MCID: LYM008
MIFTS: 41

Lymphangiosarcoma

Categories: Cancer diseases, Immune diseases, Rare diseases, Skin diseases
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Aliases & Classifications for Lymphangiosarcoma

MalaCards integrated aliases for Lymphangiosarcoma:

Name: Lymphangiosarcoma 11 43 14 16 71 75
Stewart Treves Syndrome 19 43
Skin Lymphangiosarcoma 11 71
Lymphangiosarcoma of Stewart and Treves 11
Malignant Lymphangioendothelioma 11
Stewart-Treves Syndrome 11

Classifications:



External Ids:

Disease Ontology 11 DOID:2689
NCIt 49 C3205 C4490
SNOMED-CT 68 403986008 62497000
UMLS 71 C0024224 C0346082

Summaries for Lymphangiosarcoma

Disease Ontology: 11 A lymphatic system cancer that has material basis in endothelial cells located in lymphatic vessels.

MalaCards based summary: Lymphangiosarcoma, also known as stewart treves syndrome, is related to hereditary lymphedema i and conventional angiosarcoma. An important gene associated with Lymphangiosarcoma is TNF (Tumor Necrosis Factor), and among its related pathways/superpathways are Burn wound healing and Angiogenesis (CST). The drugs Coenzyme M and Ifosfamide have been mentioned in the context of this disorder. Affiliated tissues include lymphatic vessels, endothelial and skin, and related phenotypes are homeostasis/metabolism and muscle

Wikipedia: 75 Lymphangiosarcoma is a rare cancer 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 253)
# Related Disease Score Top Affiliating Genes
1 hereditary lymphedema i 30.8 PROX1 PDPN LYVE1 FLT4
2 conventional angiosarcoma 30.6 PECAM1 CD34
3 histiocytoid hemangioma 30.5 PECAM1 CD34
4 primary lymphedema 30.2 TIE1 FLT4
5 congenital lymphedema 30.2 PDPN FLT4
6 sickle cell anemia 30.2 VWF TNF CD34
7 hemangioendothelioma 30.0 FLT4 CD34
8 hemangiopericytoma, malignant 30.0 PECAM1 CD34
9 breast angiosarcoma 29.9 TIE1 PECAM1 FLT4 CD34
10 epithelioid hemangioendothelioma 29.8 PECAM1 FLT4 CD34
11 cavernous hemangioma 29.7 VWF VEGFA PECAM1 CD34
12 lymphangitis 29.7 PDPN LYVE1 FLT4
13 hereditary lymphedema 29.7 PROX1 PDPN LYVE1 FLT4
14 keratitis, hereditary 29.5 VEGFA TNF PECAM1 LYVE1
15 cystic lymphangioma 29.2 PROX1 PECAM1 PDPN FLT4 CD34
16 deficiency anemia 29.2 VWF VEGFA TNF CD34
17 angiosarcoma 29.1 VWF VEGFA TIE1 PECAM1 PDPN FLT4
18 kaposi sarcoma 29.1 VEGFA PECAM1 PDPN LYVE1 FLT4 CD34
19 vascular cancer 28.8 VWF VEGFA PTPRB PECAM1 FLT4 CD34
20 lymphangioma 28.7 VEGFA TNF PROX1 PECAM1 PDPN LYVE1
21 capillary lymphangioma 28.6 VWF PROX1 PECAM1 PDPN LYVE1 FLT4
22 hemangioma 27.9 VWF VEGFA TIE1 PROX1 PECAM1 PDPN
23 skin angiosarcoma 11.0
24 factor viii deficiency 10.4
25 cervical cancer 10.3
26 erysipelas 10.3
27 filarial elephantiasis 10.3
28 angiokeratoma of fordyce 10.3 PROX1 PECAM1
29 skin epithelioid hemangioma 10.3 PECAM1 CD34
30 aorta angiosarcoma 10.2 PECAM1 CD34
31 central nervous system angiosarcoma 10.2 PECAM1 CD34
32 intravascular angioleiomyoma 10.2 PECAM1 CD34
33 malignant ciliary body melanoma 10.2 PROX1 LYVE1
34 ciliary body cancer 10.2 PROX1 LYVE1
35 heart fibrosarcoma 10.2 PECAM1 CD34
36 thyroid angiosarcoma 10.2 PECAM1 CD34
37 glomangioma 10.2 PECAM1 CD34
38 glomangiomatosis 10.2 PECAM1 CD34
39 gallbladder leiomyosarcoma 10.2 PECAM1 CD34
40 venous hemangioma 10.2 PECAM1 CD34
41 kidney hemangiopericytoma 10.2 PECAM1 CD34
42 glomangiomyoma 10.2 PECAM1 CD34
43 angiomyoma 10.2 PECAM1 CD34
44 glomangiosarcoma 10.2 PECAM1 CD34
45 breast hemangioma 10.2 PECAM1 CD34
46 thyroid sarcoma 10.2 PECAM1 CD34
47 liposarcoma of bone 10.2 PECAM1 CD34
48 hemangioma of spleen 10.2 PECAM1 CD34
49 meninges hemangiopericytoma 10.2 PECAM1 CD34
50 cutaneous leiomyosarcoma 10.2 PECAM1 CD34

