MCID: CNG029
MIFTS: 42

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 76 53 59 15 73
Nephroma, Mesoblastic 44

Classifications:

Orphanet: 59  
Rare renal diseases


External Ids:

Disease Ontology 12 DOID:4773
MeSH 44 D018201
NCIt 50 C6569
Orphanet 59 ORPHA2665
MESH via Orphanet 45 D018201
UMLS via Orphanet 74 C1332965
ICD10 via Orphanet 34 D41.0
UMLS 73 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 : 76 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 33.7 ETV6 NRAS NTRK3
2 wilms tumor 5 30.8 IGF2 WT1
3 beckwith-wiedemann syndrome 30.0 CTCF IGF2 WT1
4 clear cell sarcoma 29.5 IGF2 KIT WT1
5 leiomyoma 29.2 IGF2 KIT MKI67
6 classic congenital mesoblastic nephroma 12.4
7 adult mesoblastic nephroma 10.7 ETV6 NTRK3
8 pediatric fibrosarcoma 10.7 ETV6 NTRK3
9 congenital fibrosarcoma 10.6 ETV6 NTRK3
10 breast secretory carcinoma 10.6 ETV6 NTRK3
11 hemihyperplasia, isolated 10.5 IGF2 WT1
12 acinar cell carcinoma 10.5 ETV6 NTRK3
13 iris disease 10.5 IGF2 WT1
14 hereditary wilms' tumor 10.5 IGF2 WT1
15 rhabdoid cancer 10.4 IGF2 WT1
16 fibrosarcoma 10.4
17 polyhydramnios 10.4
18 wilms tumor, aniridia, genitourinary anomalies, and mental retardation syndrome 10.3 IGF2 WT1
19 wilms tumor 6 10.3
20 adult fibrosarcoma 10.3 ETV6 NTRK3 WT1
21 melanomatosis 10.3 NRAS WT1
22 hydrops fetalis, nonimmune, and/or atrial septal defect 10.2
23 renal hypertension 10.2
24 sarcoma 10.2
25 nephrocalcinosis 10.2
26 fetal edema 10.2
27 hydrops fetalis 10.2
28 wilson-turner x-linked mental retardation syndrome 10.2 IGF2 WT1
29 testicular gonadoblastoma 10.1 KIT WT1
30 acute myeloid leukemia with abnormal bone marrow eosinophils inv(16)(p13q22) or t(16;16)(p13;q22) 10.1 ETV6 KIT
31 aging 10.0
32 disseminated intravascular coagulation 10.0
33 oligohydramnios 10.0
34 von willebrand's disease 10.0
35 hypertrophic pyloric stenosis 10.0
36 pyloric stenosis 10.0
37 small intestine leiomyosarcoma 10.0 IGF2 KIT
38 small intestinal sarcoma 10.0 IGF2 KIT
39 hypereosinophilic syndrome, idiopathic 10.0 KIT WT1
40 cystic nephroma 10.0 KIT WT1
41 gastric leiomyosarcoma 10.0 IGF2 KIT
42 spindle cell liposarcoma 10.0 KIT WT1
43 heart sarcoma 10.0 KIT WT1
44 chronic eosinophilic leukemia 10.0 KIT WT1
45 wilms tumor 1 10.0 CTCF IGF2 WT1
46 differentiated thyroid carcinoma 10.0 ETV6 NRAS NTRK3
47 mast-cell leukemia 9.9 KIT NTRK3
48 dysgerminoma of ovary 9.9 KIT WT1
49 gonadoblastoma 9.9 KIT WT1
50 mast cell neoplasm 9.9 KIT NTRK3

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 (show all 19)
# 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 WT1 NTRK3
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 WT1 KIT NTRK3
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 NTRK3 KIT
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 WT1 NTRK3
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

