROX1 VEGFA
32 erythema multiforme 10.2 DSP IL2 TNF
33 critical limb ischemia 10.2 TNF VEGFA
34 streptococcal toxic-shock syndrome 10.1 IL2 TNF
35 hypoglycemic coma 10.1 HBA1 VEGFA
36 breast carcinoma in situ 10.1 FLT4 VEGFA VEGFC
37 female reproductive system disease 10.1 SMUG1 TNF VEGFA
38 drug-induced lupus erythematosus 10.1 HBA1 VEGFA VWF
39 hepatopulmonary syndrome 10.1 TNF VEGFA VWF
40 limb ischemia 10.1 TNF VEGFA VWF
41 infiltrating angiolipoma 10.1 VEGFA VEGFC VWF
42 al amyloidosis 10.1 LYZ VWF
43 nasopharyngeal disease 10.1 MYC SMUG1 VEGFA
44 portal hypertension 10.1 TNF VEGFA VWF
45 pharynx cancer 10.1 MYC SMUG1 VEGFA
46 capillary lymphangioma 10.1 HBA1 LYVE1 PROX1
47 cervix disease 10.1 MYC SMUG1 VEGFA
48 hereditary lymphedema 10.1
49 sarcoma 10.1
50 arteries, anomalies of 10.1 TNF VEGFA VWF

Graphical network of the top 20 diseases related to Lymphangiosarcoma:



Diseases related to Lymphangiosarcoma
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Symptoms & Phenotypes for Lymphangiosarcoma

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MGI Mouse Phenotypes related to Lymphangiosarcoma:

46 (show all 12)
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 10.36 DSP ESAM FLT4 IL2 KITLG LYVE1
2 cellular MP:0005384 10.34 CD34 DSP ESAM IL2 KITLG LYVE1
3 homeostasis/metabolism MP:0005376 10.31 CD34 F8 FLT4 IL2 KITLG LYZ
4 immune system MP:0005387 10.31 CD34 ESAM F8 FLT4 IL2 KITLG
5 hematopoietic system MP:0005397 10.27 CD34 ESAM F8 IL2 KITLG LYVE1
6 digestive/alimentary MP:0005381 10.22 DSP FLT4 IL2 KITLG LYZ MYC
7 mortality/aging MP:0010768 10.1 DSP F8 FLT4 IL2 KITLG LYZ
8 integument MP:0010771 10.02 CD34 DSP FLT4 KITLG LYZ MYC
9 liver/biliary system MP:0005370 9.92 FLT4 IL2 KITLG LYZ MYC PROX1
10 neoplasm MP:0002006 9.81 CD34 ESAM IL2 KITLG LYZ MYC
11 no phenotypic analysis MP:0003012 9.5 FLT4 IL2 LYZ MYC TNF VEGFA
12 reproductive system MP:0005389 9.28 F8 FLT4 IL2 KITLG LYZ MYC
Sources

Drugs & Therapeutics for Lymphangiosarcoma

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

(show all 23)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Paclitaxel Approved, Vet_approved Phase 2 33069-62-4 36314
2
Doxorubicin Approved, Investigational Phase 2 23214-92-8 31703
3
Mechlorethamine Approved, Investigational Phase 2 51-75-2 4033
4
Mesna Approved, Investigational Phase 2 3375-50-6 598
5
Ifosfamide Approved Phase 2 3778-73-2 3690
6
Doxil Approved June 1999 Phase 2 31703
7 Antineoplastic Agents, Phytogenic Phase 2
8 Antimitotic Agents Phase 2
9 Albumin-Bound Paclitaxel Phase 2
10 Alkylating Agents Phase 2
11 Antineoplastic Agents, Alkylating Phase 2
12
Isophosphamide mustard Phase 2 0
13 Antibiotics, Antitubercular Phase 2
14 Anti-Bacterial Agents Phase 2
15 Topoisomerase Inhibitors Phase 2
16
Fluorouracil Approved Phase 1 51-21-8 3385
17
Belinostat Approved, Investigational Phase 1 866323-14-0
18 Androgens Phase 1
19 Antimetabolites, Antineoplastic Phase 1
20 Immunosuppressive Agents Phase 1
21 Immunologic Factors Phase 1
22 Histone Deacetylase Inhibitors Phase 1
23 Antimetabolites Phase 1

