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MCID: LYM008
MIFTS: 51

Lymphangiosarcoma

Categories: Rare diseases, Cancer diseases, Immune 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: Immune diseases Skin diseases
See all MalaCards categories (disease lists)


External Ids:

Disease Ontology 12 DOID:2689
NCIt 50 C3205 C4490
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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 lymphedema and hereditary lymphedema. An important gene associated with Lymphangiosarcoma is PROX1 (Prospero Homeobox 1), and among its related pathways/superpathways are PI3K-Akt signaling pathway and MAPK signaling pathway. The drugs Paclitaxel and Albumin-Bound Paclitaxel have been mentioned in the context of this disorder. Affiliated tissues include lymphatic vessels, skin and endothelial, 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 malignant tumor 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 114)
# Related Disease Score Top Affiliating Genes
1 lymphedema 31.3 FLT4 GJC2 PROX1 VEGFC
2 hereditary lymphedema 31.0 FLT4 GJC2
3 congenital lymphedema 29.8 FLT4 PDPN VEGFC
4 angiosarcoma 29.6 FLT4 MYC PROX1 VEGFA VWF
5 lymphangioma 28.4 FLT4 HBA1 PDPN PROX1 VEGFA VEGFC
6 angiokeratoma of fordyce 10.9 HBA1 PROX1
7 lymphatic malformations 10.9 FLT4 PROX1
8 gorham's disease 10.8 FLT4 VEGFC
9 fainting 10.7 F8 VWF
10 mild hemophilia a 10.7 F8 VWF
11 hemifacial spasm 10.7 FLT4 VEGFA
12 acquired von willebrand syndrome 10.7 F8 VWF
13 hypoglycemic coma 10.7 HBA1 VEGFA
14 von willebrand disease, type 2 10.6 F8 VWF
15 cellulitis 10.6 FLT4 GJC2 TNF
16 von willebrand disease, type 3 10.6 F8 VWF
17 pulmonary artery leiomyosarcoma 10.6 HBA1 SMUG1
18 cerebral arteritis 10.6 F8 VWF
19 hemangioma of spleen 10.6 FLT4 SMUG1
20 intravascular fasciitis 10.6 CD34 HBA1
21 von willebrand disease, type 1 10.6 F8 VWF
22 thyroid angiosarcoma 10.6 CD34 HBA1
23 neurogenic arthropathy 10.6 CD34 HBA1
24 brain sarcoma 10.6 HBA1 SMUG1
25 lymphatic system disease 10.5 FLT4 GJC2 VEGFC
26 heart cancer 10.5 HBA1 SMUG1
27 capillary hemangioma 10.5 HBA1 PROX1 VEGFA
28 skin hemangioma 10.5 HBA1 PROX1 VEGFA
29 thyroid sarcoma 10.5 CD34 HBA1
30 epithelioid neurofibroma 10.5 CD34 DSP
31 cardiac sarcoidosis 10.5 SMUG1 TNF
32 hemangioma of liver 10.5 CD34 HBA1
33 critical limb ischemia 10.4 TNF VEGFA VWF
34 breast angiosarcoma 10.4 CD34 HBA1
35 leukodystrophy, hypomyelinating, 4 10.4 GJC2 MYC
36 breast carcinoma in situ 10.4 FLT4 VEGFA VEGFC
37 conventional angiosarcoma 10.4 CD34 HBA1
38 al amyloidosis 10.4 LYZ VWF
39 hepatopulmonary syndrome 10.4 TNF VEGFA VWF
40 limb ischemia 10.4 TNF VEGFA VWF
41 portal hypertension 10.4 TNF VEGFA VWF
42 infiltrating angiolipoma 10.4 VEGFA VEGFC VWF
43 arteries, anomalies of 10.3 TNF VEGFA VWF
44 mixed liposarcoma 10.3 CD34 HBA1
45 pericardium cancer 10.3 F8 HBA1 SMUG1
46 hennekam syndrome 10.3 FLT4 GJC2 PROX1 VEGFC
47 tibial adamantinoma 10.3 CD34 SMUG1
48 sternum cancer 10.3 CD34 SMUG1
49 deep angioma 10.3 CD34 VWF
50 breast leiomyosarcoma 10.3 CD34 SMUG1

