MCID: LYM008
MIFTS: 40

Lymphangiosarcoma

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

Aliases & Classifications for Lymphangiosarcoma

MalaCards integrated aliases for Lymphangiosarcoma:

Name: Lymphangiosarcoma 12 44 15 17 70
Stewart Treves Syndrome 20 44
Skin Lymphangiosarcoma 12 70
Angiosarcoma Associated with Chronic Lymphedema 20
Lymphangiosarcoma of Stewart and Treves 12
Lymphangiosarcoma Following Mastectomy 20
Postmastectomy Extremity Angiosarcoma 20
Malignant Lymphangioendothelioma 12
Stewart-Treves Syndrome 12

Classifications:



External Ids:

Disease Ontology 12 DOID:2689
NCIt 50 C3205 C4490
SNOMED-CT 67 403986008 62497000
UMLS 70 C0024224 C0346082

Summaries for Lymphangiosarcoma

GARD : 20 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 hereditary lymphedema i and conventional angiosarcoma. An important gene associated with Lymphangiosarcoma is TNF (Tumor Necrosis Factor), and among its related pathways/superpathways is Angiogenesis (CST). The drugs Mesna and Doxorubicin have been mentioned in the context of this disorder. Affiliated tissues include lymphatic vessels, endothelial and breast, and related phenotypes are cardiovascular system and digestive/alimentary

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

Wikipedia : 73 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 191)
# Related Disease Score Top Affiliating Genes
1 hereditary lymphedema i 31.1 PROX1 PDPN FLT4
2 conventional angiosarcoma 30.6 PECAM1 CD34
3 histiocytoid hemangioma 30.5 PECAM1 CD34
4 congenital lymphedema 30.3 PDPN FLT4
5 cellulitis 30.3 TNF FLT4
6 keratitis, hereditary 29.9 TNF PECAM1 LYVE1
7 hemangiopericytoma, malignant 29.9 PECAM1 CD34
8 skin angiosarcoma 29.7 TIE1 SNRK FLT4
9 lymphangioma 29.3 PROX1 PECAM1 PDPN LYVE1 FLT4 CD34
10 lymphangitis 29.2 PROX1 PECAM1 PDPN LYVE1 FLT4
11 angiosarcoma 29.2 VWF TIE1 PROX1 PECAM1 PDPN FLT4
12 breast angiosarcoma 28.9 TIE1 SNRK PROX1 PECAM1 FLT4 CD34
13 hemangioma 28.2 VWF TIE1 PROX1 PECAM1 PDPN LYVE1
14 sarcoma 10.4
15 spindle cell sarcoma 10.4
16 radiation proctitis 10.3 TNF PECAM1
17 skin epithelioid hemangioma 10.3 PECAM1 CD34
18 aortic malignant tumor 10.3 PECAM1 CD34
19 prostate angiosarcoma 10.3 PECAM1 CD34
20 central nervous system angiosarcoma 10.3 PECAM1 CD34
21 intravascular angioleiomyoma 10.3 PECAM1 CD34
22 malignant ciliary body melanoma 10.3 PROX1 LYVE1
23 angiokeratoma of fordyce 10.3 PROX1 PECAM1
24 ciliary body cancer 10.3 PROX1 LYVE1
25 central nervous system hemangioma 10.3 PECAM1 CD34
26 heart fibrosarcoma 10.3 PECAM1 CD34
27 ovarian angiosarcoma 10.3 PECAM1 CD34
28 cervical cancer 10.3
29 erysipelas 10.3
30 gallbladder leiomyosarcoma 10.3 PECAM1 CD34
31 thyroid sarcoma 10.3 PECAM1 CD34
32 angiomyoma 10.3 PECAM1 CD34
33 retroperitoneal hemangiopericytoma 10.3 PECAM1 CD34
34 glomangiosarcoma 10.3 PECAM1 CD34
35 hemangioma of spleen 10.3 PECAM1 CD34
36 liposarcoma of bone 10.3 PECAM1 CD34
37 liver leiomyoma 10.3 PECAM1 CD34
38 benign perivascular tumor 10.3 PECAM1 CD34
39 perivascular tumor 10.3 PECAM1 CD34
40 erythema elevatum diutinum 10.3 PECAM1 CD34
41 sarcomatoid squamous cell skin carcinoma 10.3 PECAM1 CD34
42 heart sarcoma 10.3 PECAM1 CD34
43 bacillary angiomatosis 10.3 PECAM1 CD34
44 decubitus ulcer 10.3 TNF PECAM1
45 pulmonary artery leiomyosarcoma 10.2 PECAM1 CD34
46 subungual glomus tumor 10.2 PECAM1 CD34
47 vulvar leiomyoma 10.2 PECAM1 CD34
48 angiolipoma 10.2 PECAM1 CD34
49 intravascular fasciitis 10.2 PECAM1 CD34
50 angiokeratoma circumscriptum 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:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 10.02 FLT4 LYVE1 PDPN PECAM1 PROX1 SNRK
2 digestive/alimentary MP:0005381 9.87 FLT4 PDPN PECAM1 PROX1 TIE1 TNF
3 hematopoietic system MP:0005397 9.86 CD34 F8 LYVE1 PDPN PECAM1 PROX1
4 homeostasis/metabolism MP:0005376 9.85 CD34 F8 FLT4 PDPN PECAM1 PROX1
5 immune system MP:0005387 9.7 CD34 F8 FLT4 LYVE1 PDPN PECAM1
6 muscle MP:0005369 9.17 FLT4 PDPN PECAM1 PROX1 SNRK TIE1