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Paclitaxel in Treating Patients With Locally Advanced or Metastatic Soft Tissue Angiosarcoma or Lymphangiosarcoma That Cannot Be Removed By Surgery Completed NCT00217607 Phase 2 paclitaxel
2 Hypofractionated Radiotherapy With Sequential Chemotherapy in Primary Unresectable or Marginally Resectable Soft Tissue Sarcomas of Extremities or Trunk Wall Recruiting NCT03651375 Phase 2 Sequential chemotherapy - 3 courses of AI
3 Study of PXD101 Alone and in Combination With 5-Fluorouracil (5-FU) in Patients With Advanced Solid Tumors Completed NCT00413322 Phase 1 belinostat;5-Fluorouracil (5-FU)

Search NIH Clinical Center for Lymphangiosarcoma

Cochrane evidence based reviews: lymphangiosarcoma

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Genetic Tests for Lymphangiosarcoma

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Anatomical Context for Lymphangiosarcoma

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MalaCards organs/tissues related to Lymphangiosarcoma:

41
Skin, Endothelial, Breast, Lung, Liver, Spleen, Pancreas

The Foundational Model of Anatomy Ontology organs/tissues related to Lymphangiosarcoma:

19
Lymphatic Vessels
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Publications for Lymphangiosarcoma

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Articles related to Lymphangiosarcoma:

