MCID: CNG029
MIFTS: 43

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 72 49 55 14 69
Nephroma, Mesoblastic 41

Classifications:

Orphanet: 55  
Rare renal diseases


External Ids:

Disease Ontology 12 DOID:4773
MeSH 41 D018201
NCIt 46 C6569
Orphanet 55 ORPHA2665
UMLS via Orphanet 70 C1332965
MESH via Orphanet 42 D018201
ICD10 via Orphanet 33 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 wilms tumor 5. 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 Increased shRNA abundance (Z-score > 2)

Wikipedia : 72 Congenital mesoblastic nephroma, while rare, is the most common kidney neoplasm diagnosed in the first... more...

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 87)
# Related Disease Score Top Affiliating Genes
1 cellular congenital mesoblastic nephroma 33.8 ETV6 NRAS NTRK3
2 wilms tumor 5 30.7 IGF2 WT1
3 hemihyperplasia, isolated 30.6 IGF2 WT1
4 hereditary wilms' tumor 30.5 IGF2 WT1
5 clear cell sarcoma 29.9 IGF2 KIT WT1
6 beckwith-wiedemann syndrome 29.5 CTCF IGF2 SMARCB1 WT1
7 classic congenital mesoblastic nephroma 12.4
8 hypercalcemia, infantile, 1 10.4
9 adult mesoblastic nephroma 10.4 ETV6 NTRK3
10 pediatric fibrosarcoma 10.4 ETV6 NTRK3
11 congenital fibrosarcoma 10.4 ETV6 NTRK3
12 wilms tumor 6 10.4
13 breast secretory carcinoma 10.4 ETV6 NTRK3
14 polyhydramnios 10.3
15 iris disease 10.3 IGF2 WT1
16 fibrosarcoma 10.3
17 wilms tumor, aniridia, genitourinary anomalies, and mental retardation syndrome 10.3 IGF2 WT1
18 melanomatosis 10.2 NRAS WT1
19 kidney rhabdoid cancer 10.2 SMARCB1 WT1
20 congenital heart defects, hamartomas of tongue, and polysyndactyly 10.2
21 hydrops fetalis, nonimmune, and/or atrial septal defect 10.2
22 renal hypertension 10.2
23 sarcoma 10.2
24 nephrocalcinosis 10.2
25 hydrops fetalis 10.2
26 acute myeloid leukemia with abnormal bone marrow eosinophils inv(16)(p13q22) or t(16;16)(p13;q22) 10.2 ETV6 KIT
27 testicular gonadoblastoma 10.2 KIT WT1
28 adult fibrosarcoma 10.2 ETV6 NTRK3 WT1
29 hypereosinophilic syndrome, idiopathic 10.2 KIT WT1
30 small intestine leiomyosarcoma 10.2 IGF2 KIT
31 small intestinal sarcoma 10.2 IGF2 KIT
32 cystic nephroma 10.2 KIT WT1
33 heart sarcoma 10.1 KIT WT1
34 gastric leiomyosarcoma 10.1 IGF2 KIT
35 chronic eosinophilic leukemia 10.1 KIT WT1
36 polymorphous low-grade adenocarcinoma 10.1 KIT NTRK3
37 wilson-turner x-linked mental retardation syndrome 10.1 IGF2 WT1
38 mast-cell leukemia 10.1 KIT NTRK3
39 dysgerminoma of ovary 10.1 KIT WT1
40 chondrosarcoma, extraskeletal myxoid 10.1 SMARCB1 WT1
41 small cell sarcoma 10.1 NRAS SMARCB1
42 infratentorial cancer 10.1 NTRK3 SMARCB1
43 gonadoblastoma 10.1 KIT WT1
44 mast cell neoplasm 10.1 KIT NTRK3
45 ovarian melanoma 10.1 KIT NRAS
46 malignant spindle cell melanoma 10.1 KIT NRAS
47 conventional fibrosarcoma 10.1 KIT NRAS
48 acral lentiginous melanoma 10.0 KIT NRAS
49 aging 10.0
50 disseminated intravascular coagulation 10.0

