MCID: LYM095
MIFTS: 35

Lymphangiomatosis

Categories: Immune diseases, Rare diseases

Aliases & Classifications for Lymphangiomatosis

MalaCards integrated aliases for Lymphangiomatosis:

Name: Lymphangiomatosis 53 37 73

Classifications:



External Ids:

KEGG 37 H01735
UMLS 73 C0334102

Summaries for Lymphangiomatosis

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, spleen and colon.

Wikipedia : 76 Lymphangiomatosis (LYMF) is a condition where a lymphangioma is not present in a single localised mass,... more...

Related Diseases for Lymphangiomatosis

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

(show top 50) (show all 115)
# Related Disease Score Top Affiliating Genes
1 lymphangioleiomyomatosis 29.5 DES PGR
2 kaposiform lymphangiomatosis 12.2
3 lymphangiectasia, pulmonary, congenital 11.3
4 diffuse lymphatic malformation 11.2
5 gorham's disease 10.2
6 protein-losing enteropathy 10.1
7 lymphangioma 10.1
8 cystic lymphangioma 10.1
9 hemangioendothelioma 10.1
10 kaposiform hemangioendothelioma 10.1
11 disseminated intravascular coagulation 10.1
12 splenomegaly 10.1
13 infantile digital fibromatosis 10.0 DES VIM
14 fibromatosis 10.0 DES VIM
15 angiolipoma 10.0 DES VIM
16 primitive neuroectodermal tumor of the cervix uteri 10.0 DES VIM
17 cutaneous leiomyosarcoma 10.0 DES VIM
18 ovarian mucinous cystadenocarcinoma 10.0 PCNA VIM
19 mucinous cystadenocarcinoma 10.0 PCNA VIM
20 spindle cell lipoma 10.0 DES VIM
21 parachordoma 10.0 DES VIM
22 chordoma 10.0 DES VIM
23 syringocystadenoma papilliferum 10.0 DES VIM
24 ossifying fibromyxoid tumor 10.0 DES VIM
25 epithelioid leiomyosarcoma 10.0 DES VIM
26 biphasic synovial sarcoma 10.0 DES VIM
27 syringoma 10.0 DES VIM
28 juvenile xanthogranuloma 10.0 DES VIM
29 pleomorphic liposarcoma 10.0 DES VIM
30 liposarcoma 10.0 DES VIM
31 desmoplastic small round cell tumor 10.0 DES VIM
32 odontoma 10.0 PCNA VIM
33 cutaneous fibrous histiocytoma 10.0 DES VIM
34 fibrous histiocytoma 10.0 DES VIM
35 congenital fibrosarcoma 10.0 DES VIM
36 fibrosarcoma 10.0 DES VIM
37 reticulum cell sarcoma 10.0 DES VIM
38 mesenchymal chondrosarcoma 10.0 DES VIM
39 extraskeletal ewing sarcoma 10.0 DES VIM
40 pseudosarcomatous fibromatosis 10.0 DES VIM
41 glomus tumor 10.0 DES VIM
42 spindle cell sarcoma 10.0 DES VIM
43 granular cell tumor 10.0 DES VIM
44 embryonal sarcoma 10.0 DES VIM
45 hemangiopericytoma, malignant 10.0 DES VIM
46 infantile myofibromatosis 10.0 DES VIM
47 cystic angiomatosis of bone, diffuse 10.0
48 hemangioma-thrombocytopenia syndrome 10.0
49 ascites, chylous 10.0
50 heart disease 10.0

Graphical network of the top 20 diseases related to Lymphangiomatosis:



Diseases related to Lymphangiomatosis

Symptoms & Phenotypes for Lymphangiomatosis

Drugs & Therapeutics for Lymphangiomatosis

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 6436030 5284616
2
Everolimus Approved Phase 2 159351-69-6 6442177
3
Miconazole Approved, Investigational, Vet_approved Phase 2 22916-47-8 4189
4 Antibiotics, Antitubercular Phase 2
5 Immunologic Factors Phase 2
6 Immunosuppressive Agents Phase 2
7 Anti-Infective Agents Phase 2
8 Anti-Bacterial 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

