MCID: CNG029
MIFTS: 38

Congenital Mesoblastic Nephroma

Categories: Rare diseases, Nephrological diseases, Cancer diseases

Aliases & Classifications for Congenital Mesoblastic Nephroma

MalaCards integrated aliases 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
UMLS via Orphanet 70 C1332965
MESH via Orphanet 43 D018201
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 cellular congenital mesoblastic nephroma and classic 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 Pancreatic Polypeptide have been mentioned in the context of this disorder. Affiliated tissues include kidney, liver and brain, 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 81)
id Related Disease Score Top Affiliating Genes
1 cellular congenital mesoblastic nephroma 12.3
2 classic congenital mesoblastic nephroma 12.2
3 ceroid lipofuscinosis, neuronal, 9 10.6 ETV6 NTRK3
4 intracystic papillary adenoma 10.6 ETV6 NTRK3
5 cerebral arterial disease 10.6 ETV6 NTRK3
6 follicular infundibulum tumor 10.5 ETV6 NTRK3
7 klatskin's tumor 10.5 ETV6 NTRK3
8 hemoglobin zurich 10.4 IGF2 WT1
9 cervical wilms' tumor 10.4 IGF2 WT1
10 skin granular cell tumor 10.3 IGF2 WT1
11 colon signet ring adenocarcinoma 10.3 ETV6 IGF2
12 polyhydramnios 10.3
13 fibrous meningioma 10.3 NRAS WT1
14 lateral medullary syndrome 10.2 ETV6 NTRK3 WT1
15 menkes disease 10.2 IGF2 WT1
16 renal hypertension 10.1
17 sarcoma 10.1
18 nephrocalcinosis 10.1
19 fibrosarcoma 10.1
20 hydrops fetalis 10.1
21 acute pre-b-cell lymphoblastic leukemia 10.1 ETV6 KIT
22 epithelioid trophoblastic tumor 10.1 KIT WT1
23 acute necrotizing encephalopathy 10.1 ETV6 KIT
24 wilms tumor 6 10.0 KIT WT1
25 malignant dermis tumor 10.0 IGF2 KIT
26 gallbladder leiomyosarcoma 10.0 IGF2 KIT
27 liver angiosarcoma 10.0 KIT WT1
28 retroperitoneal leiomyosarcoma 10.0 IGF2 KIT
29 lung leiomyosarcoma 10.0 KIT WT1
30 cutaneous liposarcoma 10.0 KIT WT1
31 maxillary sinus schneiderian papilloma 10.0 ETV6 NRAS NTRK3
32 cerebrum cancer 10.0 IGF2 WT1
33 alopecia antibody deficiency 10.0 KIT WT1
34 pseudounicornuate uterus 10.0 IGF2 KIT
35 histidine metabolism disease 10.0 KIT NTRK3
36 non-distal trisomy 10q 10.0 ETV6 NRAS NTRK3
37 beckwith-wiedemann syndrome 10.0
38 disseminated intravascular coagulation 10.0
39 oligohydramnios 10.0
40 hypertrophic pyloric stenosis 10.0
41 pyloric stenosis 10.0
42 leiomyoma 10.0
43 clear cell sarcoma 10.0
44 hemihypertrophy 10.0
45 polyosteolysis/hyperostosis syndrome 9.9 KIT NTRK3
46 nasal cavity squamous cell carcinoma 9.9 KIT WT1
47 nephrolithiasis/osteoporosis, hypophosphatemic, 1 9.9 CTCF IGF2 WT1
48 hyperbiliverdinemia 9.9 CTCF IGF2 WT1
49 vagina, absence of 9.9 KIT WT1
50 hydronephrosis, congenital, with cleft palate, characteristic facies, hypotonia, and mental retardation 9.9 KIT WT1

