MCID: CPL002
MIFTS: 38

Capillary Lymphangioma

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

Aliases & Classifications for Capillary Lymphangioma

MalaCards integrated aliases for Capillary Lymphangioma:

Name: Capillary Lymphangioma 12 52 58 15 71
Microcystic Infiltrating Lymphatic Malformation 52 58
Cutaneous Lymphangioma Circumscriptum 52 58
Microcystic Lymphatic Malformation 52 58
Superficial Lymphatic Malformation 52 58
Capillary Lymphatic Malformation 52 58
Microcystic Lymphangioma 52 58
Superficial Lymphangioma 52 58
Cutaneous Lymphangioma 12
Lymphangioma of Skin 71

Characteristics:

Orphanet epidemiological data:

58
microcystic lymphatic malformation
Inheritance: Not applicable; Age of onset: Infancy,Neonatal;

Classifications:

Orphanet: 58  
Rare circulatory system diseases
Developmental anomalies during embryogenesis


External Ids:

Disease Ontology 12 DOID:2286
NCIt 49 C27509
ICD10 via Orphanet 33 D18.1
Orphanet 58 ORPHA79490
UMLS 71 C0334543 C1333176

Summaries for Capillary Lymphangioma

NIH Rare Diseases : 52 Microcystic lymphatic malformation is one subtype of lymphatic malformation (LM), a congenital malformation of the lymphatic vessels in soft tissues , including the skin. LM is classified into the macrocystic type, cysts larger than 2 cm with clear margins (previously known as cystic hygromas), and the microcystic type, consisting of cysts smaller than 2 cm, that appear diffuse, and grow without clear borders (previously known as lymphangioma circumscriptum). When the two types concur it is called the combined type. Microcystic lesions are commonly found inside the mouth, throat, and in the tongue, parotid gland and submandibular gland . Symptoms include deformity, and problems with breathing and feeding. The exact cause is unknown but is likely related to a malformation of the lymphatic system at six to ten weeks of gestation, when some lymphatic tissue fails to communicate with the lymphatic and venous system. Treatment depends upon the clinical presentation, the size of the lesion, the location, and resulting complications. Superficial mucosal microcystic lesions may be treated with laser therapy .

MalaCards based summary : Capillary Lymphangioma, also known as microcystic infiltrating lymphatic malformation, is related to congenital lymphedema and hemangioendothelioma. An important gene associated with Capillary Lymphangioma is PECAM1 (Platelet And Endothelial Cell Adhesion Molecule 1), and among its related pathways/superpathways are Angiogenesis (CST) and Hypothesized Pathways in Pathogenesis of Cardiovascular Disease. The drugs Sirolimus and Everolimus have been mentioned in the context of this disorder. Affiliated tissues include skin, tongue and heart, and related phenotypes are cardiovascular system and hematopoietic system