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
Mesna Approved, Investigational Phase 2 3375-50-6 598
2
Doxorubicin Approved, Investigational Phase 2 23214-92-8 31703
3
Ifosfamide Approved Phase 2 3778-73-2 3690
4
Liposomal doxorubicin Phase 2 31703
5
Isophosphamide mustard Phase 2 100427

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 Active, not recruiting 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

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

19
Lymphatic Vessels

MalaCards organs/tissues related to Lymphangiosarcoma:

40
Endothelial, Breast, Skin, Bone, Lymph Node, Bone Marrow, Spinal Cord

Publications for Lymphangiosarcoma

Articles related to Lymphangiosarcoma:

(show top 50) (show all 468)
# Title Authors PMID Year
1
Stewart-Treves Syndrome as a Rare and Fatal Complication of Post-Traumatic Lymphedema on the Lower Extremity. 61
33747618 2021
2
Shoulder arthroplasty in patients with upper extremity lymphedema may result in transient or permanent lymphedema worsening. 61
33343716 2020
3
Stewart-Treves syndrome: Case report and literature review. 61
33088229 2020
4
A case of Stewart-Treves syndrome occurring in the abdominal wall successfully treated with eribulin: A case report. 61
32874579 2020
5
Stewart-Treves syndrome: a rapidly fatal complication of breast cancer treatment. 61
33002445 2020
6
Scalp lymphangiosarcoma: A distinct skin manifestation of edematous erythema on face and scalp without subcutaneous hemorrhage or preceding condition of lymphedema. 61
32677036 2020
7
Primary Lymphangiosarcoma of the Urinary Bladder in a Dog. 61
32958144 2020
8
Adipose-derived stem cell therapy shows promising results for secondary lymphedema. 61
32843917 2020
9
Targeted therapy for mTORC1-driven tumours through HDAC inhibition by exploiting innate vulnerability of mTORC1 hyper-activation. 61
32336756 2020
10
Pathologic and flow cytometric features of a case of canine ventral cervical lymphangiosarcoma. 61
32306890 2020
11
Effectiveness of pazopanib for local and lung metastases in Stewart-Treves syndrome. 61
32250262 2020
12
IL-23 Expression in Stewart-Treves Syndrome: Two Case Reports and Immunohistochemical Investigation. 61
32508617 2020
13
Secondary angiosarcoma: A fatal complication of chronic lymphedema. 61
31236970 2020
14
A case of Stewart-Treves Syndrome of the lower limbs: The usefulness of dermoscopy. 61
31407462 2019
15
Prevention of tumor progression in Stewart-Treves syndrome with a low dose of pazopanib. 61
30802322 2019
16
Secondary breast angiosarcoma: A multicentre retrospective survey by the national Italian association of Breast Surgeons (ANISC). 61
30877870 2019
17
Unsuspected Stewart-Treves syndrome clinically mimicked by apparent bullous erysipelas and a systematic review of dermatological presentations of the classical Stewart-Treves syndrome. 61
32721135 2019
18
Primary Lymphedema of the Upper Extremities: Clinical and Lymphoscintigraphic Features in 23 Patients. 61
30280960 2019
19
Clinicopathologic features of Stewart-Treves syndrome. 61
31933875 2019
20
Kaposi Sarcoma and Cutaneous Angiosarcoma: Guidelines for Diagnosis and Treatment. 61
30262126 2018
21
Cutaneous angiosarcoma of the lower leg. 61
30489569 2018
22
Cutaneous Angiosarcoma Postmastectomy (Stewart-Treves Syndrome). 61
30320298 2018
23
Guillain-Barré syndrome in a cancer patient treated with bevacizumab. 61
31149522 2018
24
Metastatic Angiosarcoma of Lower Extremity. 61
29854638 2018
25
[Angiosarcoma in primary lymphoedema: A rare complication]. 61
29530502 2018
26
Pretibial Located Stewart-Treves Syndrome: Uncommon Presentation in a Bulgarian Patient! 61
29731936 2018
27
Successful management of lymphangiosarcoma in a puppy using a tyrosine kinase inhibitor. 61
29606721 2018
28
Angiosarcoma complicating lower leg elephantiasis in a male patient: An unusual clinical complication, case report and literature review. 61
