MCID: CNG029
MIFTS: 42

Congenital Mesoblastic Nephroma

Categories: Cancer diseases, Nephrological diseases, Rare diseases

Aliases & Classifications for Congenital Mesoblastic Nephroma

MalaCards integrated aliases for Congenital Mesoblastic Nephroma:

Name: Congenital Mesoblastic Nephroma 12 75 53 59 15 72
Nephroma, Mesoblastic 44
Mesoblastic Nephroma 17

Classifications:

Orphanet: 59  
Rare renal diseases


External Ids:

Disease Ontology 12 DOID:4773
MeSH 44 D018201
NCIt 50 C6569
MESH via Orphanet 45 D018201
ICD10 via Orphanet 34 D41.0
UMLS via Orphanet 73 C1332965
Orphanet 59 ORPHA2665
UMLS 72 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 congenital fibrosarcoma. An important gene associated with Congenital Mesoblastic Nephroma is ETV6 (ETS Variant Transcription Factor 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 Reduced mammosphere formation and cardiovascular system

Wikipedia : 75 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 154)
# Related Disease Score Top Affiliating Genes
1 cellular congenital mesoblastic nephroma 34.4 NTRK3 NRAS ETV6
2 congenital fibrosarcoma 31.9 NTRK3 ETV6
3 rhabdoid cancer 31.3 WT1 SMARCB1
4 adult mesoblastic nephroma 31.3 NTRK3 ETV6
5 kidney rhabdoid cancer 31.2 WT1 SMARCB1
6 kidney clear cell sarcoma 31.1 WT1 ETV6
7 breast secretory carcinoma 31.1 NTRK3 ETV6
8 hemihyperplasia, isolated 31.1 WT1 IGF2
9 clear cell sarcoma 30.9 WT1 KIT IGF2
10 rhabdoid tumor predisposition syndrome 1 30.9 WT1 SMARCB1 IGF2
11 beckwith-wiedemann syndrome 30.7 WT1 IGF2 CTCF
12 cystic nephroma 30.7 WT1 KIT
13 mesenchymal cell neoplasm 30.6 SMARCB1 KIT
14 conventional fibrosarcoma 30.5 NRAS KIT
15 wilms tumor 1 30.4 WT1 SMARCB1 IGF2 CTCF
16 acute leukemia 30.3 WT1 KIT ETV6
17 leiomyoma 30.1 MKI67 KIT IGF2
18 malignant mesenchymoma 29.9 WT1 NTRK3 KIT ETV6
19 classic congenital mesoblastic nephroma 12.6
20 infantile myofibromatosis 11.8
21 myofibromatosis, infantile, 1 11.5
22 graham boyle troxell syndrome 11.5
23 wilms tumor 5 10.9
24 polyhydramnios 10.8
25 fibrosarcoma 10.8
26 chromosomal triplication 10.7
27 rare tumor 10.6
28 rare renal tumor 10.6
29 congenital heart defects, hamartomas of tongue, and polysyndactyly 10.6
30 fetal edema 10.6
31 hydrops fetalis 10.6
32 pediatric fibrosarcoma 10.6 NTRK3 ETV6
33 neuroblastoma 1 10.5
34 adenofibroma 10.5
35 nephrogenic adenofibroma 10.5
36 hereditary wilms' tumor 10.4 WT1 IGF2
37 iris disease 10.4 WT1 IGF2
38 renal hypertension 10.4
39 disseminated intravascular coagulation 10.4
40 nephrocalcinosis 10.4
41 renal cell carcinoma, nonpapillary 10.4
42 metanephric adenoma 10.4
43 adenoma 10.4
44 melanomatosis 10.4 WT1 NRAS
45 wilms tumor, aniridia, genitourinary anomalies, and mental retardation syndrome 10.3 WT1 IGF2
46 adult fibrosarcoma 10.3 WT1 NTRK3 ETV6
47 testicular gonadoblastoma 10.3 WT1 KIT
48 gonadoblastoma 10.3 WT1 KIT
49 acute myeloid leukemia with abnormal bone marrow eosinophils inv(16)(p13q22) or t(16;16)(p13;q22) 10.3 KIT ETV6
50 wilson-turner x-linked mental retardation syndrome 10.2 WT1 IGF2

