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MCID: LYM095
MIFTS: 34

Lymphangiomatosis

Categories: Immune diseases, Rare diseases
Genes Tissues Related diseases Publications Pathways Drugs
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Aliases & Classifications for Lymphangiomatosis

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MalaCards integrated aliases for Lymphangiomatosis:

Name: Lymphangiomatosis 54 38 74

Classifications:

MalaCards categories:
Global: Rare diseases
Anatomical: Immune diseases
See all MalaCards categories (disease lists)


External Ids:

KEGG 38 H01735
UMLS 74 C0334102
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Summaries for Lymphangiomatosis

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MalaCards based summary : Lymphangiomatosis is related to lymphangioleiomyomatosis and kaposiform lymphangiomatosis. An important gene associated with Lymphangiomatosis is DES (Desmin), and among its related pathways/superpathways are Cytoskeleton remodeling Neurofilaments and Primary Focal Segmental Glomerulosclerosis FSGS. The drugs Sirolimus and Everolimus have been mentioned in the context of this disorder. Affiliated tissues include bone, liver and kidney.

Wikipedia : 77 Lymphangiomatosis is a condition where a lymphangioma is not present in a single localised mass, but in... more...

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Related Diseases for Lymphangiomatosis

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

(show top 50) (show all 120)
# Related Disease Score Top Affiliating Genes
1 lymphangioleiomyomatosis 29.6 DES PGR
2 kaposiform lymphangiomatosis 12.5
3 lymphangiectasia, pulmonary, congenital 11.4
4 diffuse lymphatic malformation 11.3
5 gorham's disease 10.3
6 protein-losing enteropathy 10.2
7 lymphangioma 10.2
8 cystic lymphangioma 10.2
9 hemangioendothelioma 10.2
10 kaposiform hemangioendothelioma 10.2
11 infantile digital fibromatosis 10.1 DES VIM
12 fibromatosis 10.1 DES VIM
13 angiolipoma 10.1 DES VIM
14 primitive neuroectodermal tumor of the cervix uteri 10.1 DES VIM
15 spindle cell lipoma 10.1 DES VIM
16 parachordoma 10.1 DES VIM
17 chordoma 10.1 DES VIM
18 syringocystadenoma papilliferum 10.1 DES VIM
19 disseminated intravascular coagulation 10.1
20 splenomegaly 10.1
21 ossifying fibromyxoid tumor 10.1 DES VIM
22 epithelioid leiomyosarcoma 10.1 DES VIM
23 biphasic synovial sarcoma 10.1 DES VIM
24 syringoma 10.1 DES VIM
25 juvenile xanthogranuloma 10.1 DES VIM
26 pleomorphic liposarcoma 10.1 DES VIM
27 liposarcoma 10.1 DES VIM
28 desmoplastic small round cell tumor 10.1 DES VIM
29 cutaneous fibrous histiocytoma 10.1 DES VIM
30 fibrous histiocytoma 10.1 DES VIM
31 congenital fibrosarcoma 10.1 DES VIM
32 fibrosarcoma 10.1 DES VIM
33 reticulum cell sarcoma 10.1 DES VIM
34 mesenchymal chondrosarcoma 10.1 DES VIM
35 extraskeletal ewing sarcoma 10.1 DES VIM
36 pseudosarcomatous fibromatosis 10.1 DES VIM
37 glomus tumor 10.1 DES VIM
38 spindle cell sarcoma 10.1 DES VIM
39 granular cell tumor 10.1 DES VIM
40 embryonal sarcoma 10.1 DES VIM
41 hemangiopericytoma, malignant 10.0 DES VIM
42 benign breast phyllodes tumor 10.0 PGR VIM
43 endometrial mucinous adenocarcinoma 10.0 PGR VIM
44 infantile myofibromatosis 10.0 DES VIM
45 clear cell meningioma 10.0 PGR VIM
46 malignant fibroxanthoma 10.0 DES VIM
47 chondromyxoid fibroma 10.0 DES VIM
48 adenosarcoma 10.0 DES PGR
49 rhabdoid tumor predisposition syndrome 1 10.0 DES VIM
50 cystic angiomatosis of bone, diffuse 10.0

Graphical network of the top 20 diseases related to Lymphangiomatosis:



Diseases related to Lymphangiomatosis
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Symptoms & Phenotypes for Lymphangiomatosis

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Drugs & Therapeutics for Lymphangiomatosis