Graphical network of the top 20 diseases related to Lymphangiosarcoma:



Diseases related to Lymphangiosarcoma

Symptoms & Phenotypes for Lymphangiosarcoma

MGI Mouse Phenotypes related to Lymphangiosarcoma:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 homeostasis/metabolism MP:0005376 10.1 CD34 FLT4 PDPN PECAM1 PROX1 PTPRB
2 muscle MP:0005369 10.03 FLT4 PDPN PECAM1 PROX1 PTPRB TIE1
3 digestive/alimentary MP:0005381 9.97 FLT4 PDPN PECAM1 PROX1 TIE1 TNF
4 cardiovascular system MP:0005385 9.96 FLT4 LYVE1 PDPN PECAM1 PROX1 PTPRB
5 cellular MP:0005384 9.92 CD34 LYVE1 PECAM1 PROX1 PTPRB TNF
6 immune system MP:0005387 9.85 CD34 FLT4 LYVE1 PDPN PECAM1 PROX1
7 respiratory system MP:0005388 9.43 PDPN PECAM1 PROX1 TIE1 TNF VEGFA
8 integument MP:0010771 9.17 CD34 FLT4 PDPN PTPRB TIE1 TNF

Drugs & Therapeutics for Lymphangiosarcoma

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


# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Coenzyme M Approved, Investigational Phase 2 3375-50-6 598 23662354
2
Ifosfamide Approved Phase 2 3778-73-2 3690
3
Doxorubicin Approved, Investigational Phase 2 23214-92-8 31703
4
Palifosfamide Investigational Phase 2 31645-39-3 100427
5 Liposomal doxorubicin Phase 2

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Hypofractionated 5x5 Gy Radiotherapy With Sequential Doxorubicin and Ifosfamide-based Chemotherapy in Marginally Resectable Soft Tissue Sarcomas of Extremities or Trunk Wall Unknown status NCT03651375 Phase 2 Sequential chemotherapy - 3 courses of AI

Search NIH Clinical Center for Lymphangiosarcoma

Cochrane evidence based reviews: lymphangiosarcoma

Genetic Tests for Lymphangiosarcoma

Anatomical Context for Lymphangiosarcoma

Organs/tissues related to Lymphangiosarcoma:

FMA: Lymphatic Vessels
MalaCards : Endothelial, Skin, Breast, Bone Marrow, Lymph Node, Spinal Cord, Bone

Publications for Lymphangiosarcoma

Articles related to Lymphangiosarcoma:

(show top 50) (show all 488)
# Title Authors PMID Year
1
Dusky patches in chronic leg lymphedema - An uncommon presentation of Stewart-Treves syndrome. 62
35775097 2022
2
Case for diagnosis. Stewart-Treves syndrome after mastectomy. 62
35811194 2022
3
Prospective evaluation of lymphatic embolization as part of the treatment in dogs with presumptive idiopathic chylothorax. 62
35692101 2022
4
A rare and highly malignant Stwart-Treves syndrome case after breast cancer surgery: a case report. 62
35935569 2022
5
Unusual coexistence of Stewart-Treves syndrome and sickle cell anaemia: a case of dual pathology. 62
35793854 2022
6
Chronic lymphoedema: a nidus for squamous cell carcinoma. 62
35537771 2022
7
Remarkable response to radiation therapy with concurrent chemotherapy in Stewart-Treves syndrome. 62
35900571 2022
8
Stewart-Treves Syndrome in the Lower Limb after Arthroplasty- A Case Report. 62
35287427 2022
9
Stewart-Treves syndrome in an older woman successfully treated by metronomic chemotherapy: case report and literature survey. 62
34845161 2022
10
Stewart-Treves syndrome and other cutaneous malignancies in the context of chronic lymphedema: a systematic review. 62
34196958 2022
11
Rare Case of Cystic Lymphangioma Transforming Into Lymphangiosarcoma: A Case Report. 62
35251984 2022
12
A case of Stewart-Treves Syndrome in a prostatectomized patient. 62
33890737 2021
13
Stewart-Treves Syndrome: A Rare But Aggressive Complication of Breast Cancer-Related Lymphedema. 62
34651118 2021
14
Stewart-Treves Syndrome in Obesity-Associated Chronic Lymphedema: A Case Report. 62
34691335 2021
15
Stewart-Treves syndrome associated with disuse edema in amyotrophic lateral sclerosis. 62
34058020 2021
16
Stewart-Treves Syndrome. 62
33909015 2021
17
Stewart-Treves Syndrome as a Rare and Fatal Complication of Post-Traumatic Lymphedema on the Lower Extremity. 62
33747618 2021
18
Stewart-Treves syndrome: a diagnosis to keep in mind. 62
33865293 2021
19
Case Report: Cutaneous Pleomorphic Lymphangiosarcoma in a Dog Exhibiting Features of Human Composite Hemangioendothelioma. 62
34746271 2021
20
[Stewart-Treves syndrome]. 62
34160946 2021
21
Shoulder arthroplasty in patients with upper extremity lymphedema may result in transient or permanent lymphedema worsening. 62
33343716 2020
22
Stewart-Treves syndrome: Case report and literature review. 62
33088229 2020
23
A case of Stewart-Treves syndrome occurring in the abdominal wall successfully treated with eribulin: A case report. 62
32874579 2020
24
Stewart-Treves syndrome: a rapidly fatal complication of breast cancer treatment. 62
33002445 2020
25
Scalp lymphangiosarcoma: A distinct skin manifestation of edematous erythema on face and scalp without subcutaneous hemorrhage or preceding condition of lymphedema. 62
32677036 2020
26
Primary Lymphangiosarcoma of the Urinary Bladder in a Dog. 62
32958144 2020
27
Adipose-derived stem cell therapy shows promising results for secondary lymphedema. 62
32843917 2020
28
Targeted therapy for mTORC1-driven tumours through HDAC inhibition by exploiting innate vulnerability of mTORC1 hyper-activation. 62
32336756 2020
29
Pathologic and flow cytometric features of a case of canine ventral cervical lymphangiosarcoma. 62
32306890 2020
30
Effectiveness of pazopanib for local and lung metastases in Stewart-Treves syndrome. 62
32250262 2020
31
IL-23 Expression in Stewart-Treves Syndrome: Two Case Reports and Immunohistochemical Investigation. 62
32508617 2020
32
Secondary angiosarcoma: A fatal complication of chronic lymphedema. 62
31236970 2020
33
A case of Stewart-Treves Syndrome of the lower limbs: The usefulness of dermoscopy. 62
31407462 2019
34
Prevention of tumor progression in Stewart-Treves syndrome with a low dose of pazopanib. 62
30802322 2019
35
Secondary breast angiosarcoma: A multicentre retrospective survey by the national Italian association of Breast Surgeons (ANISC). 62
30877870 2019
36
Unsuspected Stewart-Treves syndrome clinically mimicked by apparent bullous erysipelas and a systematic review of dermatological presentations of the classical Stewart-Treves syndrome. 62
32721135 2019
37
Primary Lymphedema of the Upper Extremities: Clinical and Lymphoscintigraphic Features in 23 Patients. 62
30280960 2019
38
Clinicopathologic features of Stewart-Treves syndrome. 62
31933875 2019
39
Kaposi Sarcoma and Cutaneous Angiosarcoma: Guidelines for Diagnosis and Treatment. 62
30262126 2018
40
Cutaneous Angiosarcoma Postmastectomy (Stewart-Treves Syndrome). 62
30320298 2018
41
Cutaneous angiosarcoma of the lower leg. 62
30489569 2018
42
Guillain-Barré syndrome in a cancer patient treated with bevacizumab. 62
31149522 2018
43
Metastatic Angiosarcoma of Lower Extremity. 62
29854638 2018
44
[Angiosarcoma in primary lymphoedema: A rare complication]. 62
29530502 2018
45
Pretibial Located Stewart-Treves Syndrome: Uncommon Presentation in a Bulgarian Patient! 62
29731936 2018
46
Successful management of lymphangiosarcoma in a puppy using a tyrosine kinase inhibitor. 62
29606721 2018
47
Stewart-Treves syndrome: Magnetic resonance imaging data compared with pathological results from a single center. 62
29391898 2018
48
Angiosarcoma complicating lower leg elephantiasis in a male patient: An unusual clinical complication, case report and literature review. 62
30397474 2018
49
A Supernumerary Nipple-Like Clinical Presentation of Lymphangioma Circumscriptum. 62
29796322 2018
50
Morphofunctional basis of the different types of angiogenesis and formation of postnatal angiogenesis-related secondary structures. 62
28762232 2017