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 10.04 WT1
2 Increased shRNA abundance (Z-score > 2) GR00366-A-102 10.04 KIT
3 Increased shRNA abundance (Z-score > 2) GR00366-A-122 10.04 WT1
4 Increased shRNA abundance (Z-score > 2) GR00366-A-130 10.04 NTRK3
5 Increased shRNA abundance (Z-score > 2) GR00366-A-137 10.04 NTRK3
6 Increased shRNA abundance (Z-score > 2) GR00366-A-152 10.04 KIT NTRK3 WT1
7 Increased shRNA abundance (Z-score > 2) GR00366-A-157 10.04 KIT
8 Increased shRNA abundance (Z-score > 2) GR00366-A-170 10.04 KIT
9 Increased shRNA abundance (Z-score > 2) GR00366-A-214 10.04 WT1
10 Increased shRNA abundance (Z-score > 2) GR00366-A-23 10.04 WT1
11 Increased shRNA abundance (Z-score > 2) GR00366-A-30 10.04 KIT NTRK3 WT1
12 Increased shRNA abundance (Z-score > 2) GR00366-A-35 10.04 WT1
13 Increased shRNA abundance (Z-score > 2) GR00366-A-41 10.04 NTRK3
14 Increased shRNA abundance (Z-score > 2) GR00366-A-42 10.04 KIT NTRK3
15 Increased shRNA abundance (Z-score > 2) GR00366-A-43 10.04 WT1
16 Increased shRNA abundance (Z-score > 2) GR00366-A-47 10.04 WT1
17 Increased shRNA abundance (Z-score > 2) GR00366-A-85 10.04 KIT NTRK3 WT1
18 Increased shRNA abundance (Z-score > 2) GR00366-A-9 10.04 KIT
19 Increased shRNA abundance (Z-score > 2) GR00366-A-99 10.04 KIT
20 Decreased shRNA abundance (Z-score < -2) GR00366-A-109 9.86 NTRK3 IGF2
21 Decreased shRNA abundance (Z-score < -2) GR00366-A-134 9.86 IGF2
22 Decreased shRNA abundance (Z-score < -2) GR00366-A-148 9.86 NRAS NTRK3 IGF2
23 Decreased shRNA abundance (Z-score < -2) GR00366-A-184 9.86 NTRK3
24 Decreased shRNA abundance (Z-score < -2) GR00366-A-195 9.86 IGF2
25 Decreased shRNA abundance (Z-score < -2) GR00366-A-204 9.86 NRAS
26 Decreased shRNA abundance (Z-score < -2) GR00366-A-28 9.86 NRAS
27 Decreased shRNA abundance (Z-score < -2) GR00366-A-55 9.86 NTRK3
28 Decreased shRNA abundance (Z-score < -2) GR00366-A-60 9.86 NTRK3
29 Decreased shRNA abundance (Z-score < -2) GR00366-A-66 9.86 IGF2
30 Decreased shRNA abundance (Z-score < -2) GR00366-A-72 9.86 NRAS
31 Decreased shRNA abundance (Z-score < -2) GR00366-A-87 9.86 IGF2
32 Decreased shRNA abundance (Z-score < -2) GR00366-A-91 9.86 IGF2

MGI Mouse Phenotypes related to Congenital Mesoblastic Nephroma:

44
id Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 9.93 ETV6 IGF2 KIT NRAS NTRK3 WT1
2 embryo MP:0005380 9.91 CTCF ETV6 IGF2 KIT NRAS WT1
3 endocrine/exocrine gland MP:0005379 9.88 IGF2 KIT NRAS WT1 CTCF ETV6
4 hematopoietic system MP:0005397 9.85 CTCF ETV6 IGF2 KIT NRAS WT1
5 immune system MP:0005387 9.8 CTCF ETV6 IGF2 KIT NRAS WT1
6 mortality/aging MP:0010768 9.8 CTCF ETV6 IGF2 KIT NRAS NTRK3
7 neoplasm MP:0002006 9.46 KIT NRAS WT1 ETV6
8 no phenotypic analysis MP:0003012 9.43 ETV6 IGF2 KIT NRAS NTRK3 WT1
9 normal MP:0002873 9.02 ETV6 KIT NRAS NTRK3 WT1