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

(show all 12)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Sirolimus Approved, Investigational Phase 2 53123-88-9 46835353 5284616 6436030
2
Everolimus Approved Phase 2 159351-69-6 70789204 6442177
3
Miconazole Approved, Investigational, Vet_approved Phase 2 22916-47-8 4189
4 Anti-Infective Agents Phase 2
5 Anti-Bacterial Agents Phase 2
6 Immunologic Factors Phase 2
7 Antibiotics, Antitubercular Phase 2
8 Immunosuppressive Agents Phase 2
9 Antifungal Agents Phase 2
10 Anesthetics Not Applicable
11 Endothelial Growth Factors
12 Mitogens

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Safety and Efficacy Study of Sirolimus in Complicated Vascular Anomalies Active, not recruiting NCT00975819 Phase 2 sirolimus
2 Imaging of Lymphatic Anomalies Completed NCT02744027 Not Applicable
3 Characterization of the Pathogenesis of Primary and Secondary Lymphatic Disorders Recruiting NCT02156115
4 Identification of Biomarkers for Patients With Vascular Anomalies Recruiting NCT03001180
5 Lymphatic Anomalies Registry Recruiting NCT02399527
6 International Lymphatic Disease and Lymphedema Registry Recruiting NCT01336790

Search NIH Clinical Center for Lymphangiomatosis

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Genetic Tests for Lymphangiomatosis

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Anatomical Context for Lymphangiomatosis

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MalaCards organs/tissues related to Lymphangiomatosis:

42
Bone, Liver, Kidney, Heart, Spleen, Colon, Endothelial
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Publications for Lymphangiomatosis

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Articles related to Lymphangiomatosis:

(show top 50) (show all 301)
# Title Authors Year
1
Signaling pathways and inhibitors of cells from patients with kaposiform lymphangiomatosis. ( 31045327 )
2019
2
Diffuse pulmonary lymphangiomatosis treated with bevacizumab. ( 30510764 )
2019
3
Propranolol treatment for chylothorax due to diffuse lymphangiomatosis. ( 30609288 )
2019
4
Lymphangiomatosis of the ileum with perforation: A case report and review of the literature. ( 30992989 )
2019
5
Thoracic involvement of diffuse lymphangiomatosis successfully treated with sildenafil. ( 31015245 )
2019
6
Diagnosis of Lymphangiomatosis: A Study Based on CT Lymphangiography. ( 31076330 )
2019
7
Pseudotumor cerebri in kaposiform lymphangiomatosis: a case report and pathogenetic hypothesis. ( 29654359 )
2018
8
Chest imaging in generalized lymphatic anomaly and kaposiform lymphangiomatosis. ( 29923669 )
2018
9
Proliferative Cells From Kaposiform Lymphangiomatosis Lesions Resemble Mesenchyme Stem Cell-like Pericytes Defective in Vessel Formation. ( 30256265 )
2018
10
A somatic activating NRAS variant associated with kaposiform lymphangiomatosis. ( 30542204 )
2018
11
Abdominal Lymphangiomatosis With Intestinal Lymphangiectasia Diagnosed by Magnetic Resonance Lymphangiography: A Case Report. ( 28554788 )
2018
12
Lymphoscintigraphy of Chylous Anomalies: Chylothorax, Chyloperitoneum, Chyluria, and Lymphangiomatosis-15-Year Experience in a Pediatric Setting and Review of the Literature. ( 29438003 )
2018
13
Lymphangiomatosis: a rare entity presenting with involvement of the sacral plexus. ( 29468291 )
2018
14
Isolated cystic lymphangiomatosis of spleen in an adult: a diagnostic conundrum. ( 29622712 )
2018
15
Lymphangiomatosis in a 14-year-old female presenting with chylothorax and multiple cystic lesions. ( 30002781 )
2018
16
Splenic cystic lymphangiomatosis in association with omental varices and portal hypertension: A case report. ( 30290613 )
2018
17
Diffuse Pulmonary Lymphangiomatosis. ( 30324183 )
2018
18
Kaposiform Lymphangiomatosis with Human Papillomavirus Infection. ( 28398067 )
2017
19
MR imaging findings of vertebral involvement in Gorham-Stout disease, generalized lymphatic anomaly, and kaposiform lymphangiomatosis. ( 28795277 )
2017
20
Diffuse Pulmonary Lymphangiomatosis: MDCT Findings After Direct Lymphangiography. ( 27845836 )
2017
21
Lymphangiomatosis of the sigmoid colon - a rare cause of lower gastrointestinal bleeding: A case report and review of the literature. ( 28123564 )
2017
22
A novel case of lymphangiomatosis in HIV. ( 28189546 )
2017
23
Renal lymphangiomatosis, a rare differential diagnosis for autosomal recessive polycystic kidney disease in pediatric patients. ( 28228882 )
2017
24
Detection of mosaic 15q11.1-q11.2 deletion encompassing NBEAP1 and POTEB in a fetus with diffuse lymphangiomatosis. ( 28420514 )
2017
25
Giant pulmonary teratoma with huge splenic lymphangiomatosis: a very rare case. ( 28928924 )
2017
26
Multiple Submucosal Masses in the Esophagus: Esophageal Lymphangiomatosis ( 28978954 )
2017
27
Genital reconstruction with Integra™ artificial dermis after radical resection in a boy with diffuse lymphangiomatosis. ( 29205164 )
2017
28
Lymphangiomatosis involving the pulmonary and extrapulmonary lymph nodes and surrounding soft tissue: A rare case report. ( 29245295 )
2017
29
Lymphangiomatosis involving the mediastinum and spleen in an adult woman. ( 26207000 )
2017
30
Percutaneous transoral clivoplasty and upper cervical vertebroplasties for multifocal skeletal lymphangiomatosis resulting in complete resolution of pain: case report. ( 27767681 )
2017
31
Gorham disease: lymphangiomatosis with massive osteolysis. ( 27829197 )
2017
32
Abdominal Kaposiform Hemangioendothelioma Associated With Lymphangiomatosis Involving Mesentery and Ileum: A Case Report of MRI, CT, and 18F-FDG PET/CT Findings. ( 26871848 )
2016
33
Clinical Features and Prognosis of Generalized Lymphatic Anomaly, Kaposiform Lymphangiomatosis, and Gorham-Stout Disease. ( 26806875 )
2016
34
Imaging features of kaposiform lymphangiomatosis. ( 27053281 )
2016
35
Point-of-Care Thoracic Ultrasonography in the Diagnosis and Management of Kaposiform Lymphangiomatosis. ( 27898632 )
2016
36
Young patient with generalized lymphangiomatosis: Differentiating the differential. ( 27857472 )
2016
37
Sudden death of a 14-year-old girl with lymphangiomatosis. ( 25047633 )
2016
38
Lymphatic Malformation in Newborns as the First Sign of Diffuse Lymphangiomatosis: Successful Treatment with Sirolimus. ( 26506225 )
2016
39
Whole-body MRI in generalized cystic lymphangiomatosis in the pediatric population: diagnosis, differential diagnoses, and follow-up. ( 26545884 )
2016
40
Imatinib Treatment of Lymphangiomatosis (Generalized Lymphatic Anomaly). ( 27059187 )
2016
41
Pathology analysis of a rare case of diffuse pulmonary lymphangiomatosis. ( 27127767 )
2016
42
Thoracic involvement in generalised lymphatic anomaly (or lymphangiomatosis). ( 27246594 )
2016
43
Disseminated lymphangiomatosis presenting as chylous ascites and diagnosed with endoscopic ultrasound. ( 27386481 )
2016
44
Primary intestinal lymphangiomatosis of the ileum in an adult-the role of surgical approach. ( 27534888 )
2016
45
Disseminated spinal lymphangiomatosis. ( 27606034 )
2016
46
Kaposiform lymphangiomatosis: unifying features of a heterogeneous disorder. ( 25307772 )
2015
47
Successful treatment of kaposiform lymphangiomatosis with sirolimus. ( 25598153 )
2015
48
Lymphangiomatosis with dental involvement. ( 25192473 )
2015
49
Colonic lymphangiomatosis resolved after excisional biopsy. ( 25674533 )
2015
50
Giant splenic lymphangiomatosis in adult: a diagnostic dilemma. ( 25972674 )
2015
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Variations for Lymphangiomatosis

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Expression for Lymphangiomatosis

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Search GEO for disease gene expression data for Lymphangiomatosis.
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Pathways for Lymphangiomatosis

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Pathways related to Lymphangiomatosis according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Cytoskeleton remodeling Neurofilaments 23
Show member pathways
 11.29 DES VIM
2
Primary Focal Segmental Glomerulosclerosis FSGS 1
11.02 PCNA VIM
3
Striated Muscle Contraction 1 67
10.81 DES VIM
4
Aurora B signaling 1
Show member pathways
 10.28 DES VIM
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GO Terms for Lymphangiomatosis

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

# Name GO ID Score Top Affiliating Genes
1 intermediate filament GO:0005882 8.62 DES VIM

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

# Name GO ID Score Top Affiliating Genes
1 muscle filament sliding GO:0030049 8.96 DES VIM
2 intermediate filament organization GO:0045109 8.62 DES VIM

Molecular functions related to Lymphangiomatosis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 structural constituent of cytoskeleton GO:0005200 8.96 DES VIM
2 identical protein binding GO:0042802 8.92 DES PCNA PGR VIM
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Sources for Lymphangiomatosis

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3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 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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