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:

25 (show all 23)
# 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 KIT WT1
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 KIT WT1
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 KIT NTRK3
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 NTRK3 KIT SMARCB1 WT1
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:

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

Drugs & Therapeutics for Congenital Mesoblastic Nephroma

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

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

Interventional clinical trials:


# 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 Phase 1/2 Study of LOXO-195 in Patients With Previously Treated NTRK Fusion Cancers Recruiting NCT03215511 Phase 1, Phase 2 LOXO-195
3 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)
4 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
5 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
6 Study of Genes and Environment in Patients With Cancer in East Anglia, Trent, or West Midlands Regions of the United Kingdom Unknown status NCT00757614
7 Study of Kidney Tumors in Younger Patients Recruiting NCT00898365
8 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:

38
Kidney, Liver, Brain, Testes

Publications for Congenital Mesoblastic Nephroma

Articles related to Congenital Mesoblastic Nephroma:

(show top 50) (show all 151)
# Title Authors Year
1
ETV6-NTRK3 in congenital mesoblastic nephroma: A report of the SIOP/GPOH nephroblastoma study. ( 29286563 )
2018
2
Congenital mesoblastic nephroma 50 years after its recognition: A narrative review. ( 28124468 )
2017
3
Management of mixed type congenital mesoblastic nephroma: Case series and review of the literature. ( 28282819 )
2017
4
Cellular Congenital Mesoblastic Nephroma in a Newborn. ( 28770142 )
2017
5
Recurrent and metastatic congenital mesoblastic nephroma: where does the evidence stand? ( 28856451 )
2017
6
Congenital Mesoblastic Nephroma Presenting With Refractory Hypertension in a Premature Neonate: A Case Study. ( 28137351 )
2017
7
Targeting NTRK fusions for the treatment of congenital mesoblastic nephroma. ( 28440051 )
2017
8
Recurrent EML4-NTRK3 fusions in infantile fibrosarcoma and congenital mesoblastic nephroma suggest a revised testing strategy. ( 29099503 )
2017
9
Congenital mesoblastic nephroma: a study of 19 cases using immunohistochemistry and ETV6-NTRK3 fusion gene rearrangement. ( 27020209 )
2016
10
The Value of Mitotic Count and Ki67 Proliferation Index in Congenital Mesoblastic Nephroma. ( 27484189 )
2016
11
Congenital Mesoblastic Nephroma Presenting With Hematuria in a Neonate: A Case Report. ( 26616096 )
2016
12
Prenatal diagnosis of congenital mesoblastic nephroma. ( 26430667 )
2015
13
Congenital mesoblastic nephroma with focal anaplastic lesion. ( 25818639 )
2015
14
Congenital mesoblastic nephroma: Its diverse clinical features - A literature review with a case report. ( 26467634 )
2015
15
Mixed subtype of congenital mesoblastic nephroma with poor evolution: a case report and literature review. ( 26811558 )
2015
16
Maternal manifestation of Ballantyne's syndrome occurring concomitantly with the development of fetal congenital mesoblastic nephroma. ( 24428571 )
2014
17
Congenital mesoblastic nephroma: Clinical analysis of eight cases and a review of the literature. ( 25295083 )
2014
18
Congenital mesoblastic nephroma in a premature neonate: a case report and review of literature. ( 24553043 )
2014
19
Congenital mesoblastic nephroma: A rare cause of recurrent hematuria beyond infancy. ( 23960358 )
2013
20
Congenital mesoblastic nephroma: a rare pediatric neoplasm. ( 23354798 )
2013
21
Prenatal sonographic diagnosis of multicystic congenital mesoblastic nephroma. ( 23280386 )
2013
22