Drugs & Therapeutics for Congenital Mesoblastic Nephroma

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

(show all 25)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1
Fentanyl Approved, Illicit, Investigational, Vet_approved Phase 3 437-38-7 3345
2 Pancreatic Polypeptide Investigational Phase 3 59763-91-6
3 Adjuvants, Anesthesia Phase 3
4 Analgesics Phase 3
5 Analgesics, Opioid Phase 3
6 Anesthetics Phase 3
7 Anesthetics, General Phase 3
8 Anesthetics, Intravenous Phase 3
9 Central Nervous System Depressants Phase 3
10 Liver Extracts Phase 3,Phase 1,Phase 2
11 Narcotics 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, Phytogenic Phase 1, Phase 2
21 Etoposide phosphate Phase 1, Phase 2
22 Isophosphamide mustard Phase 1, Phase 2
23 Protein Kinase Inhibitors Phase 1, Phase 2
24 Topoisomerase Inhibitors Phase 1, Phase 2
25 tyrosine Nutraceutical Phase 1

Interventional clinical trials:


id Name Status NCT ID Phase Drugs
1 Fentanyl Sublingual Spray in Treating Patients With Breakthrough Cancer Pain Completed NCT00538850 Phase 3 Fentanyl sublingual spray;Placebo
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 LOXO-101 (larotrectinib)
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 carboplatin;dasatinib;etoposide phosphate;ifosfamide
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 Entrectinib
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, Liver, Brain

Publications for Congenital Mesoblastic Nephroma

Articles related to Congenital Mesoblastic Nephroma:

(show top 50) (show all 149)
id Title Authors Year
1
Targeting NTRK fusions for the treatment of congenital mesoblastic nephroma. ( 28440051 )
2017
2
Management of mixed type congenital mesoblastic nephroma: Case series and review of the literature. ( 28282819 )
2017
3
Congenital mesoblastic nephroma 50 years after its recognition: A narrative review. ( 28124468 )
2017
4
Congenital Mesoblastic Nephroma Presenting With Refractory Hypertension in a Premature Neonate: A Case Study. ( 28137351 )
2017
5
Cellular Congenital Mesoblastic Nephroma in a Newborn. ( 28770142 )
2017
6
Recurrent and metastatic congenital mesoblastic nephroma: where does the evidence stand? ( 28856451 )
2017
7
Congenital mesoblastic nephroma: a study of 19 cases using immunohistochemistry and ETV6-NTRK3 fusion gene rearrangement. ( 27020209 )
2016
8
Congenital Mesoblastic Nephroma Presenting With Hematuria in a Neonate: A Case Report. ( 26616096 )
2016
9
The Value of Mitotic Count and Ki67 Proliferation Index in Congenital Mesoblastic Nephroma. ( 27484189 )
2016
10
Mixed subtype of congenital mesoblastic nephroma with poor evolution: a case report and literature review. ( 26811558 )
2015
11
Congenital mesoblastic nephroma with focal anaplastic lesion. ( 25818639 )
2015
12
Congenital mesoblastic nephroma: Its diverse clinical features - A literature review with a case report. ( 26467634 )
2015
13
Prenatal diagnosis of congenital mesoblastic nephroma. ( 26430667 )
2015
14
Congenital mesoblastic nephroma in a premature neonate: a case report and review of literature. ( 24553043 )
2014
15
Congenital mesoblastic nephroma: Clinical analysis of eight cases and a review of the literature. ( 25295083 )
2014
16
Maternal manifestation of Ballantyne's syndrome occurring concomitantly with the development of fetal congenital mesoblastic nephroma. ( 24428571 )
2014
17
Congenital mesoblastic nephroma: a rare pediatric neoplasm. ( 23354798 )
2013
18
Congenital mesoblastic nephroma: A rare cause of recurrent hematuria beyond infancy. ( 23960358 )
2013
19
Cytodiagnosis of congenital mesoblastic nephroma: a case report. ( 23417966 )
2013
20
Prenatal sonographic diagnosis of multicystic congenital mesoblastic nephroma. ( 23280386 )
2013
21
Prenatal diagnosis of congenital mesoblastic nephroma. ( 22354312 )
2013
22
AIRP best cases in radiologic-pathologic correlation: congenital mesoblastic nephroma. ( 22236896 )
2012
23
Congenital mesoblastic nephroma in a young basset hound dog. ( 23020172 )
2012
24
Pathology in practice. Congenital mesoblastic nephroma. ( 21961632 )
2011
25
Intrauterine demise due to congenital mesoblastic nephroma in a fetus conceived by assisted reproductive technology. ( 21075369 )
2011
26
Cellular congenital mesoblastic nephroma: case report. ( 21541470 )
2011
27
Parathyroid-hormone-related protein-mediated hypercalcemia in benign congenital mesoblastic nephroma. ( 21161280 )
2011
28
Congenital mesoblastic nephroma. ( 20096866 )
2010
29
Regression of a congenital mesoblastic nephroma. ( 20582939 )
2010
30
Elevated levels of aldosterone in the amniotic fluid in two cases of congenital mesoblastic nephroma. ( 20455206 )
2010
31
Imaging of congenital mesoblastic nephroma with pathological correlation. ( 19629463 )
2009
32
Pamidronate therapy for hypercalcemia and congenital mesoblastic nephroma: a case report. ( 20062638 )
2009
33
Congenital mesoblastic nephroma: a case report. ( 23012173 )
2008
34
Congenital mesoblastic nephroma rich from mitosis after in vitro fertilization: a case report. ( 18485932 )
2008
35
WT1 expression and hemihypertrophy in congenital mesoblastic nephroma. ( 19011477 )
2008
36
Atypical congenital mesoblastic nephroma presenting in the perinatal period. ( 17093992 )
2007
37
A case of fetal congenital mesoblastic nephroma with oligohydramnios. ( 17449950 )
2007
38
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
39
Expression of ETV6-NTRK in classical, cellular and mixed subtypes of congenital mesoblastic nephroma. ( 16681692 )
2006
40
Congenital mesoblastic nephroma presenting with massive hematuria and hemorrhagic shock: report of one case. ( 17078466 )
2006
41
Congenital mesoblastic nephroma presenting with neonatal hypertension. ( 15858607 )
2005
42
Prenatal diagnosis of congenital mesoblastic nephroma associated with renal hypertension in a premature child. ( 15711357 )
2005
43
Cellular congenital mesoblastic nephroma with contralateral medullary nephrocalcinosis. ( 15121709 )
2004
44
Pathologic quiz case: renal mass in a 3-week-old infant. Congenital mesoblastic nephroma, mixed type. ( 15270602 )
2004
45
Prenatal diagnosis of congenital mesoblastic nephroma associated with renal hypertension in a premature child. ( 14678186 )
2004
46
Prenatal diagnosis of congenital mesoblastic nephroma in mid-second trimester by sonography and magnetic resonance imaging. ( 14634980 )
2003
47
Use of sarcoma-based chemotherapy in a case of congenital mesoblastic nephroma with liver metastases. ( 12809924 )
2003
48
Prenatal diagnosis of congenital mesoblastic nephroma in 2 siblings. ( 12901411 )
2003
49
Complete response of recurrent cellular congenital mesoblastic nephroma to chemotherapy. ( 12218597 )
2002
50
Functional and gene expression analysis of the p53 signaling pathway in clear cell sarcoma of the kidney and congenital mesoblastic nephroma. ( 12007018 )
2002

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 11.2 CTCF IGF2
4 11.19 IGF2 NRAS NTRK3
5 11.09 IGF2 KIT
6 10.61 KIT NRAS NTRK3
7
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 negative regulation of transcription from RNA polymerase II promoter GO:0000122 9.13 CTCF ETV6 WT1
2 regulation of gene expression by genetic imprinting GO:0006349 8.62 CTCF IGF2

Molecular functions related to Congenital Mesoblastic Nephroma according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 transmembrane receptor protein tyrosine kinase activity GO:0004714 8.62 KIT NTRK3

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 HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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