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
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Reduced mammosphere formation GR00396-S 9.02 ETV6 IGF2 NRAS NT5C1B SMARCB1

MGI Mouse Phenotypes related to Congenital Mesoblastic Nephroma:

46 (show all 12)
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 10.05 ETV6 IGF2 KIT NRAS NTRK3 SMARCB1
2 cellular MP:0005384 10.04 CTCF ETV6 IGF2 KIT NRAS SMARCB1
3 embryo MP:0005380 10 CTCF ETV6 IGF2 KIT NRAS SMARCB1
4 hematopoietic system MP:0005397 9.95 CTCF ETV6 IGF2 KIT NRAS SMARCB1
5 endocrine/exocrine gland MP:0005379 9.91 CTCF ETV6 IGF2 KIT NRAS WT1
6 immune system MP:0005387 9.91 CTCF ETV6 IGF2 KIT NRAS SMARCB1
7 digestive/alimentary MP:0005381 9.88 ETV6 IGF2 KIT NRAS SMARCB1
8 mortality/aging MP:0010768 9.86 CTCF ETV6 IGF2 KIT NRAS NTRK3
9 liver/biliary system MP:0005370 9.72 IGF2 KIT NRAS SMARCB1 WT1
10 neoplasm MP:0002006 9.55 ETV6 KIT NRAS SMARCB1 WT1
11 no phenotypic analysis MP:0003012 9.5 ETV6 IGF2 KIT MKI67 NRAS NTRK3
12 normal MP:0002873 9.1 ETV6 KIT MKI67 NRAS NTRK3 WT1

Drugs & Therapeutics for Congenital Mesoblastic Nephroma

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

(show all 27)
# 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 Liver Extracts Phase 3
4 Analgesics Phase 3
5 Anesthetics, Intravenous Phase 3
6 Narcotics Phase 3
7 Cola Phase 3
8 Peripheral Nervous System Agents Phase 3
9 Analgesics, Opioid Phase 3
10 Adjuvants, Anesthesia Phase 3
11 Central Nervous System Depressants Phase 3
12 Anesthetics, General Phase 3
13 Anesthetics Phase 3
14
Ifosfamide Approved Phase 1, Phase 2 3778-73-2 3690
15
Etoposide Approved Phase 1, Phase 2 33419-42-0 36462
16
Dasatinib Approved, Investigational Phase 1, Phase 2 302962-49-8 3062316
17
Mechlorethamine Approved, Investigational Phase 1, Phase 2 51-75-2 4033
18
Carboplatin Approved Phase 1, Phase 2 41575-94-4 10339178 498142 38904
19 Protein Kinase Inhibitors Phase 1, Phase 2
20 Antineoplastic Agents, Phytogenic Phase 1, Phase 2
21 Alkylating Agents Phase 1, Phase 2
22 Topoisomerase Inhibitors Phase 1, Phase 2
23
Isophosphamide mustard Phase 1, Phase 2 0
24 Etoposide phosphate Phase 1, Phase 2
25 Antineoplastic Agents, Alkylating Phase 1, Phase 2
26
Entrectinib Approved, Investigational Phase 1 1108743-60-7
27
Tyrosine Approved, Investigational, Nutraceutical Phase 1 60-18-4 6057