Related Diseases for Capillary Lymphangioma

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

(show top 50) (show all 175)
# Related Disease Score Top Affiliating Genes
1 congenital lymphedema 30.6 PDPN FLT4
2 hemangioendothelioma 30.5 FLT4 CD34
3 cystic lymphangioma 30.2 PROX1 PECAM1 CD34
4 hobnail hemangioma 29.8 PECAM1 PDPN FLT4 CD34
5 lymphangioma 29.5 PROX1 PECAM1 PDPN LYVE1 FLT4 CD34
6 kaposiform hemangioendothelioma 29.4 VWF PROX1 PECAM1 FLT4 CD34
7 vascular cancer 28.6 VWF PECAM1 PDPN FLT4 ENG CD34
8 lymphangiosarcoma 28.6 VWF PROX1 PECAM1 PDPN LYVE1 FLT4
9 thrombocytopenia 28.4 VWF PECAM1 FLNA CRP CD34
10 hemangioma 28.2 VWF PROX1 PECAM1 PDPN LYVE1 FLT4
11 venous hemangioma 10.5 PROX1 PECAM1
12 skin epithelioid hemangioma 10.5 PECAM1 CD34
13 prostate angiosarcoma 10.4 PECAM1 CD34
14 central nervous system angiosarcoma 10.4 PECAM1 CD34
15 intravascular angioleiomyoma 10.4 PECAM1 CD34
16 heart fibrosarcoma 10.4 PECAM1 CD34
17 central nervous system hemangioma 10.4 PECAM1 CD34
18 ovarian angiosarcoma 10.4 PECAM1 CD34
19 conventional angiosarcoma 10.4 PECAM1 CD34
20 angiomyoma 10.4 PECAM1 CD34
21 aortic malignant tumor 10.4 PECAM1 CD34
22 gallbladder leiomyosarcoma 10.4 PECAM1 CD34
23 hemangioma of spleen 10.4 PECAM1 CD34
24 chylothorax, congenital 10.4 PROX1 FLT4
25 ovary sarcoma 10.4 PECAM1 CD34
26 heart leiomyosarcoma 10.4 PECAM1 CD34
27 colonic lymphangioma 10.4 PECAM1 CD34
28 benign perivascular tumor 10.4 PECAM1 CD34
29 liposarcoma of bone 10.4 PECAM1 CD34
30 hereditary lymphedema ii 10.4 PROX1 FLT4
31 intravascular fasciitis 10.4 PECAM1 CD34
32 retroperitoneal hemangiopericytoma 10.4 PECAM1 CD34
33 erythema elevatum diutinum 10.4 PECAM1 CD34
34 liver leiomyoma 10.4 PECAM1 CD34
35 spleen cancer 10.4 PECAM1 CD34
36 heart sarcoma 10.4 PECAM1 CD34
37 vulvar leiomyoma 10.4 PECAM1 CD34
38 stork bite 10.4 PROX1 FLT4
39 skin sarcoma 10.4 PECAM1 CD34
40 intramuscular hemangioma 10.4 LYVE1 CD34
41 benign mesothelioma 10.4 PECAM1 CD34
42 bacillary angiomatosis 10.4 PECAM1 CD34
43 histiocytoid hemangioma 10.4 PECAM1 CD34
44 angiolipoma 10.4 PECAM1 CD34
45 benign mesenchymoma 10.4 PECAM1 CD34
46 glomangioma 10.4 PECAM1 CD34
47 angiokeratoma circumscriptum 10.4 PECAM1 CD34
48 pulmonary artery leiomyosarcoma 10.4 PECAM1 CD34
49 adenoid squamous cell carcinoma 10.4 PECAM1 CD34
50 orbital lymphangioma 10.4 PDPN FLT4

Graphical network of the top 20 diseases related to Capillary Lymphangioma:



Diseases related to Capillary Lymphangioma

Symptoms & Phenotypes for Capillary Lymphangioma

MGI Mouse Phenotypes related to Capillary Lymphangioma:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 10.07 CRBN CRP ENG FLNA FLT4 LYVE1
2 hematopoietic system MP:0005397 9.96 CD34 CRBN ENG FLNA LYVE1 NTRK1
3 digestive/alimentary MP:0005381 9.87 ENG FLNA FLT4 PDPN PECAM1 PROX1
4 homeostasis/metabolism MP:0005376 9.85 CD34 CRBN CRP ENG FLNA FLT4
5 immune system MP:0005387 9.7 CD34 CRBN CRP FLNA FLT4 LYVE1
6 muscle MP:0005369 9.1 ENG FLT4 NTRK1 PDPN PECAM1 PROX1

Drugs & Therapeutics for Capillary Lymphangioma

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

(show all 10)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Sirolimus Approved, Investigational Phase 2 53123-88-9 5284616 6436030 46835353
2
Everolimus Approved Phase 2 159351-69-6 6442177 70789204
3
Miconazole Approved, Investigational, Vet_approved Phase 2 22916-47-8 4189
4 Anti-Infective Agents Phase 2
5 Antibiotics, Antitubercular Phase 2
6 Pharmaceutical Solutions Phase 2
7 Anti-Bacterial Agents Phase 2
8 Antifungal Agents Phase 2
9 Immunosuppressive Agents Phase 2
10 Immunologic Factors Phase 2

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 TOPical Sirolimus in linGUal Microcystic Lymphatic Malformation -TOPGUN Active, not recruiting NCT04128722 Phase 2 Sirolimus Oral Liquid Product 1mg/mL