Variations for Lymphangiosarcoma

Expression for Lymphangiosarcoma

Search GEO for disease gene expression data for Lymphangiosarcoma.

Pathways for Lymphangiosarcoma

Pathways related to Lymphangiosarcoma according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1 11.01 VEGFA TNF PECAM1
2 10.97 VWF PROX1 PECAM1 PDPN FLT4
3 10.85 VEGFA TNF
4 10.77 VEGFA TNF
5 10.69 VEGFA TNF
6
Show member pathways
10.36 VEGFA FLT4

GO Terms for Lymphangiosarcoma

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

# Name GO ID Score Top Affiliating Genes
1 plasma membrane GO:0005886 9.7 TNF TIE1 PTPRB PECAM1 PDPN LYVE1
2 plasma membrane GO:0005887 9.7 TNF TIE1 PTPRB PECAM1 PDPN LYVE1

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

(show all 15)
# Name GO ID Score Top Affiliating Genes
1 angiogenesis GO:0001525 10.1 VEGFA TIE1 PTPRB FLT4
2 positive regulation of cell migration GO:0030335 10.06 VEGFA PECAM1 PDPN FLT4
3 lung development GO:0030324 10 VEGFA PROX1 PDPN
4 positive regulation of protein phosphorylation GO:0001934 9.97 VEGFA TNF PECAM1 FLT4
5 positive regulation of endothelial cell proliferation GO:0001938 9.91 FLT4 PROX1 VEGFA
6 vascular endothelial growth factor signaling pathway GO:0038084 9.84 VEGFA FLT4
7 positive regulation of endothelial cell migration GO:0010595 9.8 VEGFA PROX1 FLT4
8 positive regulation of protein kinase C signaling GO:0090037 9.78 VEGFA FLT4
9 positive regulation of DNA biosynthetic process GO:2000573 9.72 VEGFA TNF
10 vascular wound healing GO:0061042 9.71 VEGFA CD34
11 lymph vessel development GO:0001945 9.69 PROX1 FLT4
12 lymphatic endothelial cell differentiation GO:0060836 9.56 TIE1 PROX1
13 glomerular endothelium development GO:0072011 9.46 PECAM1 CD34
14 lymphatic endothelial cell fate commitment GO:0060838 9.26 PROX1 PDPN
15 lymphangiogenesis GO:0001946 9.1 PROX1 PDPN FLT4

Sources for Lymphangiosarcoma

2 CDC
6 CNVD
8 Cosmic
9 dbSNP
10 DGIdb
16 EFO
17 ExPASy
18 FMA
19 GARD
27 GO
28 GTR
29 HMDB
30 HPO
31 ICD10
32 ICD10 via Orphanet
33 ICD11
34 ICD9CM
35 IUPHAR
36 LifeMap
38 LOVD
40 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
52 NINDS
53 Novoseek
55 ODiseA
56 OMIM via Orphanet
57 OMIM® (Updated 08-Dec-2022)
61 PubChem
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 Tocris
71 UMLS
72 UMLS via Orphanet
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