MGI Mouse Phenotypes related to Congenital Mesoblastic Nephroma:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 9.8 NRAS NTRK3 WT1 ETV6 IGF2 KIT
2 embryo MP:0005380 9.73 NRAS WT1 CTCF ETV6 IGF2 KIT
3 endocrine/exocrine gland MP:0005379 9.63 NRAS WT1 CTCF ETV6 IGF2 KIT
4 no phenotypic analysis MP:0003012 9.5 MKI67 NRAS NTRK3 WT1 ETV6 IGF2
5 normal MP:0002873 9.1 MKI67 NRAS NTRK3 WT1 ETV6 KIT

Drugs & Therapeutics for Congenital Mesoblastic Nephroma

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

(show all 26)
# 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
Dasatinib Approved, Investigational Phase 1, Phase 2 302962-49-8 3062316
16
Carboplatin Approved Phase 1, Phase 2 41575-94-4 10339178 498142 38904
17
Mechlorethamine Approved, Investigational Phase 1, Phase 2 51-75-2 4033
18
Ifosfamide Approved Phase 1, Phase 2 3778-73-2 3690
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, Alkylating Phase 1, Phase 2
25 Antineoplastic Agents, Phytogenic Phase 1, Phase 2
26 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:

41
Kidney, Liver, Brain, Bone, Testes

Publications for Congenital Mesoblastic Nephroma

Articles related to Congenital Mesoblastic Nephroma:

(show top 50) (show all 154)
# Title Authors Year
1
Dramatic bone remodeling following larotrectinib administration for bone metastasis in a patient with TRK fusion congenital mesoblastic nephroma. ( 29893456 )
2018
2
ETV6-NTRK3 in congenital mesoblastic nephroma: A report of the SIOP/GPOH nephroblastoma study. ( 29286563 )
2018
3
Fetal biparametric MR imaging in the diagnosis of congenital mesoblastic nephroma. ( 29733803 )
2018
4
Congenital mesoblastic nephroma 50 years after its recognition: A narrative review. ( 28124468 )
2017
5
Cellular Congenital Mesoblastic Nephroma in a Newborn. ( 28770142 )
2017
6
Congenital Mesoblastic Nephroma Presenting With Refractory Hypertension in a Premature Neonate: A Case Study. ( 28137351 )
2017
7
Management of mixed type congenital mesoblastic nephroma: Case series and review of the literature. ( 28282819 )
2017
8
Targeting NTRK fusions for the treatment of congenital mesoblastic nephroma. ( 28440051 )
2017
9
Recurrent EML4-NTRK3 fusions in infantile fibrosarcoma and congenital mesoblastic nephroma suggest a revised testing strategy. ( 29099503 )
2017
10
Recurrent and metastatic congenital mesoblastic nephroma: where does the evidence stand? ( 28856451 )
2017
11
Congenital Mesoblastic Nephroma Presenting With Hematuria in a Neonate: A Case Report. ( 26616096 )
2016
12
Congenital mesoblastic nephroma: a study of 19 cases using immunohistochemistry and ETV6-NTRK3 fusion gene rearrangement. ( 27020209 )
2016
13
The Value of Mitotic Count and Ki67 Proliferation Index in Congenital Mesoblastic Nephroma. ( 27484189 )
2016
14
Congenital mesoblastic nephroma: Its diverse clinical features - A literature review with a case report. ( 26467634 )
2015
15
Prenatal diagnosis of congenital mesoblastic nephroma. ( 26430667 )
2015
16
Congenital mesoblastic nephroma with focal anaplastic lesion. ( 25818639 )
2015
17
Mixed subtype of congenital mesoblastic nephroma with poor evolution: a case report and literature review. ( 26811558 )
2015
18
Congenital mesoblastic nephroma: Clinical analysis of eight cases and a review of the literature. ( 25295083 )
2014
19
Maternal manifestation of Ballantyne's syndrome occurring concomitantly with the development of fetal congenital mesoblastic nephroma. ( 24428571 )
2014
20
Congenital mesoblastic nephroma in a premature neonate: a case report and review of literature. ( 24553043 )
2014
21
Cytodiagnosis of congenital mesoblastic nephroma: a case report. ( 23417966 )
2013
22
Congenital mesoblastic nephroma: A rare cause of recurrent hematuria beyond infancy. ( 23960358 )
2013
23
Prenatal sonographic diagnosis of multicystic congenital mesoblastic nephroma. ( 23280386 )
2013
24
Prenatal diagnosis of congenital mesoblastic nephroma. ( 22354312 )
2013
25
Congenital mesoblastic nephroma: a rare pediatric neoplasm. ( 23354798 )
2013
26
AIRP best cases in radiologic-pathologic correlation: congenital mesoblastic nephroma. ( 22236896 )
2012
27
Congenital mesoblastic nephroma in a young basset hound dog. ( 23020172 )
2012
28
Intrauterine demise due to congenital mesoblastic nephroma in a fetus conceived by assisted reproductive technology. ( 21075369 )
2011
29
Cellular congenital mesoblastic nephroma: case report. ( 21541470 )
2011
30
Parathyroid-hormone-related protein-mediated hypercalcemia in benign congenital mesoblastic nephroma. ( 21161280 )
2011
31
Pathology in practice. Congenital mesoblastic nephroma. ( 21961632 )
2011
32
Congenital mesoblastic nephroma. ( 20096866 )
2010
33
Elevated levels of aldosterone in the amniotic fluid in two cases of congenital mesoblastic nephroma. ( 20455206 )
2010
34
Regression of a congenital mesoblastic nephroma. ( 20582939 )
2010
35
Imaging of congenital mesoblastic nephroma with pathological correlation. ( 19629463 )
2009
36
Pamidronate therapy for hypercalcemia and congenital mesoblastic nephroma: a case report. ( 20062638 )
2009
37
WT1 expression and hemihypertrophy in congenital mesoblastic nephroma. ( 19011477 )
2008
38
Congenital mesoblastic nephroma: a case report. ( 23012173 )
2008
39
Congenital mesoblastic nephroma rich from mitosis after in vitro fertilization: a case report. ( 18485932 )
2008
40
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
41
Atypical congenital mesoblastic nephroma presenting in the perinatal period. ( 17093992 )
2007
42
A case of fetal congenital mesoblastic nephroma with oligohydramnios. ( 17449950 )
2007
43
Congenital mesoblastic nephroma presenting with massive hematuria and hemorrhagic shock: report of one case. ( 17078466 )
2006
44
Expression of ETV6-NTRK in classical, cellular and mixed subtypes of congenital mesoblastic nephroma. ( 16681692 )
2006
45
Prenatal diagnosis of congenital mesoblastic nephroma associated with renal hypertension in a premature child. ( 15711357 )
2005
46
Congenital mesoblastic nephroma presenting with neonatal hypertension. ( 15858607 )
2005
47
Cellular congenital mesoblastic nephroma with contralateral medullary nephrocalcinosis. ( 15121709 )
2004
48
Prenatal diagnosis of congenital mesoblastic nephroma associated with renal hypertension in a premature child. ( 14678186 )
2004
49
Pathologic quiz case: renal mass in a 3-week-old infant. Congenital mesoblastic nephroma, mixed type. ( 15270602 )
2004
50
Prenatal diagnosis of congenital mesoblastic nephroma in mid-second trimester by sonography and magnetic resonance imaging. ( 14634980 )
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
12.45 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

Cellular components related to Congenital Mesoblastic Nephroma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 nucleolus GO:0005730 9.26 CTCF ETV6 MKI67 WT1
2 condensed chromosome GO:0000793 8.62 CTCF MKI67

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 9.62 CTCF ETV6 IGF2 WT1
2 negative regulation of apoptotic process GO:0043066 9.56 IGF2 KIT NTRK3 WT1
3 positive regulation of MAPK cascade GO:0043410 9.33 IGF2 KIT NTRK3
4 regulation of gene expression by genetic imprinting GO:0006349 8.96 CTCF IGF2
5 activation of MAPK activity GO:0000187 8.8 IGF2 KIT NTRK3

Sources for Congenital Mesoblastic Nephroma

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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