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
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 10.22 DSP MYC FLT4 PDPN LYVE1 LYZ
2 cellular MP:0005384 10.2 LYZ DSP MYC GJC2 LYVE1 CD34
3 homeostasis/metabolism MP:0005376 10.15 LYZ F8 MYC FLT4 PDPN CD34
4 hematopoietic system MP:0005397 10.14 F8 MYC GJC2 PDPN LYVE1 CD34
5 immune system MP:0005387 10.13 MYC F8 FLT4 PDPN GJC2 LYVE1
6 digestive/alimentary MP:0005381 10.09 MYC FLT4 PDPN LYZ DSP PROX1
7 mortality/aging MP:0010768 9.97 DSP F8 MYC FLT4 GJC2 PDPN
8 integument MP:0010771 9.86 LYZ DSP MYC FLT4 CD34 VEGFA
9 muscle MP:0005369 9.5 DSP MYC FLT4 PDPN VEGFA PROX1
10 neoplasm MP:0002006 9.1 LYZ MYC CD34 VEGFA SMUG1 TNF
Sources

Drugs & Therapeutics for Lymphangiosarcoma

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

(show all 11)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Paclitaxel Approved, Vet_approved Phase 2 33069-62-4 36314
2 Albumin-Bound Paclitaxel Phase 2
3 Antimitotic Agents Phase 2
4 Antineoplastic Agents, Phytogenic Phase 2
5
Belinostat Approved, Investigational Phase 1 866323-14-0
6
Fluorouracil Approved Phase 1 51-21-8 3385
7 Androgens Phase 1
8 Antimetabolites Phase 1
9 Antimetabolites, Antineoplastic Phase 1
10 Histone Deacetylase Inhibitors Phase 1
11 Immunosuppressive Agents 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 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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The Foundational Model of Anatomy Ontology organs/tissues related to Lymphangiosarcoma:

19
Lymphatic Vessels

MalaCards organs/tissues related to Lymphangiosarcoma:

41
Skin, Endothelial, Cervix, Testes, Bone, Lung, Lymph Node
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Publications for Lymphangiosarcoma

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Articles related to Lymphangiosarcoma:

(show top 50) (show all 177)
# Title Authors Year
1
Successful management of lymphangiosarcoma in a puppy using a tyrosine kinase inhibitor. ( 29606721 )
2018
2
Lymphangiosarcoma of the hip arising in a congenital non-irradiated lymphangioma. ( 29068520 )
2017
3
Pathology in Practice. Ventral abdominal lymphangiosarcoma. ( 28263114 )
2017
4
Pancreatic metastasis of angiosarcoma (Stewart-Treves syndrome) diagnosed using endoscopic ultrasound-guided fine needle aspiration: A case report. ( 27537557 )
2016
5
Modeling lymphangiosarcoma in mice. ( 27105012 )
2016
6
Lymphangiosarcoma: Is Stewart-Treves Syndrome a Preventable Condition? ( 26584023 )
2016
7
Constitutive Activation of mTORC1 in Endothelial Cells Leads to the Development and Progression of Lymphangiosarcoma through VEGF Autocrine Signaling. ( 26777415 )
2015
8
Lymphangiosarcoma with bone formation of the auricle in a dog. ( 25716121 )
2015
9
Presumed primary ocular lymphangiosarcoma with metastasis in a miniature horse. ( 25581559 )
2015
10
Lymphangiosarcoma with systemic metastases in a Japanese domestic cat. ( 25482607 )
2015
11
Stewart-Treves Phenomenon: Lymphangiosarcoma Arising in Lymphedematous Tissue Is Not a Syndrome. ( 25658055 )
2015
12
Lymphangiosarcoma in 12 dogs: a case series (1998-2013). ( 24612140 )
2014
13
Complex karyotype in a case of cutaneous lymphangiosarcoma associated with chronic lymphedema of the lower limb. ( 24947410 )
2014
14
Lymphangiosarcoma of the scalp. ( 24636440 )
2014
15
Lymphangiosarcoma with strong positivity of D2-40 and LYVE-1 presenting different clinical features from angiosarcoma. ( 24985547 )
2014
16
Lymphangiosarcoma of the scalp. ( 24342442 )
2014
17
Lymphangiosarcoma of mons pubis following radiotherapy for carcinoma cervix. ( 25404831 )
2014
18
An unusual case of skin metastasis from a breast cancer mimicking a lymphangiosarcoma. ( 24977428 )
2014
19
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
20
Mailgnant degeneration to lymphangiosarcoma of a chronic lymphedema in the lower left extremity. ( 25304756 )
2014
21
Cutaneous lymphangiosarcoma following chronic lymphedema of filarial origin. ( 23372218 )
2013
22
Chronic lymphedema of filarial origin: a very rare etiology of cutaneous lymphangiosarcoma. ( 23372219 )
2013
23
A novel approach to treatment of lymphangiosarcoma in a boxer dog. ( 23560802 )
2013
24
Stewart-Treves syndrome angiosarcoma expresses phenotypes of both blood and lymphatic capillaries. ( 23324269 )
2013
25
Lymphangiosarcoma and filariasis. ( 22575002 )
2012
26
Use of surgery and mitoxantrone chemotherapy in a dog with disseminated lymphangiosarcoma. ( 23216040 )
2012
27
Lymphangiosarcoma of the thorax and thoracic vertebrae in a 16-year-old girl. ( 22915659 )
2012
28
Plasma-cell rich lymphangiosarcoma: an under-recognized variant and potential diagnostic pitfall. ( 22188566 )
2012
29
Lymphangiosarcoma arising after 33 years within a background of chronic filariasis: a case report with review of literature. ( 22211335 )
2012
30
Stewart-Treves syndrome arising in patients with lymphaticovenular anastomosis for chronic lymphedema of the leg. ( 22522788 )
2012
31
Elevated coagulation factor VIII plasma activity in a patient with lymphangiosarcoma. ( 23154736 )
2012
32
Establishment and characterization of a novel lymphangiosarcoma cell line (MO-LAS) compared with the hemangiosarcoma cell line (ISO-HAS). ( 23342252 )
2012
33
Locally invasive lymphangiosarcoma in a young domestic shorthair. ( 21880529 )
2011
34
Lymphangiosarcoma of the vocal cord: a rare entity defined by a D2-40 immunohistochemical and ultrastructural study. ( 20975065 )
2011
35
Intra-arterial mitoxantrone/paclitaxel in angiosarcoma of the lower limb associated with chronic lymphedema (Stewart-Treves syndrome) in a patient with cervical cancer. ( 21233070 )
2011
36
FDG-PET/CT imaging in a patient with lymphangiosarcoma. ( 20479592 )
2010
37
Lymphangiosarcoma complicating extensive congenital mixed vascular malformations. ( 20863270 )
2010
38
Angiosarcoma (Stewart-Treves syndrome): palliative role of Mohs' ointment. ( 20883379 )
2010
39
Can lymphangiosarcoma be resurrected? A clinicopathological and immunohistochemical study of lymphatic differentiation in 49 angiosarcomas. ( 20459536 )