Cytodiagnosis of congenital mesoblastic nephroma: a case report. ( 23417966 )
2013
23
Prenatal diagnosis of congenital mesoblastic nephroma. ( 22354312 )
2013
24
AIRP best cases in radiologic-pathologic correlation: congenital mesoblastic nephroma. ( 22236896 )
2012
25
Congenital mesoblastic nephroma in a young basset hound dog. ( 23020172 )
2012
26
Parathyroid-hormone-related protein-mediated hypercalcemia in benign congenital mesoblastic nephroma. ( 21161280 )
2011
27
Cellular congenital mesoblastic nephroma: case report. ( 21541470 )
2011
28
Intrauterine demise due to congenital mesoblastic nephroma in a fetus conceived by assisted reproductive technology. ( 21075369 )
2011
29
Pathology in practice. Congenital mesoblastic nephroma. ( 21961632 )
2011
30
Regression of a congenital mesoblastic nephroma. ( 20582939 )
2010
31
Elevated levels of aldosterone in the amniotic fluid in two cases of congenital mesoblastic nephroma. ( 20455206 )
2010
32
Congenital mesoblastic nephroma. ( 20096866 )
2010
33
Imaging of congenital mesoblastic nephroma with pathological correlation. ( 19629463 )
2009
34
Pamidronate therapy for hypercalcemia and congenital mesoblastic nephroma: a case report. ( 20062638 )
2009
35
WT1 expression and hemihypertrophy in congenital mesoblastic nephroma. ( 19011477 )
2008
36
Congenital mesoblastic nephroma rich from mitosis after in vitro fertilization: a case report. ( 18485932 )
2008
37
Congenital mesoblastic nephroma: a case report. ( 23012173 )
2008
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
Atypical congenital mesoblastic nephroma presenting in the perinatal period. ( 17093992 )
2007
40
A case of fetal congenital mesoblastic nephroma with oligohydramnios. ( 17449950 )
2007
41
Congenital mesoblastic nephroma presenting with massive hematuria and hemorrhagic shock: report of one case. ( 17078466 )
2006
42
Expression of ETV6-NTRK in classical, cellular and mixed subtypes of congenital mesoblastic nephroma. ( 16681692 )
2006
43
Prenatal diagnosis of congenital mesoblastic nephroma associated with renal hypertension in a premature child. ( 15711357 )
2005
44
Congenital mesoblastic nephroma presenting with neonatal hypertension. ( 15858607 )
2005
45
Pathologic quiz case: renal mass in a 3-week-old infant. Congenital mesoblastic nephroma, mixed type. ( 15270602 )
2004
46
Cellular congenital mesoblastic nephroma with contralateral medullary nephrocalcinosis. ( 15121709 )
2004
47
Prenatal diagnosis of congenital mesoblastic nephroma associated with renal hypertension in a premature child. ( 14678186 )
2004
48
Prenatal diagnosis of congenital mesoblastic nephroma in 2 siblings. ( 12901411 )
2003
49
Prenatal diagnosis of congenital mesoblastic nephroma in mid-second trimester by sonography and magnetic resonance imaging. ( 14634980 )
2003
50
Use of sarcoma-based chemotherapy in a case of congenital mesoblastic nephroma with liver metastases. ( 12809924 )
2003

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:

# Super pathways Score Top Affiliating Genes
1
Show member pathways
12.51 IGF2 KIT NRAS NTRK3
2
Show member pathways
12.44 IGF2 KIT NRAS NTRK3
3
Show member pathways
11.92 IGF2 KIT NRAS SMARCB1
4 11.09 IGF2 KIT
5 10.61 KIT NRAS NTRK3
6
Show member pathways
10.57 IGF2 NRAS

GO Terms for Congenital Mesoblastic Nephroma

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

# Name GO ID Score Top Affiliating Genes
1 negative regulation of transcription by RNA polymerase II GO:0000122 9.26 CTCF ETV6 IGF2 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:

# 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
27 GO
28 GTR
29 HGMD
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 MedGen
41 MeSH
42 MESH via Orphanet
43 MGI
45 NCI
46 NCIt
47 NDF-RT
50 NINDS
51 Novoseek
53 OMIM
54 OMIM via Orphanet
58 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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