Genetic Tests for Lymphangiomatosis

Anatomical Context for Lymphangiomatosis

MalaCards organs/tissues related to Lymphangiomatosis:

41
Bone, Spleen, Colon, Liver, Endothelial, Heart, Kidney

Publications for Lymphangiomatosis

Articles related to Lymphangiomatosis:

(show top 50) (show all 297)
# Title Authors Year
1
Diffuse pulmonary lymphangiomatosis treated with bevacizumab. ( 30510764 )
2019
2
Isolated cystic lymphangiomatosis of spleen in an adult: a diagnostic conundrum. ( 29622712 )
2018
3
Pseudotumor cerebri in kaposiform lymphangiomatosis: a case report and pathogenetic hypothesis. ( 29654359 )
2018
4
Lymphoscintigraphy of chylous anomalies: chylothorax, chyloperitoneum, chyluria and lymphangiomatosis. Fifteen year experience in a pediatric setting and review of the literature. ( 29438003 )
2018
5
Lymphangiomatosis: a rare entity presenting with involvement of the sacral plexus. ( 29468291 )
2018
6
Chest imaging in generalized lymphatic anomaly and kaposiform lymphangiomatosis. ( 29923669 )
2018
7
Proliferative Cells From Kaposiform Lymphangiomatosis Lesions Resemble Mesenchyme Stem Cell-like Pericytes Defective in Vessel Formation. ( 30256265 )
2018
8
A somatic activating NRAS variant associated with kaposiform lymphangiomatosis. ( 30542204 )
2018
9
Lymphangiomatosis in a 14-year-old female presenting with chylothorax and multiple cystic lesions. ( 30002781 )
2018
10
Splenic cystic lymphangiomatosis in association with omental varices and portal hypertension: A case report. ( 30290613 )
2018
11
Diffuse Pulmonary Lymphangiomatosis. ( 30324183 )
2018
12
Gorham disease: lymphangiomatosis with massive osteolysis. ( 27829197 )
2017
13
Abdominal Lymphangiomatosis With Intestinal Lymphangiectasia Diagnosed by Magnetic Resonance Lymphangiography: A Case Report. ( 28554788 )
2017
14
Kaposiform Lymphangiomatosis with Human Papillomavirus Infection. ( 28398067 )
2017
15
MR imaging findings of vertebral involvement in Gorham-Stout disease, generalized lymphatic anomaly, and kaposiform lymphangiomatosis. ( 28795277 )
2017
16
Lymphangiomatosis involving the pulmonary and extrapulmonary lymph nodes and surrounding soft tissue: A rare case report. ( 29245295 )
2017
17
Renal lymphangiomatosis, a rare differential diagnosis for autosomal recessive polycystic kidney disease in pediatric patients. ( 28228882 )
2017
18
Detection of mosaic 15q11.1-q11.2 deletion encompassing NBEAP1 andA POTEB in a fetus with diffuse lymphangiomatosis. ( 28420514 )
2017
19
Diffuse Pulmonary Lymphangiomatosis: MDCT Findings After Direct Lymphangiography. ( 27845836 )
2017
20
Giant pulmonary teratoma with huge splenic lymphangiomatosis: a very rare case. ( 28928924 )
2017
21
Multiple Submucosal Masses in the Esophagus: Esophageal Lymphangiomatosis ( 28978954 )
2017
22
Lymphangiomatosis of the sigmoid colon - a rare cause of lower gastrointestinal bleeding: A case report and review of the literature. ( 28123564 )
2017
23
A novel case of lymphangiomatosis in HIV. ( 28189546 )
2017
24
Genital reconstruction with Integraa8c artificial dermis after radical resection in a boy with diffuse lymphangiomatosis. ( 29205164 )
2017
25
Imaging features of kaposiform lymphangiomatosis. ( 27053281 )
2016
26
Disseminated lymphangiomatosis presenting as chylous ascites and diagnosed with endoscopic ultrasound. ( 27386481 )