(show top 50) (show all 247)
# Title Authors Year
1
Successful management of lymphangiosarcoma in a puppy using a tyrosine kinase inhibitor. ( 29606721 )
2018
2
Cutaneous Angiosarcoma Postmastectomy (Stewart-Treves Syndrome). ( 30320298 )
2018
3
Stewart-Treves syndrome: Magnetic resonance imaging data compared with pathological results from a single center. ( 29391898 )
2018
4
Pretibial Located Stewart-Treves Syndrome: Uncommon Presentation in a Bulgarian Patient! ( 29731936 )
2018
5
Lymphangiosarcoma of the hip arising in a congenital non-irradiated lymphangioma. ( 29068520 )
2017
6
Pathology in Practice. Ventral abdominal lymphangiosarcoma. ( 28263114 )
2017
7
Stewart-Treves Syndrome on the Lower Extremity Associated to Idiopathic Chronic Lymphedema Visualized on FDG PET/CT. ( 29035997 )
2017
8
Stewart-Treves Syndrome Involving Chronic Lymphedema after Mastectomy of Breast Cancer. ( 28280645 )
2017
9
Stewart-Treves syndrome in a spinal cord injury patient with MYC amplification. ( 29264380 )
2017
10
Pancreatic metastasis of angiosarcoma (Stewart-Treves syndrome) diagnosed using endoscopic ultrasound-guided fine needle aspiration: A case report. ( 27537557 )
2016
11
Modeling lymphangiosarcoma in mice. ( 27105012 )
2016
12
Lymphangiosarcoma: Is Stewart-Treves Syndrome a Preventable Condition? ( 26584023 )
2016
13
Angiosarcoma on Lymphedema (Stewart-Treves Syndrome): A 12-Year Follow-up after Isolated Limb Perfusion, Limb Infusion, and Electrochemotherapy. ( 26916941 )
2016
14
Stewart Treves Syndrome in a Woman with Mastectomy. ( 27042566 )
2016
15
Stewart-Treves Syndrome: A Case Report and Review of the Literature. ( 27099606 )
2016
16
An unusual presentation of Stewart-Treves syndrome on the lower extremity. ( 27766300 )
2016
17
A case of Stewart-Treves syndrome with the longest lag: 44 years after radical mastectomy for breast cancer. ( 29896826 )
2016
18
Constitutive Activation of mTORC1 in Endothelial Cells Leads to the Development and Progression of Lymphangiosarcoma through VEGF Autocrine Signaling. ( 26777415 )
2015
19
Lymphangiosarcoma with bone formation of the auricle in a dog. ( 25716121 )
2015
20
Presumed primary ocular lymphangiosarcoma with metastasis in a miniature horse. ( 25581559 )
2015
21
Lymphangiosarcoma with systemic metastases in a Japanese domestic cat. ( 25482607 )
2015
22
Angiosarcoma (Stewart-Treves syndrome) in postmastectomy patients: report of 10 cases and review of literature. ( 26617830 )
2015
23
Case of angiosarcoma on the abdominal wall, an extremely rare variant, putatively shared the pathogenesis with Stewart-Treves syndrome. ( 25703383 )
2015
24
Stewart-Treves Phenomenon: Lymphangiosarcoma Arising in Lymphedematous Tissue Is not a Syndrome. ( 25658055 )
2015
25
Stewart-Treves Syndrome of the Breast after Quadrantectomy for Breast Carcinoma. ( 26205517 )
2015
26
Stewart-Treves Syndrome after Bilateral Mastectomy and Radiotherapy for Breast Carcinoma: Case Report. ( 28331699 )
2015
27
Stewart-Treves syndrome following idiopathic leg lymphoedema: remember sarcoma. ( 26075513 )
2015
28
Long-term control of pleural metastasis in Stewart-Treves syndrome with single-agent chemotherapies followed by maintenance chemotherapy. ( 26213822 )
2015
29
Stewart Treves Syndrome. ( 26312725 )
2015
30
Stewart-Treves Syndrome of the Lower Extremity. ( 26312726 )
2015
31
Lymphangiosarcoma in 12 dogs: a case series (1998-2013). ( 24612140 )
2014
32
Complex karyotype in a case of cutaneous lymphangiosarcoma associated with chronic lymphedema of the lower limb. ( 24947410 )
2014
33
Lymphangiosarcoma of the scalp. ( 24636440 )
2014
34
Lymphangiosarcoma with strong positivity of D2-40 and LYVE-1 presenting different clinical features from angiosarcoma. ( 24985547 )
2014
35
Lymphangiosarcoma of the scalp. ( 24342442 )
2014
36
Lymphangiosarcoma of mons pubis following radiotherapy for carcinoma cervix. ( 25404831 )
2014
37
An unusual case of skin metastasis from a breast cancer mimicking a lymphangiosarcoma. ( 24977428 )
2014
38
Post-mastectomy benign lymphangioendothelioma of the skin following chronic lymphedema for breast carcinoma: a teaching case mimicking low-grade angiosarcoma and masquerading as Stewart-Treves syndrome. ( 25358645 )
2014
39
Mailgnant degeneration to lymphangiosarcoma of a chronic lymphedema in the lower left extremity. ( 25304756 )
2014
40
Stewart-Treves syndrome: a case report. ( 25574331 )
2014
41
Distant metastases in a young woman with Stewart-Treves syndrome demonstrated by an FDG-PET/CT scan. ( 24561687 )
2014
42
Stewart-Treves syndrome. ( 24604055 )
2014
43
Cutaneous lymphangiosarcoma following chronic lymphedema of filarial origin. ( 23372218 )
2013
44
Chronic lymphedema of filarial origin: a very rare etiology of cutaneous lymphangiosarcoma. ( 23372219 )
2013
45
A novel approach to treatment of lymphangiosarcoma in a boxer dog. ( 23560802 )
2013
46
Stewart-Treves syndrome angiosarcoma expresses phenotypes of both blood and lymphatic capillaries. ( 23324269 )
2013
47
Clinical experience of stewart-treves syndrome in the lower leg. ( 23730611 )
2013
48
Stewart-Treves syndrome: a case report. ( 23838488 )
2013
49
Lymphangiosarcoma and filariasis. ( 22575002 )
2012
50
Use of surgery and mitoxantrone chemotherapy in a dog with disseminated lymphangiosarcoma. ( 23216040 )
2012
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Variations for Lymphangiosarcoma

