MCID: LYM095
MIFTS: 35

Lymphangiomatosis

Categories: Rare diseases, Immune 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 Miconazole 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 122)
# Related Disease Score Top Affiliating Genes
1 lymphangioleiomyomatosis 28.7 DES PGR
2 kaposiform lymphangiomatosis 12.1
3 diffuse lymphatic malformation 11.4
4 lymphangiectasia, pulmonary, congenital 11.2
5 infantile digital fibromatosis 10.3 DES VIM
6 angiolipoma 10.3 DES VIM
7 primitive neuroectodermal tumor of the cervix uteri 10.3 DES VIM
8 spindle cell lipoma 10.3 DES VIM
9 parachordoma 10.3 DES VIM
10 ossifying fibromyxoid tumor 10.3 DES VIM
11 epithelioid leiomyosarcoma 10.3 DES VIM
12 syringocystadenoma papilliferum 10.2 DES VIM
13 biphasic synovial sarcoma 10.2 DES VIM
14 congenital fibrosarcoma 10.2 DES VIM
15 syringoma 10.2 DES VIM
16 juvenile xanthogranuloma 10.2 DES VIM
17 pleomorphic liposarcoma 10.2 DES VIM
18 desmoplastic small round cell tumor 10.2 DES VIM
19 reticulum cell sarcoma 10.2 DES VIM
20 cutaneous fibrous histiocytoma 10.2 DES VIM
21 mesenchymal chondrosarcoma 10.2 DES VIM
22 extraskeletal ewing sarcoma 10.2 DES VIM
23 glomus tumor 10.2 DES VIM
24 lymphangioma 10.2
25 spindle cell sarcoma 10.2 DES VIM
26 pseudosarcomatous fibromatosis 10.2 DES VIM
27 granular cell tumor 10.2 DES VIM
28 embryonal sarcoma 10.2 DES VIM
29 rhabdoid cancer 10.2 DES VIM
30 hepatitis 10.2
31 hemangiopericytoma, malignant 10.2 DES VIM
32 infantile myofibromatosis 10.2 DES VIM
33 ovarian mucinous cystadenocarcinoma 10.1 PCNA VIM
34 chondromyxoid fibroma 10.1 DES VIM
35 malignant fibroxanthoma 10.1 DES VIM
36 fasciitis 10.1 DES VIM
37 glomangioma 10.1 DES VIM
38 fibromatosis 10.1 DES VIM
39 odontoma 10.1 PCNA VIM
40 mucinous cystadenocarcinoma 10.1 PCNA VIM
41 neurilemmoma 10.1 DES VIM
42 atypical teratoid rhabdoid tumor 10.1 DES VIM
43 fibrous histiocytoma 10.0 DES VIM
44 alveolar soft part sarcoma 10.0 DES VIM
45 gorham's disease 10.0
46 mediastinitis 10.0
47 dermatofibrosarcoma protuberans 10.0 DES VIM
48 giant cell tumor 10.0 DES VIM
49 cystic lymphangioma 10.0
50 kaposiform hemangioendothelioma 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 11)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Miconazole Approved, Investigational, Vet_approved Phase 2 22916-47-8 4189
2
Everolimus Approved Phase 2 159351-69-6 6442177
3
Sirolimus Approved, Investigational Phase 2 53123-88-9 5284616 6436030 46835353
4 Anti-Bacterial Agents Phase 2
5 Antibiotics, Antitubercular Phase 2
6 Immunosuppressive Agents Phase 2
7 Anti-Infective Agents Phase 2
8 Antifungal Agents Phase 2
9 Anesthetics Not Applicable
10 Mitogens
11 Endothelial Growth Factors

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, Lung, Heart

Publications for Lymphangiomatosis

Articles related to Lymphangiomatosis:

(show top 50) (show all 280)
# Title Authors Year
1
Isolated cystic lymphangiomatosis of spleen in an adult: a diagnostic conundrum. ( 29622712 )
2018
2
Pseudotumor cerebri in kaposiform lymphangiomatosis: a case report and pathogenetic hypothesis. ( 29654359 )
2018
3
Lymphoscintigraphy of chylous anomalies: chylothorax, chyloperitoneum, chyluria and lymphangiomatosis. Fifteen year experience in a pediatric setting and review of the literature. ( 29438003 )
2018
4
Lymphangiomatosis: a rare entity presenting with involvement of the sacral plexus. ( 29468291 )
2018
5
Chest imaging in generalized lymphatic anomaly and kaposiform lymphangiomatosis. ( 29923669 )
2018
6
Gorham disease: lymphangiomatosis with massive osteolysis. ( 27829197 )
2017
7
Abdominal Lymphangiomatosis With Intestinal Lymphangiectasia Diagnosed by Magnetic Resonance Lymphangiography: A Case Report. ( 28554788 )
2017
8
Kaposiform Lymphangiomatosis with Human Papillomavirus Infection. ( 28398067 )
2017
9
MR imaging findings of vertebral involvement in Gorham-Stout disease, generalized lymphatic anomaly, and kaposiform lymphangiomatosis. ( 28795277 )
2017
10
Lymphangiomatosis involving the pulmonary and extrapulmonary lymph nodes and surrounding soft tissue: A rare case report. ( 29245295 )
2017
11
Renal lymphangiomatosis, a rare differential diagnosis for autosomal recessive polycystic kidney disease in pediatric patients. ( 28228882 )
2017
12
Detection of mosaic 15q11.1-q11.2 deletion encompassing NBEAP1 andA POTEB in a fetus with diffuse lymphangiomatosis. ( 28420514 )
2017
13
Diffuse Pulmonary Lymphangiomatosis: MDCT Findings After Direct Lymphangiography. ( 27845836 )
2017
14
Giant pulmonary teratoma with huge splenic lymphangiomatosis: a very rare case. ( 28928924 )
2017
15
Multiple Submucosal Masses in the Esophagus: Esophageal Lymphangiomatosis ( 28978954 )
2017
16
Lymphangiomatosis of the sigmoid colon - a rare cause of lower gastrointestinal bleeding: A case report and review of the literature. ( 28123564 )
2017
17
A novel case of lymphangiomatosis in HIV. ( 28189546 )
2017
18
Genital reconstruction with Integraa8c artificial dermis after radical resection in a boy with diffuse lymphangiomatosis. ( 29205164 )
2017
19
Imaging features of kaposiform lymphangiomatosis. ( 27053281 )
2016
20
Disseminated lymphangiomatosis presenting as chylous ascites and diagnosed with endoscopic ultrasound. ( 27386481 )
2016
21
Young patient with generalized lymphangiomatosis: Differentiating the differential. ( 27857472 )
2016
22
Thoracic involvement in generalised lymphatic anomaly (or lymphangiomatosis). ( 27246594 )
2016
23
Pathology analysis of a rare case of diffuse pulmonary lymphangiomatosis. ( 27127767 )
2016
24
Disseminated spinal lymphangiomatosis. ( 27606034 )
2016
25
Lymphatic Malformation in Newborns as the First Sign of Diffuse Lymphangiomatosis: Successful Treatment with Sirolimus. ( 26506225 )
2016
26
Primary intestinal lymphangiomatosis of the ileum in an adult-the role of surgical approach. ( 27534888 )
2016
27
Percutaneous transoral clivoplasty and upper cervical vertebroplasties for multifocal skeletal lymphangiomatosis resulting in complete resolution of pain: case report. ( 27767681 )
2016
28
Point-of-Care Thoracic Ultrasonography in the Diagnosis and Management of Kaposiform Lymphangiomatosis. ( 27898632 )
2016
29
Abdominal Kaposiform Hemangioendothelioma Associated With Lymphangiomatosis Involving Mesentery and Ileum: A Case Report of MRI, CT, and 18F-FDG PET/CT Findings. ( 26871848 )
2016
30
Imatinib Treatment of Lymphangiomatosis (Generalized Lymphatic Anomaly). ( 27059187 )
2016
31
Clinical Features and Prognosis of Generalized Lymphatic Anomaly, Kaposiform Lymphangiomatosis, and Gorham-Stout Disease. ( 26806875 )
2016
32
Colonic lymphangiomatosis resolved after excisional biopsy. ( 25674533 )
2015
33
A case of diffuse pulmonary lymphangiomatosis with unilateral lung invasion. ( 26512334 )
2015
34
Whole-body MRI in generalized cystic lymphangiomatosis in the pediatric population: diagnosis, differential diagnoses, and follow-up. ( 26545884 )
2015
35
Near-Infrared Fluorescence Lymphatic Imaging in Lymphangiomatosis. ( 26287470 )
2015
36
Lymphangiomatosis: The Cause of Refractory Pleural Effusion in a Patient with Lupus Erythematosus. ( 26528371 )
2015
37
Successful treatment of kaposiform lymphangiomatosis with sirolimus. ( 25598153 )
2015
38
Systemic Generalized Lymphangiomatosis: A Diagnostic Challenge. ( 26897985 )
2015
39
Lymphangiomatosis involving the mediastinum and spleen in an adult woman. ( 26207000 )
2015
40
Giant splenic lymphangiomatosis in adult: a diagnostic dilemma. ( 25972674 )
2015
41
Lymphangiomatosis: Two cases with unique presentations, salience of nomenclature, and diagnosis. ( 26458634 )
2015
42
Radiographical resolution of renal lymphangiomatosis following cardiac transplantation. ( 24775581 )
2014
43
Kaposiform lymphangiomatosis, a newly characterized vascular anomaly presenting with hemoptysis in an adult woman. ( 24460439 )
2014
44
Diffuse lymphangiomatosis: gorham-stout syndrome. ( 24390534 )
2014
45
Laboratory models for the investigation of lymphangiomatosis. ( 25173588 )
2014
46
Sudden death of a 14-year-old girl with lymphangiomatosis. ( 25047633 )
2014
47
Importance of MRI in the diagnosis of vertebral involvement in generalized cystic lymphangiomatosis. ( 24950879 )
2014
48
A rare case of diffuse pulmonary lymphangiomatosis in a middle-aged woman. ( 24642766 )
2014
49
Lymphangiomatosis with Dental Involvement: Possible Pathophysiology and Literature Review. ( 25192473 )
2014
50
Kaposiform lymphangiomatosis: Unifying features of a heterogeneous disorder. ( 25307772 )
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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