2016
27
Young patient with generalized lymphangiomatosis: Differentiating the differential. ( 27857472 )
2016
28
Thoracic involvement in generalised lymphatic anomaly (or lymphangiomatosis). ( 27246594 )
2016
29
Pathology analysis of a rare case of diffuse pulmonary lymphangiomatosis. ( 27127767 )
2016
30
Disseminated spinal lymphangiomatosis. ( 27606034 )
2016
31
Lymphatic Malformation in Newborns as the First Sign of Diffuse Lymphangiomatosis: Successful Treatment with Sirolimus. ( 26506225 )
2016
32
Primary intestinal lymphangiomatosis of the ileum in an adult-the role of surgical approach. ( 27534888 )
2016
33
Percutaneous transoral clivoplasty and upper cervical vertebroplasties for multifocal skeletal lymphangiomatosis resulting in complete resolution of pain: case report. ( 27767681 )
2016
34
Point-of-Care Thoracic Ultrasonography in the Diagnosis and Management of Kaposiform Lymphangiomatosis. ( 27898632 )
2016
35
Abdominal Kaposiform Hemangioendothelioma Associated With Lymphangiomatosis Involving Mesentery and Ileum: A Case Report of MRI, CT, and 18F-FDG PET/CT Findings. ( 26871848 )
2016
36
Imatinib Treatment of Lymphangiomatosis (Generalized Lymphatic Anomaly). ( 27059187 )
2016
37
Clinical Features and Prognosis of Generalized Lymphatic Anomaly, Kaposiform Lymphangiomatosis, and Gorham-Stout Disease. ( 26806875 )
2016
38
Colonic lymphangiomatosis resolved after excisional biopsy. ( 25674533 )
2015
39
A case of diffuse pulmonary lymphangiomatosis with unilateral lung invasion. ( 26512334 )
2015
40
Whole-body MRI in generalized cystic lymphangiomatosis in the pediatric population: diagnosis, differential diagnoses, and follow-up. ( 26545884 )
2015
41
Near-Infrared Fluorescence Lymphatic Imaging in Lymphangiomatosis. ( 26287470 )
2015
42
Lymphangiomatosis: The Cause of Refractory Pleural Effusion in a Patient with Lupus Erythematosus. ( 26528371 )
2015
43
Successful treatment of kaposiform lymphangiomatosis with sirolimus. ( 25598153 )
2015
44
Systemic Generalized Lymphangiomatosis: A Diagnostic Challenge. ( 26897985 )
2015
45
Lymphangiomatosis involving the mediastinum and spleen in an adult woman. ( 26207000 )
2015
46
Giant splenic lymphangiomatosis in adult: a diagnostic dilemma. ( 25972674 )
2015
47
Lymphangiomatosis: Two cases with unique presentations, salience of nomenclature, and diagnosis. ( 26458634 )
2015
48
Radiographical resolution of renal lymphangiomatosis following cardiac transplantation. ( 24775581 )
2014
49
Kaposiform lymphangiomatosis, a newly characterized vascular anomaly presenting with hemoptysis in an adult woman. ( 24460439 )
2014
50
Diffuse lymphangiomatosis: gorham-stout syndrome. ( 24390534 )
2014

Variations for Lymphangiomatosis

Expression for Lymphangiomatosis

Search GEO for disease gene expression data for Lymphangiomatosis.

Pathways for Lymphangiomatosis

Pathways related to Lymphangiomatosis according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Show member pathways
11.29 DES VIM
2 11.02 PCNA VIM
3 10.81 DES VIM
4
Show member pathways
10.28 DES VIM

GO Terms for Lymphangiomatosis

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

Sources for Lymphangiomatosis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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