MCID: CNG029
MIFTS: 37

Congenital Mesoblastic Nephroma malady

Categories: Rare diseases, Nephrological diseases, Cancer diseases

Aliases & Classifications for Congenital Mesoblastic Nephroma

Aliases & Descriptions for Congenital Mesoblastic Nephroma:

Name: Congenital Mesoblastic Nephroma 12 50 56 14 69
Nephroma, Mesoblastic 42

Classifications:

Orphanet: 56  
Rare renal diseases


External Ids:

Disease Ontology 12 DOID:4773
MeSH 42 D018201
NCIt 47 C6569
Orphanet 56 ORPHA2665
MESH via Orphanet 43 D018201
UMLS via Orphanet 70 C1332965
ICD10 via Orphanet 34 D41.0
UMLS 69 C1332965

Summaries for Congenital Mesoblastic Nephroma

MalaCards based summary : Congenital Mesoblastic Nephroma, also known as nephroma, mesoblastic, is related to fibrosarcoma and cellular congenital mesoblastic nephroma. An important gene associated with Congenital Mesoblastic Nephroma is ETV6 (ETS Variant 6), and among its related pathways/superpathways are Phospholipase-C Pathway and p70S6K Signaling. The drugs Fentanyl and Adjuvants, Anesthesia have been mentioned in the context of this disorder. Affiliated tissues include kidney, brain and liver, and related phenotypes are Increased shRNA abundance (Z-score > 2) and cardiovascular system

Related Diseases for Congenital Mesoblastic Nephroma

Diseases in the Adult Mesoblastic Nephroma family:

Congenital Mesoblastic Nephroma

Diseases related to Congenital Mesoblastic Nephroma via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 85)
id Related Disease Score Top Affiliating Genes
1 fibrosarcoma 30.2 KIT SMARCB1
2 cellular congenital mesoblastic nephroma 12.3
3 classic congenital mesoblastic nephroma 12.3
4 patent ductus arteriosus and bicuspid aortic valve with hand anomalies 10.3 ETV6 NTRK3
5 intracystic papillary adenoma 10.3 ETV6 NTRK3
6 anterior cerebral artery infarction 10.3 ETV6 NTRK3
7 intestinal obstruction 10.3 ETV6 NTRK3
8 polyhydramnios 10.3
9 nasal cavity adenocarcinoma 10.2 ETV6 NTRK3
10 hemorrhagic fever 10.2 IGF2 WT1
11 intermixed schwannian stroma-rich ganglioneuroblastoma 10.2 IGF2 WT1
12 severe combined immunodeficiency, x-linked 10.2 IGF2 WT1
13 skin granular cell tumor 10.2 IGF2 WT1
14 plexopathy 10.2 SMARCB1 WT1
15 gallbladder pleomorphic giant cell adenocarcinoma 10.2 NRAS WT1
16 sick building syndrome 10.2 SMARCB1 WT1
17 choriocarcinoma 10.2 KIT WT1
18 agammaglobulinemia 8, autosomal dominant 10.2 ETV6 KIT
19 brain stem infarction 10.1 ETV6 NTRK3 WT1
20 kahrizi syndrome 10.1 KIT WT1
21 adducted thumbs dundar type 10.1 ETV6 KIT
22 liver angiosarcoma 10.1 KIT WT1
23 gastric leiomyosarcoma 10.1 IGF2 KIT
24 prostate leiomyosarcoma 10.1 IGF2 KIT
25 idiopathic cd4-positive t-lymphocytopenia 10.1 NRAS SMARCB1
26 uterine hypoplasia 10.1 IGF2 KIT
27 hyperandrogenism due to cortisone reductase deficiency 10.1 KIT WT1
28 larynx leiomyosarcoma 10.1 IGF2 KIT
29 priapism 10.1 KIT NTRK3
30 orofaciodigital syndrome x 10.1 KIT WT1
31 pancreatic cancer 10.1 IGF2 SMARCB1
32 small intestinal sarcoma 10.1 KIT WT1
33 renal hypertension 10.1
34 sarcoma 10.1
35 nephrocalcinosis 10.1
36 hydrops fetalis 10.1
37 temporal lobe neoplasm 10.1 IGF2 SMARCB1
38 parotid gland adenoid cystic carcinoma 10.1 KIT NRAS
39 cellular phase chronic idiopathic myelofibrosis 10.1 ETV6 NRAS NTRK3
40 acute disseminated encephalomyelitis 10.1 KIT NRAS
41 may-thurner syndrome 10.1 KIT NTRK3
42 cone dystrophy 10.1 KIT WT1
43 verrucous papilloma 10.1 KIT NRAS
44 non-distal monosomy 10q 10.1 ETV6 NRAS NTRK3
45 vulval paget's disease 10.1 KIT NRAS
46 cerebral arterial disease 10.1 KIT NRAS
47 lung mucoepidermoid carcinoma 10.1 KIT NRAS
48 fanconi renotubular syndrome 2 10.1 CTCF IGF2 WT1
49 diarrhea, chronic, with villous atrophy 10.0 IGF2 KIT
50 central nervous system sarcoma 10.0 KIT SMARCB1