2010
40
Fatal lymphangiosarcoma revealed by lymphoedema of the neck. ( 19479140 )
2009
41
Lymphangiosarcoma of the arm presenting with lymphedema in a woman 16 years after mastectomy: a case report. ( 19918554 )
2009
42
Lymphangiosarcoma in the nictitating membrane of a horse. ( 18182523 )
2008
43
Paresis in an Asian small clawed otter (Aonyx cinereus) associated with vertebral and ischial osteolysis caused by a malignant lymphangiosarcoma. ( 18634215 )
2008
44
Lymphangiosarcoma presenting as asymptomatic swelling of the cheek. ( 17317502 )
2007
45
Lymphangiosarcoma in a cat. ( 17706243 )
2007
46
Lymphangiosarcoma in two cats. ( 17307008 )
2007
47
Case 3. Upper limb lymphangiosarcoma following breast cancer therapy. ( 16549844 )
2006
48
Lymphangiosarcoma in a 3.5-year-old Bullmastiff bitch with vaginal prolapse, primary lymph node fibrosis and other congenital defects. ( 16300186 )
2005
49
Lymphangiosarcoma of dogs: a review. ( 16300179 )
2005
50
Lymphangiosarcoma associated with primary lymphedema in a Bouvier des Flandres. ( 14765744 )
2004
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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 12)
# Super pathways Score Top Affiliating Genes
1
PI3K-Akt signaling pathway 37
Show member pathways
 12.46 FLT4 MYC TNF VEGFA VEGFC VWF
2
MAPK signaling pathway 1 37
12.43 FLT4 MYC TNF VEGFA VEGFC
3
Embryonic and Induced Pluripotent Stem Cell Differentiation Pathways and Lineage-specific Markers 65
11.81 MYC PROX1 VEGFA
4
Interleukin-4 and 13 signaling 66
11.73 MYC TNF VEGFA
5
AGE-RAGE signaling pathway in diabetic complications 37
11.65 TNF VEGFA VEGFC
6
Signaling events mediated by VEGFR1 and VEGFR2 1
Show member pathways
 11.6 FLT4 VEGFA VEGFC
7
C-MYB transcription factor network 1
11.5 CD34 LYZ MYC
8
Aryl Hydrocarbon Receptor 1
11.18 MYC TNF VEGFA
9
Vemurafenib Pathway, Pharmacodynamics 61
11.06 FLT4 MYC VEGFA
10
Angiogenesis (CST) 4
10.97 FLT4 PDPN PROX1 VEGFC VWF
11
miR-148a/miR-31/FIH1/HIF1andalpha;-Notch signaling in glioblastoma 1
10.36 VEGFA VEGFC
12
VEGF ligand-receptor interactions 66
Show member pathways
 10.34 FLT4 VEGFA VEGFC
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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 region GO:0005576 9.61 CD34 F8 FLT4 HBA1 LYZ TNF
2 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 22)
# Name GO ID Score Top Affiliating Genes
1 positive regulation of cell proliferation GO:0008284 9.91 FLT4 MYC PROX1 VEGFA VEGFC
2 platelet activation GO:0030168 9.75 F8 PDPN VWF
3 lung development GO:0030324 9.73 PDPN PROX1 VEGFA
4 negative regulation of gene expression GO:0010629 9.73 CD34 MYC TNF VEGFA
5 response to wounding GO:0009611 9.67 LYVE1 MYC VWF
6 positive regulation of gene expression GO:0010628 9.65 CD34 GJC2 MYC TNF VEGFA
7 vascular endothelial growth factor receptor signaling pathway GO:0048010 9.63 FLT4 VEGFA VEGFC
8 positive regulation of neuroblast proliferation GO:0002052 9.61 VEGFA VEGFC
9 blood coagulation, intrinsic pathway GO:0007597 9.59 F8 VWF
10 oxygen transport GO:0015671 9.58 HBA1 MYC
11 positive regulation of epithelial cell proliferation GO:0050679 9.58 MYC VEGFA VEGFC
12 induction of positive chemotaxis GO:0050930 9.57 VEGFA VEGFC
13 lymph vessel development GO:0001945 9.55 FLT4 PROX1
14 negative regulation of glucose import GO:0046325 9.54 MYC TNF
15 positive regulation of cellular component movement GO:0051272 9.52 PDPN VEGFA
16 positive regulation of endothelial cell migration GO:0010595 9.5 FLT4 PROX1 VEGFA
17 positive regulation of protein kinase C signaling GO:0090037 9.48 FLT4 VEGFA
18 platelet degranulation GO:0002576 9.46 F8 VEGFA VEGFC VWF
19 positive regulation of mast cell chemotaxis GO:0060754 9.37 VEGFA VEGFC
20 positive regulation of endothelial cell proliferation GO:0001938 9.26 FLT4 PROX1 VEGFA VEGFC
21 lymphangiogenesis GO:0001946 8.8 FLT4 PDPN PROX1
22 multicellular organism development GO:0007275 10.08 FLT4 PDPN PROX1 TNF VEGFA VEGFC
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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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