30397474 2018
29
A Supernumerary Nipple-Like Clinical Presentation of Lymphangioma Circumscriptum. 61
29796322 2018
30
Stewart-Treves syndrome: Magnetic resonance imaging data compared with pathological results from a single center. 61
29391898 2018
31
Lymphangiosarcoma of the hip arising in a congenital non-irradiated lymphangioma. 61
29068520 2017
32
Stewart-Treves Syndrome on the Lower Extremity Associated to Idiopathic Chronic Lymphedema Visualized on FDG PET/CT. 61
29035997 2017
33
Morphofunctional basis of the different types of angiogenesis and formation of postnatal angiogenesis-related secondary structures. 61
28762232 2017
34
Stewart-Treves syndrome in a spinal cord injury patient with MYC amplification. 61
29264380 2017
35
Pathology in Practice. Ventral abdominal lymphangiosarcoma. 61
28263114 2017
36
Cutaneous angiosarcoma: report of three different and typical cases admitted in a unique dermatology clinic. 61
28538886 2017
37
Stewart-Treves Syndrome Involving Chronic Lymphedema after Mastectomy of Breast Cancer. 61
28280645 2017
38
Lymphangiosarcoma of the jejunum in a 44-year-old man: report of a case and review of literature. 61
31966736 2017
39
MYC immunohistochemistry in angiosarcoma and atypical vascular lesions: practical considerations based on a single institutional experience. 61
27780597 2016
40
A case of Stewart-Treves syndrome with the longest lag: 44 years after radical mastectomy for breast cancer. 61
29896826 2016
41
An unusual presentation of Stewart-Treves syndrome on the lower extremity. 61
27766300 2016
42
The use of novel lymphatic endothelial cell-specific immunohistochemical markers to differentiate cutaneous angiosarcomas in dogs. 61
24593773 2016
43
Pancreatic metastasis of angiosarcoma (Stewart-Treves syndrome) diagnosed using endoscopic ultrasound-guided fine needle aspiration: A case report. 61
27537557 2016
44
Angiosarcoma after excisional surgery for chronic lymphedema. 61
27318054 2016
45
Modeling lymphangiosarcoma in mice. 61
27105012 2016
46
Lymphangiosarcoma in 12 dogs: a case series (1998-2013). 61
24612140 2016
47
Lymphangiosarcoma: Is Stewart-Treves Syndrome a Preventable Condition? 61
26584023 2016
48
Angiosarcoma on Lymphedema (Stewart-Treves Syndrome): A 12-Year Follow-up after Isolated Limb Perfusion, Limb Infusion, and Electrochemotherapy. 61
26916941 2016
49
Stewart Treves Syndrome in a Woman with Mastectomy. 61
27042566 2016
50
Stewart-Treves Syndrome: A Case Report and Review of the Literature. 61
27099606 2016

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 10.97 VWF PROX1 PECAM1 PDPN FLT4

GO Terms for Lymphangiosarcoma

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

# Name GO ID Score Top Affiliating Genes
1 integral component of plasma membrane GO:0005887 9.17 TNF TIE1 PECAM1 PDPN LYVE1 FLT4

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

(show all 12)
# Name GO ID Score Top Affiliating Genes
1 multicellular organism development GO:0007275 9.88 TNF TIE1 PROX1 PDPN FLT4
2 positive regulation of protein phosphorylation GO:0001934 9.69 TNF PECAM1 FLT4
3 leukocyte migration GO:0050900 9.67 TNF PECAM1 CD34
4 cell-cell adhesion GO:0098609 9.63 PECAM1 PDPN CD34
5 platelet degranulation GO:0002576 9.58 VWF PECAM1 F8
6 platelet activation GO:0030168 9.5 VWF PDPN F8
7 blood coagulation, intrinsic pathway GO:0007597 9.43 VWF F8
8 lymph vessel development GO:0001945 9.37 PROX1 FLT4
9 lymphatic endothelial cell differentiation GO:0060836 9.26 TIE1 PROX1
10 glomerular endothelium development GO:0072011 9.16 PECAM1 CD34
11 lymphatic endothelial cell fate commitment GO:0060838 8.96 PROX1 PDPN
12 lymphangiogenesis GO:0001946 8.8 PROX1 PDPN FLT4

Sources for Lymphangiosarcoma

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
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