MCID: LYM008
MIFTS: 51

Lymphangiosarcoma

Categories: Rare diseases, Immune diseases, Skin diseases, Cancer diseases

Aliases & Classifications for Lymphangiosarcoma

MalaCards integrated aliases for Lymphangiosarcoma:

Name: Lymphangiosarcoma 12 41 14 69
Stewart Treves Syndrome 49 41
Skin Lymphangiosarcoma 12 69
Angiosarcoma Associated with Chronic Lymphedema 49
Lymphangiosarcoma of Stewart and Treves 12
Lymphangiosarcoma Following Mastectomy 49
Postmastectomy Extremity Angiosarcoma 49
Malignant Lymphangioendothelioma 12
Stewart-Treves Syndrome 12

Classifications:



External Ids:

Disease Ontology 12 DOID:2689
NCIt 46 C3205 C4490

Summaries for Lymphangiosarcoma

NIH Rare Diseases : 49 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. Last updated: 5/23/2017

MalaCards based summary : Lymphangiosarcoma, also known as stewart treves syndrome, is related to lymphedema and hereditary lymphedema. An important gene associated with Lymphangiosarcoma is TNF (Tumor Necrosis Factor), 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, endothelial and skin, 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 : 72 Lymphangiosarcoma is a rare malignant tumor which occurs in long-standing cases of primary or secondary... more...

Related Diseases for Lymphangiosarcoma

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

(show top 50) (show all 112)
# Related Disease Score Top Affiliating Genes
1 lymphedema 31.1 FLT4 GJC2 PROX1 VEGFC
2 hereditary lymphedema 30.5 FLT4 GJC2
3 congenital lymphedema 29.8 FLT4 PDPN VEGFC
4 angiosarcoma 29.7 FLT4 MYC PROX1 VEGFA VWF
5 lymphangioma 29.3 FLT4 PDPN PROX1 VEGFA VEGFC
6 angiokeratoma of fordyce 10.6 HBA1 PROX1
7 lymphatic malformations 10.5 FLT4 PROX1
8 gorham's disease 10.5 FLT4 PROX1
9 mild hemophilia a 10.4 F8 VWF
10 critical limb ischemia 10.4 TNF VEGFA
11 fainting 10.4 F8 VWF
12 hemifacial spasm 10.4 FLT4 VEGFA
13 pulmonary artery leiomyosarcoma 10.4 HBA1 SMUG1
14 von willebrand disease, type 2 10.4 F8 VWF
15 hemangioma of spleen 10.4 FLT4 SMUG1
16 cellulitis 10.4 FLT4 GJC2 TNF
17 acquired von willebrand syndrome 10.4 F8 VWF
18 von willebrand disease, type 3 10.4 F8 VWF
19 hennekam syndrome 10.4 FLT4 PROX1 VEGFC
20 cerebral arteritis 10.4 F8 VWF
21 brain sarcoma 10.4 HBA1 SMUG1
22 intravascular fasciitis 10.4 CD34 HBA1
23 thyroid angiosarcoma 10.4 CD34 HBA1
24 von willebrand disease, type 1 10.4 F8 VWF
25 heart cancer 10.3 HBA1 SMUG1
26 neurogenic arthropathy 10.3 CD34 HBA1
27 lymphatic system disease 10.3 FLT4 GJC2 VEGFC
28 skin hemangioma 10.3 HBA1 PROX1 VEGFA
29 epithelioid neurofibroma 10.3 CD34 DSP
30 thyroid sarcoma 10.3 CD34 HBA1
31 capillary hemangioma 10.3 FLT4 HBA1 VEGFA
32 gastrointestinal neuroendocrine tumor 10.3 SMUG1 VEGFA
33 hemangioma of liver 10.3 CD34 HBA1
34 leukodystrophy, hypomyelinating, 4 10.3 GJC2 MYC
35 breast carcinoma in situ 10.3 FLT4 VEGFA VEGFC
36 al amyloidosis 10.2 LYZ VWF
37 pericardium cancer 10.2 F8 HBA1 SMUG1
38 hepatopulmonary syndrome 10.2 TNF VEGFA VWF
39 limb ischemia 10.2 TNF VEGFA VWF
40 tibial adamantinoma 10.2 CD34 SMUG1
41 breast angiosarcoma 10.2 CD34 HBA1
42 portal hypertension 10.2 TNF VEGFA VWF
43 breast leiomyosarcoma 10.2 CD34 SMUG1
44 conventional angiosarcoma 10.2 CD34 HBA1
45 infiltrating angiolipoma 10.2 VEGFA VEGFC VWF
46 arteries, anomalies of 10.2 TNF VEGFA VWF
47 sternum cancer 10.2 CD34 SMUG1
48 anomalous left coronary artery from the pulmonary artery 10.2 SMUG1 VWF
49 cardiac sarcoidosis 10.2 SMUG1 TNF
50 spleen cancer 10.2 CD34 F8 HBA1