Graphical network of the top 20 diseases related to Congenital Mesoblastic Nephroma:



Diseases related to Congenital Mesoblastic Nephroma

Symptoms & Phenotypes for Congenital Mesoblastic Nephroma

GenomeRNAi Phenotypes related to Congenital Mesoblastic Nephroma according to GeneCards Suite gene sharing:

26
id Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Increased shRNA abundance (Z-score > 2) GR00366-A-100 10.25 SMARCB1 WT1
2 Increased shRNA abundance (Z-score > 2) GR00366-A-102 10.25 KIT
3 Increased shRNA abundance (Z-score > 2) GR00366-A-11 10.25 SMARCB1
4 Increased shRNA abundance (Z-score > 2) GR00366-A-122 10.25 SMARCB1 WT1
5 Increased shRNA abundance (Z-score > 2) GR00366-A-130 10.25 NTRK3
6 Increased shRNA abundance (Z-score > 2) GR00366-A-137 10.25 NTRK3
7 Increased shRNA abundance (Z-score > 2) GR00366-A-152 10.25 NTRK3 WT1 KIT
8 Increased shRNA abundance (Z-score > 2) GR00366-A-157 10.25 KIT
9 Increased shRNA abundance (Z-score > 2) GR00366-A-170 10.25 KIT
10 Increased shRNA abundance (Z-score > 2) GR00366-A-176 10.25 SMARCB1
11 Increased shRNA abundance (Z-score > 2) GR00366-A-194 10.25 SMARCB1
12 Increased shRNA abundance (Z-score > 2) GR00366-A-214 10.25 WT1
13 Increased shRNA abundance (Z-score > 2) GR00366-A-23 10.25 WT1
14 Increased shRNA abundance (Z-score > 2) GR00366-A-30 10.25 NTRK3 WT1 KIT
15 Increased shRNA abundance (Z-score > 2) GR00366-A-35 10.25 WT1
16 Increased shRNA abundance (Z-score > 2) GR00366-A-41 10.25 NTRK3
17 Increased shRNA abundance (Z-score > 2) GR00366-A-42 10.25 NTRK3 KIT
18 Increased shRNA abundance (Z-score > 2) GR00366-A-43 10.25 WT1
19 Increased shRNA abundance (Z-score > 2) GR00366-A-47 10.25 WT1
20 Increased shRNA abundance (Z-score > 2) GR00366-A-83 10.25 SMARCB1
21 Increased shRNA abundance (Z-score > 2) GR00366-A-85 10.25 SMARCB1 WT1 KIT NTRK3
22 Increased shRNA abundance (Z-score > 2) GR00366-A-9 10.25 KIT
23 Increased shRNA abundance (Z-score > 2) GR00366-A-99 10.25 KIT

MGI Mouse Phenotypes related to Congenital Mesoblastic Nephroma:

44 (show all 13)
id Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 10.07 ETV6 IGF2 KIT NRAS NTRK3 SMARCB1
2 cellular MP:0005384 10.05 CTCF ETV6 IGF2 KIT NRAS SMARCB1
3 embryo MP:0005380 10.02 CTCF ETV6 IGF2 KIT NRAS SMARCB1
4 growth/size/body region MP:0005378 9.98 ETV6 IGF2 KIT NRAS NTRK3 SMARCB1
5 hematopoietic system MP:0005397 9.95 CTCF ETV6 IGF2 KIT NRAS SMARCB1
6 endocrine/exocrine gland MP:0005379 9.93 CTCF ETV6 IGF2 KIT NRAS WT1
7 immune system MP:0005387 9.91 CTCF ETV6 IGF2 KIT NRAS SMARCB1
8 digestive/alimentary MP:0005381 9.89 NRAS SMARCB1 ETV6 IGF2 KIT
9 mortality/aging MP:0010768 9.86 CTCF ETV6 IGF2 KIT NRAS NTRK3
10 liver/biliary system MP:0005370 9.72 IGF2 KIT NRAS SMARCB1 WT1
11 neoplasm MP:0002006 9.55 ETV6 KIT NRAS SMARCB1 WT1
12 no phenotypic analysis MP:0003012 9.43 NTRK3 WT1 ETV6 IGF2 KIT NRAS
13 vision/eye MP:0005391 9.02 IGF2 KIT NRAS NTRK3 SMARCB1

Drugs & Therapeutics for Congenital Mesoblastic Nephroma

Drugs for Congenital Mesoblastic Nephroma (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 26)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1
Fentanyl Approved, Illicit, Investigational, Vet_approved Phase 3 437-38-7 3345
2 Adjuvants, Anesthesia Phase 3
3 Analgesics Phase 3
4 Analgesics, Opioid Phase 3
5 Anesthetics Phase 3
6 Anesthetics, General Phase 3
7 Anesthetics, Intravenous Phase 3
8 Central Nervous System Depressants Phase 3
9 Liver Extracts Phase 3,Phase 1,Phase 2
10 Narcotics Phase 3
11 pancreatic polypeptide Phase 3
12 Peripheral Nervous System Agents Phase 3
13 Cola Nutraceutical Phase 3
14
Carboplatin Approved Phase 1, Phase 2 41575-94-4 10339178 498142 38904
15
Dasatinib Approved, Investigational Phase 1, Phase 2 302962-49-8 3062316
16
Etoposide Approved Phase 1, Phase 2 33419-42-0 36462
17
Ifosfamide Approved Phase 1, Phase 2 3778-73-2 3690
18
Mechlorethamine Approved Phase 1, Phase 2 51-75-2 4033
19 Alkylating Agents Phase 1, Phase 2
20 Antineoplastic Agents, Alkylating Phase 1, Phase 2
21 Antineoplastic Agents, Phytogenic Phase 1, Phase 2
22 Etoposide phosphate Phase 1, Phase 2
23 Isophosphamide mustard Phase 1, Phase 2
24 Protein Kinase Inhibitors Phase 1, Phase 2
25 Topoisomerase Inhibitors Phase 1, Phase 2
26 tyrosine Nutraceutical Phase 1

Interventional clinical trials:


id Name Status NCT ID Phase
1 Fentanyl Sublingual Spray in Treating Patients With Breakthrough Cancer Pain Completed NCT00538850 Phase 3
2 Oral TRK Inhibitor LOXO-101 (Larotrectinib) for Treatment of Advanced Pediatric Solid or Primary Central Nervous System Tumors Recruiting NCT02637687 Phase 1, Phase 2
3 Dasatinib, Ifosfamide, Carboplatin, and Etoposide in Treating Young Patients With Metastatic or Recurrent Malignant Solid Tumors Active, not recruiting NCT00788125 Phase 1, Phase 2
4 Study of RXDX-101 in Children With Recurrent or Refractory Solid Tumors and Primary CNS Tumors, With or Without TRK, ROS1, or ALK Fusions Recruiting NCT02650401 Phase 1
5 Study of Genes and Environment in Patients With Cancer in East Anglia, Trent, or West Midlands Regions of the United Kingdom Unknown status NCT00757614
6 Study of Kidney Tumors in Younger Patients Recruiting NCT00898365
7 Studying Biomarkers in Samples From Younger Patients With Kidney Cancer Withdrawn NCT01642095