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Expression for Lymphangiosarcoma

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Search GEO for disease gene expression data for Lymphangiosarcoma.
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Pathways for Lymphangiosarcoma

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Pathways related to Lymphangiosarcoma according to GeneCards Suite gene sharing:

(show all 26)
# Super pathways Score Top Affiliating Genes
1
TGF-Beta Pathway 64
Show member pathways
 13.44 FLT4 IL2 KITLG MYC TNF TYMP
2
Response to elevated platelet cytosolic Ca2+ 66
Show member pathways
 13.19 ESAM F8 IL2 PDPN VEGFA VEGFC
3
Nanog in Mammalian ESC Pluripotency 64
Show member pathways
 13.14 FLT4 IL2 KITLG TNF TYMP VEGFA
4
Pathways in cancer 37
12.68 FLT4 IL2 KITLG MYC VEGFA VEGFC
5
PI3K-Akt signaling pathway 37
Show member pathways
 12.6 FLT4 IL2 KITLG MYC TNF VEGFA
6
MAPK signaling pathway 37 1
12.58 FLT4 KITLG MYC TNF VEGFA VEGFC
7
NF-KappaB Family Pathway 64
Show member pathways
 12.5 FLT4 IL2 KITLG TNF TYMP VEGFC
8
G-protein signaling RAC1 in cellular process 22
Show member pathways
 12.29 FLT4 IL2 KITLG TYMP VEGFC
9
Embryonic and Induced Pluripotent Stem Cell Differentiation Pathways and Lineage-specific Markers 65
11.94 KITLG MYC PROX1 VEGFA
10
Hematopoietic Stem Cell Differentiation Pathways and Lineage-specific Markers 65
11.82 CD34 IL2 KITLG TNF
11
AGE-RAGE signaling pathway in diabetic complications 37
11.8 TNF VEGFA VEGFC
12
Hematopoietic cell lineage 37
11.79 CD34 KITLG TNF
13
Signaling events mediated by VEGFR1 and VEGFR2 1
Show member pathways
 11.76 FLT4 VEGFA VEGFC
14
Immune response IFN gamma signaling pathway 22
Show member pathways
 11.75 FLT4 IL2 KITLG MYC TYMP VEGFC
15
Immune response IFN alpha/beta signaling pathway 22
Show member pathways
 11.71 FLT4 IL2 KITLG MYC TNF TYMP
16
Innate Lymphoid Cell Differentiation Pathways 65
11.69 CD34 IL2 TNF
17
Dendritic Cells Developmental Lineage Pathway 65
11.64 CD34 IL2 TNF
18
Bladder cancer 37 1
11.62 MYC TYMP VEGFA
19
C-MYB transcription factor network 1
11.56 CD34 KITLG LYZ MYC
20
Photodynamic therapy-induced NF-kB survival signaling 1
Show member pathways
 11.53 IL2 TNF VEGFA
21
Aryl Hydrocarbon Receptor 1
11.39 MYC TNF VEGFA
22
Vemurafenib Pathway, Pharmacodynamics 61
11.32 FLT4 MYC VEGFA
23
Angiogenesis (CST) 4
11.05 FLT4 PDPN PROX1 TYMP VEGFC VWF
24
G-protein signaling_Rap2B regulation pathway 22
10.87 FLT4 IL2 KITLG TNF TYMP VEGFC
25
VEGF binds to VEGFR leading to receptor dimerization 66
Show member pathways
 10.7 FLT4 VEGFA VEGFC
26
miR-148a/miR-31/FIH1/HIF1andalpha;-Notch signaling in glioblastoma 1
10.53 VEGFA VEGFC
Sources