Search NIH Clinical Center for Capillary Lymphangioma

Genetic Tests for Capillary Lymphangioma

Anatomical Context for Capillary Lymphangioma

MalaCards organs/tissues related to Capillary Lymphangioma:

40
Skin, Tongue, Heart, Breast, Spleen, Liver, Bone

Publications for Capillary Lymphangioma

Articles related to Capillary Lymphangioma:

(show all 12)
# Title Authors PMID Year
1
Topical rapamycin in superficial lymphatic malformation: microcystic lymphatic malformation or verrucous venous malformation? 61
30710605 2019
2
Net-like superficial lymphatic malformation: a new entity? 61
29736938 2018
3
Dermoscopy of lymphangioma circumscriptum: A morphological study of 45 cases. 61
28752523 2018
4
Hobnail hemangioma reclassified as superficial lymphatic malformation: a study of 52 cases. 61
21821311 2012
5
[Diagnosis and surgical treatment of cervical lymphangioma]. 61
20223116 2009
6
Treatment of lymphangioma circumscriptum with the intense pulsed light system. 61
17973881 2007
7
Lymphangioma circumscriptum of the vulva: a review of the literature. 61
12738156 2003
8
Late-onset superficial lymphatic malformation: report of a case and review of the literature. 61
12614426 2003
9
[Surgical analysis of mediastinal lymphangioma--analysis of 7 cases]. 61
1613288 1992
10
[A capillary lymphangioma of the vestibular portion of the larynx]. 61
5161795 1971
11
Capillary lymphangioma of the tongue complicated by glossitis. 61
13753050 1961
12
[Surgical failure in Torok's congenital varicose capillary lymphangioma]. 61
14405823 1959

Variations for Capillary Lymphangioma

Cosmic variations for Capillary Lymphangioma:

9
# Cosmic Mut ID Gene Symbol COSMIC Disease Classification
(Primary site, Site subtype, Primary histology, Histology subtype)
Mutation CDS Mutation AA GRCh38 Location Conf
1 COSM87132301 PIK3CA soft tissue,tongue,Overgrowth syndrome,lymphangioma c.1624G>A p.E542K 3:179218294-179218294 4

Expression for Capillary Lymphangioma

Search GEO for disease gene expression data for Capillary Lymphangioma.

Pathways for Capillary Lymphangioma

Pathways related to Capillary Lymphangioma according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1 11.05 VWF PROX1 PECAM1 PDPN FLT4 ENG
2 10.55 FLNA ENG
3 9.96 VWF FLNA

GO Terms for Capillary Lymphangioma

Cellular components related to Capillary Lymphangioma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 cell periphery GO:0071944 8.8 PECAM1 LYVE1 CD34

Biological processes related to Capillary Lymphangioma according to GeneCards Suite gene sharing:

(show all 11)
# Name GO ID Score Top Affiliating Genes
1 cell adhesion GO:0007155 9.77 VWF PECAM1 LYVE1 ENG CD34
2 platelet degranulation GO:0002576 9.65 VWF PECAM1 FLNA
3 platelet activation GO:0030168 9.61 VWF PDPN FLNA
4 positive regulation of neural precursor cell proliferation GO:2000179 9.49 PROX1 FLNA
5 wound healing, spreading of cells GO:0044319 9.46 PDPN FLNA
6 positive regulation of protein phosphorylation GO:0001934 9.46 PECAM1 NTRK1 FLT4 ENG
7 lymph vessel development GO:0001945 9.4 PROX1 FLT4
8 venous blood vessel morphogenesis GO:0048845 9.32 PROX1 ENG
9 atrial cardiac muscle tissue morphogenesis GO:0055009 9.26 PROX1 ENG
10 glomerular endothelium development GO:0072011 8.96 PECAM1 CD34
11 lymphangiogenesis GO:0001946 8.8 PROX1 PDPN FLT4

Molecular functions related to Capillary Lymphangioma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 protein homodimerization activity GO:0042803 9.1 VWF PECAM1 NTRK1 FLT4 FLNA ENG

Sources for Capillary Lymphangioma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
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
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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