Search NIH Clinical Center for Congenital Mesoblastic Nephroma

Cochrane evidence based reviews: nephroma, mesoblastic

Genetic Tests for Congenital Mesoblastic Nephroma

Anatomical Context for Congenital Mesoblastic Nephroma

MalaCards organs/tissues related to Congenital Mesoblastic Nephroma:

39
Kidney, Brain, Liver

Publications for Congenital Mesoblastic Nephroma

Articles related to Congenital Mesoblastic Nephroma:

(show top 50) (show all 143)
id Title Authors Year
1
The Value of Mitotic Count and Ki67 Proliferation Index in Congenital Mesoblastic Nephroma. ( 27484189 )
2016
2
Congenital Mesoblastic Nephroma Presenting With Hematuria in a Neonate: A Case Report. ( 26616096 )
2016
3
Congenital mesoblastic nephroma: a study of 19 cases using immunohistochemistry and ETV6-NTRK3 fusion gene rearrangement. ( 27020209 )
2016
4
Prenatal diagnosis of congenital mesoblastic nephroma. ( 26430667 )
2015
5
Congenital mesoblastic nephroma: Its diverse clinical features - A literature review with a case report. ( 26467634 )
2015
6
Mixed subtype of congenital mesoblastic nephroma with poor evolution: a case report and literature review. ( 26811558 )
2015
7
Congenital mesoblastic nephroma with focal anaplastic lesion. ( 25818639 )
2015
8
Congenital mesoblastic nephroma in a premature neonate: a case report and review of literature. ( 24553043 )
2014
9
Congenital mesoblastic nephroma: Clinical analysis of eight cases and a review of the literature. ( 25295083 )
2014
10
Maternal manifestation of Ballantyne's syndrome occurring concomitantly with the development of fetal congenital mesoblastic nephroma. ( 24428571 )
2014
11
Congenital mesoblastic nephroma: a rare pediatric neoplasm. ( 23354798 )
2013
12
Cytodiagnosis of congenital mesoblastic nephroma: a case report. ( 23417966 )
2013
13
Prenatal sonographic diagnosis of multicystic congenital mesoblastic nephroma. ( 23280386 )
2013
14
Congenital mesoblastic nephroma: A rare cause of recurrent hematuria beyond infancy. ( 23960358 )
2013
15
Prenatal diagnosis of congenital mesoblastic nephroma. ( 22354312 )
2013
16
AIRP best cases in radiologic-pathologic correlation: congenital mesoblastic nephroma. ( 22236896 )
2012
17
Congenital mesoblastic nephroma in a young basset hound dog. ( 23020172 )
2012
18
Parathyroid-hormone-related protein-mediated hypercalcemia in benign congenital mesoblastic nephroma. ( 21161280 )
2011
19
Intrauterine demise due to congenital mesoblastic nephroma in a fetus conceived by assisted reproductive technology. ( 21075369 )
2011
20
Cellular congenital mesoblastic nephroma: case report. ( 21541470 )
2011
21
Pathology in practice. Congenital mesoblastic nephroma. ( 21961632 )
2011
22
Regression of a congenital mesoblastic nephroma. ( 20582939 )
2010
23
Elevated levels of aldosterone in the amniotic fluid in two cases of congenital mesoblastic nephroma. ( 20455206 )
2010
24
Congenital mesoblastic nephroma. ( 20096866 )
2010
25
Imaging of congenital mesoblastic nephroma with pathological correlation. ( 19629463 )
2009
26
Pamidronate therapy for hypercalcemia and congenital mesoblastic nephroma: a case report. ( 20062638 )
2009
27
WT1 expression and hemihypertrophy in congenital mesoblastic nephroma. ( 19011477 )
2008
28
Congenital mesoblastic nephroma rich from mitosis after in vitro fertilization: a case report. ( 18485932 )
2008
29
Congenital mesoblastic nephroma: a case report. ( 23012173 )
2008
30
Duplication of the paternal IGF2 allele in trisomy 11 and elevated expression levels of IGF2 mRNA in congenital mesoblastic nephroma of the cellular or mixed type. ( 17639583 )
2007
31