GO Terms for Lymphangiosarcoma

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

# Name GO ID Score Top Affiliating Genes
1 extracellular space GO:0005615 9.76 F8 HBA1 IL2 KITLG LYZ TNF
2 extracellular region GO:0005576 9.7 CD34 F8 FLT4 HBA1 IL2 KITLG
3 platelet alpha granule lumen GO:0031093 8.92 F8 VEGFA VEGFC VWF

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

(show all 28)
# Name GO ID Score Top Affiliating Genes
1 cell adhesion GO:0007155 9.97 CD34 ESAM IL2 KITLG LYVE1 VWF
2 cytokine-mediated signaling pathway GO:0019221 9.94 IL2 MYC TNF VEGFA
3 negative regulation of apoptotic process GO:0043066 9.93 FLT4 IL2 KITLG MYC PDPN VEGFA
4 negative regulation of gene expression GO:0010629 9.91 CD34 MYC TNF VEGFA
5 angiogenesis GO:0001525 9.9 FLT4 TYMP VEGFA VEGFC
6 MAPK cascade GO:0000165 9.88 IL2 KITLG MYC TNF
7 regulation of signaling receptor activity GO:0010469 9.85 IL2 KITLG TNF TYMP VEGFA VEGFC
8 lung development GO:0030324 9.8 PDPN PROX1 VEGFA
9 response to wounding GO:0009611 9.76 LYVE1 MYC VWF
10 positive regulation of epithelial cell proliferation GO:0050679 9.73 MYC VEGFA VEGFC
11 platelet degranulation GO:0002576 9.73 F8 VEGFA VEGFC VWF
12 positive regulation of MAP kinase activity GO:0043406 9.72 KITLG TNF VEGFA
13 vascular endothelial growth factor receptor signaling pathway GO:0048010 9.71 FLT4 VEGFA VEGFC
14 positive regulation of endothelial cell migration GO:0010595 9.69 FLT4 PROX1 VEGFA
15 ovarian follicle development GO:0001541 9.65 KITLG MYC VEGFA
16 negative regulation of glucose import GO:0046325 9.63 MYC TNF
17 induction of positive chemotaxis GO:0050930 9.62 VEGFA VEGFC
18 positive regulation of leukocyte migration GO:0002687 9.62 KITLG VEGFA
19 positive regulation of endothelial cell proliferation GO:0001938 9.62 FLT4 PROX1 VEGFA VEGFC
20 lymph vessel development GO:0001945 9.61 FLT4 PROX1
21 positive regulation of cellular component movement GO:0051272 9.59 PDPN VEGFA
22 sprouting angiogenesis GO:0002040 9.58 FLT4 VEGFA VEGFC
23 positive regulation of protein kinase C signaling GO:0090037 9.57 FLT4 VEGFA
24 positive regulation of protein phosphorylation GO:0001934 9.55 FLT4 IL2 TNF VEGFA VEGFC
25 positive regulation of mast cell chemotaxis GO:0060754 9.52 VEGFA VEGFC
26 positive regulation of cell proliferation GO:0008284 9.5 FLT4 IL2 KITLG MYC PROX1 VEGFA
27 vascular endothelial growth factor signaling pathway GO:0038084 9.43 FLT4 VEGFA VEGFC
28 lymphangiogenesis GO:0001946 8.8 FLT4 PDPN PROX1

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

# Name GO ID Score Top Affiliating Genes
1 cytokine activity GO:0005125 9.46 IL2 KITLG TNF VEGFA
2 growth factor activity GO:0008083 9.02 IL2 KITLG TYMP VEGFA VEGFC
3 vascular endothelial growth factor receptor 3 binding GO:0043185 8.96 VEGFA VEGFC
Sources

Sources for Lymphangiosarcoma

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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
The MalaCards human disease database index: 1-9 A B C D E F G H I J K L M N O P Q R S T U V W X Y Z
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