Graphical network of the top 20 diseases related to Lymphangiosarcoma:



Diseases related to Lymphangiosarcoma

Symptoms & Phenotypes for Lymphangiosarcoma

MGI Mouse Phenotypes related to Lymphangiosarcoma:

43
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 10.22 PDPN LYVE1 LYZ DSP MYC FLT4
2 cellular MP:0005384 10.2 GJC2 LYVE1 CD34 LYZ DSP MYC
3 homeostasis/metabolism MP:0005376 10.15 PDPN CD34 LYZ F8 MYC FLT4
4 hematopoietic system MP:0005397 10.14 GJC2 PDPN LYVE1 CD34 LYZ F8
5 immune system MP:0005387 10.13 LYVE1 CD34 LYZ MYC F8 FLT4
6 digestive/alimentary MP:0005381 10.09 LYZ DSP MYC FLT4 PDPN VEGFA
7 mortality/aging MP:0010768 9.97 GJC2 PDPN LYZ DSP F8 MYC
8 integument MP:0010771 9.86 CD34 LYZ DSP MYC FLT4 PDPN
9 muscle MP:0005369 9.5 DSP MYC FLT4 PDPN VEGFA PROX1
10 neoplasm MP:0002006 9.1 CD34 LYZ MYC VEGFA SMUG1 TNF

Drugs & Therapeutics for Lymphangiosarcoma

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

Genetic Tests for Lymphangiosarcoma

Anatomical Context for Lymphangiosarcoma

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

18
Lymphatic Vessels

MalaCards organs/tissues related to Lymphangiosarcoma:

38
Endothelial, Skin, Lymph Node, Cervix, Testes, Bone, Lung

Publications for Lymphangiosarcoma

Articles related to Lymphangiosarcoma:

(show top 50) (show all 165)
# Title Authors Year
1
Lymphangiosarcoma of the hip arising in a congenital non-irradiated lymphangioma. ( 29068520 )
2017
2
Pathology in Practice. Ventral abdominal lymphangiosarcoma. ( 28263114 )
2017
3
Modeling lymphangiosarcoma in mice. ( 27105012 )
2016
4
Lymphangiosarcoma: Is Stewart-Treves Syndrome a Preventable Condition? ( 26584023 )
2016
5
Lymphangiosarcoma with systemic metastases in a Japanese domestic cat. ( 25482607 )
2015
6
Stewart-Treves Phenomenon: Lymphangiosarcoma Arising in Lymphedematous Tissue Is Not a Syndrome. ( 25658055 )
2015
7
Presumed primary ocular lymphangiosarcoma with metastasis in a miniature horse. ( 25581559 )
2015
8
Lymphangiosarcoma with bone formation of the auricle in a dog. ( 25716121 )
2015
9
Constitutive Activation of mTORC1 in Endothelial Cells Leads to the Development and Progression of Lymphangiosarcoma through VEGF Autocrine Signaling. ( 26777415 )
2015
10
Mailgnant degeneration to lymphangiosarcoma of a chronic lymphedema in the lower left extremity. ( 25304756 )
2014
11
Lymphangiosarcoma in 12 dogs: a case series (1998-2013). ( 24612140 )
2014
12
Lymphangiosarcoma with strong positivity of D2-40 and LYVE-1 presenting different clinical features from angiosarcoma. ( 24985547 )
2014
13
Lymphangiosarcoma of the scalp. ( 24636440 )
2014
14
Lymphangiosarcoma of the scalp. ( 24342442 )
2014
15
An unusual case of skin metastasis from a breast cancer mimicking a lymphangiosarcoma. ( 24977428 )
2014
16
Complex karyotype in a case of cutaneous lymphangiosarcoma associated with chronic lymphedema of the lower limb. ( 24947410 )
2014
17
Lymphangiosarcoma of mons pubis following radiotherapy for carcinoma cervix. ( 25404831 )
2014
18
A novel approach to treatment of lymphangiosarcoma in a boxer dog. ( 23560802 )
2013
19
Chronic lymphedema of filarial origin: a very rare etiology of cutaneous lymphangiosarcoma. ( 23372219 )
2013
20
Cutaneous lymphangiosarcoma following chronic lymphedema of filarial origin. ( 23372218 )
2013
21
Lymphangiosarcoma arising after 33 years within a background of chronic filariasis: a case report with review of literature. ( 22211335 )
2012
22
Plasma-cell rich lymphangiosarcoma: an under-recognized variant and potential diagnostic pitfall. ( 22188566 )
2012
23
Lymphangiosarcoma of the thorax and thoracic vertebrae in a 16-year-old girl. ( 22915659 )
2012
24
Establishment and characterization of a novel lymphangiosarcoma cell line (MO-LAS) compared with the hemangiosarcoma cell line (ISO-HAS). ( 23342252 )
2012
25
Use of surgery and mitoxantrone chemotherapy in a dog with disseminated lymphangiosarcoma. ( 23216040 )
2012
26
Lymphangiosarcoma and filariasis. ( 22575002 )
2012
27
Elevated coagulation factor VIII plasma activity in a patient with lymphangiosarcoma. ( 23154736 )
2012
28
Lymphangiosarcoma of the vocal cord: a rare entity defined by a D2-40 immunohistochemical and ultrastructural study. ( 20975065 )
2011
29
Locally invasive lymphangiosarcoma in a young domestic shorthair. ( 21880529 )
2011
30
Can lymphangiosarcoma be resurrected? A clinicopathological and immunohistochemical study of lymphatic differentiation in 49 angiosarcomas. ( 20459536 )
2010
31
FDG-PET/CT imaging in a patient with lymphangiosarcoma. ( 20479592 )
2010
32
Lymphangiosarcoma complicating extensive congenital mixed vascular malformations. ( 20863270 )
2010
33
Fatal lymphangiosarcoma revealed by lymphoedema of the neck. ( 19479140 )
2009
34
Lymphangiosarcoma of the arm presenting with lymphedema in a woman 16 years after mastectomy: a case report. ( 19918554 )
2009
35
Lymphangiosarcoma in the nictitating membrane of a horse. ( 18182523 )
2008
36
Paresis in an Asian small clawed otter (Aonyx cinereus) associated with vertebral and ischial osteolysis caused by a malignant lymphangiosarcoma. ( 18634215 )
2008
37
Lymphangiosarcoma in two cats. ( 17307008 )
2007
38
Lymphangiosarcoma presenting as asymptomatic swelling of the cheek. ( 17317502 )
2007
39
Lymphangiosarcoma in a cat. ( 17706243 )
2007
40
Case 3. Upper limb lymphangiosarcoma following breast cancer therapy. ( 16549844 )
2006
41
Lymphangiosarcoma of dogs: a review. ( 16300179 )
2005
42
Lymphangiosarcoma in a 3.5-year-old Bullmastiff bitch with vaginal prolapse, primary lymph node fibrosis and other congenital defects. ( 16300186 )
2005
43
Lymphangiosarcoma associated with primary lymphedema in a Bouvier des Flandres. ( 14765744 )
2004
44
Lymphangiosarcoma in a dog treated with surgery and chemotherapy. ( 15031550 )
2004
45
Feline lymphangiosarcoma--definitive identification using a lymphatic vascular marker. ( 14989700 )
2004
46
Cutaneous lymphangiosarcoma in a young dog: clinical, anatomopathological and lectinhistochemical description. ( 15109239 )
2003
47
Isolated limb perfusion with tumor necrosis factor and melphalan for nonresectable sSewart-Treves lymphangiosarcoma. ( 12464594 )
2002
48
Metastatic lymphangiosarcoma in a horse. ( 12009065 )
2002
49
Chylothorax in a dog with pulmonary lymphangiosarcoma. ( 11204481 )
2001
50
Lymphangiosarcoma (Stewart-Treves syndrome) in postmastectomy patients. ( 11119680 )
2000

Variations for Lymphangiosarcoma

Expression for Lymphangiosarcoma

Search GEO for disease gene expression data for Lymphangiosarcoma.

Pathways for Lymphangiosarcoma

GO Terms for Lymphangiosarcoma

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 21)
# 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 9.76 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 vascular endothelial growth factor receptor signaling pathway GO:0048010 9.65 FLT4 VEGFA VEGFC
7 positive regulation of gene expression GO:0010628 9.65 CD34 GJC2 MYC TNF VEGFA
8 positive regulation of neuroblast proliferation GO:0002052 9.6 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 negative regulation of glucose import GO:0046325 9.55 MYC TNF
14 lymph vessel development GO:0001945 9.54 FLT4 PROX1
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 9.46 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

Sources for Lymphangiosarcoma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
27 GO
28 GTR
29 HGMD
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 MedGen
41 MeSH
42 MESH via Orphanet
43 MGI
45 NCI
46 NCIt
47 NDF-RT
50 NINDS
51 Novoseek
53 OMIM
54 OMIM via Orphanet
58 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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