Atypical congenital mesoblastic nephroma presenting in the perinatal period. ( 17093992 )
2007
32
A case of fetal congenital mesoblastic nephroma with oligohydramnios. ( 17449950 )
2007
33
Expression of ETV6-NTRK in classical, cellular and mixed subtypes of congenital mesoblastic nephroma. ( 16681692 )
2006
34
Congenital mesoblastic nephroma presenting with massive hematuria and hemorrhagic shock: report of one case. ( 17078466 )
2006
35
Congenital mesoblastic nephroma presenting with neonatal hypertension. ( 15858607 )
2005
36
Prenatal diagnosis of congenital mesoblastic nephroma associated with renal hypertension in a premature child. ( 15711357 )
2005
37
Prenatal diagnosis of congenital mesoblastic nephroma associated with renal hypertension in a premature child. ( 14678186 )
2004
38
Pathologic quiz case: renal mass in a 3-week-old infant. Congenital mesoblastic nephroma, mixed type. ( 15270602 )
2004
39
Cellular congenital mesoblastic nephroma with contralateral medullary nephrocalcinosis. ( 15121709 )
2004
40
Prenatal diagnosis of congenital mesoblastic nephroma in mid-second trimester by sonography and magnetic resonance imaging. ( 14634980 )
2003
41
Use of sarcoma-based chemotherapy in a case of congenital mesoblastic nephroma with liver metastases. ( 12809924 )
2003
42
Prenatal diagnosis of congenital mesoblastic nephroma in 2 siblings. ( 12901411 )
2003
43
Prenatal diagnosis of congenital mesoblastic nephroma. ( 11876819 )
2002
44
Multicystic congenital mesoblastic nephroma. ( 11927972 )
2002
45
Congenital mesoblastic nephroma associated with polyhydramnios and hypercalcemia. ( 11956858 )
2002
46
Complete response of recurrent cellular congenital mesoblastic nephroma to chemotherapy. ( 12218597 )
2002
47
Cryptic t(12;15)(p13;q26) producing the ETV6-NTRK3 fusion gene and no loss of IGF2 imprinting in congenital mesoblastic nephroma with trisomy 11: fluorescence in situ hybridization and IGF2 allelic expression analysis. ( 12165445 )
2002
48
Functional and gene expression analysis of the p53 signaling pathway in clear cell sarcoma of the kidney and congenital mesoblastic nephroma. ( 12007018 )
2002
49
Mixed epithelial and stromal tumor of the kidney lacks the genetic alterations of cellular congenital mesoblastic nephroma. ( 11381370 )
2001
50
ETV6 rearrangements in patients with infantile fibrosarcomas and congenital mesoblastic nephromas by fluorescence in situ hybridization. ( 11743047 )
2001

Variations for Congenital Mesoblastic Nephroma

Expression for Congenital Mesoblastic Nephroma

Search GEO for disease gene expression data for Congenital Mesoblastic Nephroma.

Pathways for Congenital Mesoblastic Nephroma

Pathways related to Congenital Mesoblastic Nephroma according to GeneCards Suite gene sharing:

id Super pathways Score Top Affiliating Genes
1
Show member pathways
12.45 IGF2 KIT NRAS NTRK3
2
Show member pathways
12.34 IGF2 KIT NRAS NTRK3
3 11.05 IGF2 KIT
4 10.61 KIT NRAS NTRK3
5
Show member pathways
10.37 IGF2 NRAS

GO Terms for Congenital Mesoblastic Nephroma

Biological processes related to Congenital Mesoblastic Nephroma according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 regulation of gene expression by genetic imprinting GO:0006349 8.62 CTCF IGF2

Sources for Congenital Mesoblastic Nephroma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 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 MedGen
42 MeSH
43 MESH via Orphanet
44 MGI
46 NCI
47 NCIt
48 NDF-RT
51 NINDS
52 Novoseek
54 OMIM
55 OMIM via Orphanet
59 PubMed
60 QIAGEN
65 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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