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 A Randomized, Double-blind, Placebo-controlled Multi-center Study to Evaluate the Safety and Efficacy of Fentanyl Sublingual Spray (Fentanyl SL Spray) for the Treatment of Breakthrough Cancer Pain Completed NCT00538850 Phase 3 Fentanyl sublingual spray;Placebo
2 Dasatinib With Ifosfamide, Carboplatin, Etoposide: A Pediatric Phase I/II Trial Active, not recruiting NCT00788125 Phase 1, Phase 2 carboplatin;dasatinib;etoposide phosphate;ifosfamide
3 A Phase 1/2, Open-Label, Dose-Escalation and Expansion Study Of Entrectinib (Rxdx-101) in Children and Adolescents With No Curative First-Line Treatment Option, Recurrent or Refractory Solid Tumors And Primary Cns Tumors, With or Without Trk, Ros1, or Alk Fusions Recruiting NCT02650401 Phase 1 Entrectinib
4 A Population Based Study of Genetic Predisposition and Gene-Environment Interactions in Cancer in East Anglia, Trent and West Midlands Unknown status NCT00757614
5 The Project: EveryChild Protocol: A Registry, Eligibility Screening, Biology and Outcome Study Recruiting NCT02402244
6 Renal Tumors Classification, Biology, and Banking Study Suspended NCT00898365
7 Protocol for the Enrollment on the Official COG Registry, The Childhood Cancer Research Network (CCRN) Children s Oncology Group Terminated NCT01117168
8 Focal Adhesion Kinase Expression in Pediatric Renal Tumors 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, Lung, Testes, Breast

Publications for Congenital Mesoblastic Nephroma

Articles related to Congenital Mesoblastic Nephroma:

(show top 50) (show all 319)
# Title Authors PMID Year
1
WT1 expression and hemihypertrophy in congenital mesoblastic nephroma. 9 38
19011477 2008
2
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. 9 38
17639583 2007
3
KIT expression in normal and neoplastic renal tissues: immunohistochemical and molecular genetic analysis. 9 38
15998375 2005
4
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. 9 38
12165445 2002
5
ETV6 rearrangements in patients with infantile fibrosarcomas and congenital mesoblastic nephromas by fluorescence in situ hybridization. 9 38
11743047 2001
6
Insulin-like growth factor II gene expression by congenital mesoblastic nephroma. 9 38
8634785 1995
7
Good prognosis of cellular mesoblastic nephroma with hyperdiploidy and relaxation of imprinting of the maternal IGF2 gene. 9 38
8597855 1995
8
Laparoscopic management of congenital mesoblastic nephroma- case report. 38
31388496 2019
9
Unusual Case of Concurrent Retroperitoneal Congenital Infantile Fibrosarcoma and Cellular Type Congenital Mesoblastic Nephroma. 38
31343481 2019
10
Re: Recurrent and Metastatic Congenital Mesoblastic Nephroma: Where Does the Evidence Stand? 38
31343939 2019
11
Paraneoplastic disorders associated with miscellaneous neoplasms with focus on selected soft tissue and Undifferentiated/ rhabdoid malignancies. 38
30819530 2019
12
The clinical presentation, imaging features and differential diagnoses of congenital Wilms tumour. 38
31151974 2019
13
Anaplastic sarcoma of the kidney: Case report and literature review. 38
31007495 2019
14
Neurotrophic tropomyosin or tyrosine receptor kinase (NTRK) genes. 38
30636697 2019
15
TRK Fusion Cancers in Children: A Clinical Review and Recommendations for Screening. 38
30592640 2019
16
Specific computed tomography imaging characteristics of congenital mesoblastic nephroma and correlation with ultrasound and pathology. 38
30145031 2018
17
Estimated glomerular filtration rate after nephrectomy for Wilms tumor. 38
30003640 2018
18
Dramatic bone remodeling following larotrectinib administration for bone metastasis in a patient with TRK fusion congenital mesoblastic nephroma. 38
29893456 2018
19
Recurrent intragenic rearrangements of EGFR and BRAF in soft tissue tumors of infants. 38
29915264 2018
20
Evaluation of renal tumors in children. 38
29733801 2018
21
Fetal biparametric MR imaging in the diagnosis of congenital mesoblastic nephroma. 38
29733803 2018
22
ETV6-NTRK3 in congenital mesoblastic nephroma: A report of the SIOP/GPOH nephroblastoma study. 38
29286563 2018
23
Use of Pretherapy Core Biopsy in the Diagnosis of Pediatric Renal Tumors. 38
29681695 2018
24
Recurrent EML4-NTRK3 fusions in infantile fibrosarcoma and congenital mesoblastic nephroma suggest a revised testing strategy. 38
29099503 2018
25
Neonatal solid tumors. 38
28736178 2018
26
A rare cause of neonatal hypertension: Congenital mesoblastic nephroma. 38
30325129 2018
27
Targeting NTRK fusions for the treatment of congenital mesoblastic nephroma. 38
28440051 2018
28
Congenital Mesoblastic Nephroma Presenting with Hematuria in a Neonate. 38
30464149 2018
29
Recurrent and metastatic congenital mesoblastic nephroma: where does the evidence stand? 38
28856451 2017
30
Unusual Presentation of Metanephric Stromal Tumor in a Neonate. 38
29200690 2017
31
Congenital mesoblastic nephroma 50 years after its recognition: A narrative review. 38
28124468 2017
32
Cellular Congenital Mesoblastic Nephroma in a Newborn. 38
28770142 2017
33
Renal Tumors in Children Younger Than 12 Months of Age: A 65-Year Single Institution Review. 38
27820132 2017
34
Management of mixed type congenital mesoblastic nephroma: Case series and review of the literature. 38
28282819 2017
35
Congenital Mesoblastic Nephroma Presenting With Refractory Hypertension in a Premature Neonate: A Case Study. 38
28137351 2017
36
Cytological diagnosis of mesoblastic nephroma: A report of three cases with summary of prior published cases. 38
27324404 2016
37
[Congenital mesoblastic nephroma: a clinicopathologic analysis of 17 cases]. 38
27646897 2016
38
Uncommon renal tumors in children: A single center experience. 38
27046976 2016
39
Congenital Mesoblastic Nephroma Presenting With Hematuria in a Neonate: A Case Report. 38
26616096 2016
40
Congenital mesoblastic nephroma: Its diverse clinical features - A literature review with a case report. 38
26467634 2016
41
The Value of Mitotic Count and Ki67 Proliferation Index in Congenital Mesoblastic Nephroma. 38
27484189 2016
42
Congenital mesoblastic nephroma: a study of 19 cases using immunohistochemistry and ETV6-NTRK3 fusion gene rearrangement. 38
27020209 2016
43
Mixed subtype of congenital mesoblastic nephroma with poor evolution: a case report and literature review. 38
26811558 2015
44
Congenital mesoblastic nephroma with focal anaplastic lesion. 38
25818639 2015
45
Prenatal diagnosis of congenital mesoblastic nephroma. 38
26430667 2015
46
Diffuse and strong cyclin D1 immunoreactivity in clear cell sarcoma of the kidney. 38
25556515 2015
47
Growing Renal Mass: Lessons Learned on the Road From an Atypical Presentation to Successful Therapy. 38
26793537 2015
48
Intestinal congenital/infantile fibrosarcoma: a new clinico-pathological entity? 38
25652760 2015
49
Congenital mesoblastic nephroma: Clinical analysis of eight cases and a review of the literature. 38
25295083 2014
50
Maternal manifestation of Ballantyne's syndrome occurring concomitantly with the development of fetal congenital mesoblastic nephroma. 38
24428571 2014

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
12.56 NTRK3 NRAS KIT IGF2
2
Show member pathways
12.51 NTRK3 NRAS KIT IGF2
3
Show member pathways
11.92 SMARCB1 NRAS KIT IGF2
4
Show member pathways
11.75 NTRK3 NRAS KIT IGF2
5
Show member pathways
10.67 NRAS IGF2
6 10.64 NTRK3 NRAS KIT

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 9.35 WT1 SMARCB1 MKI67 ETV6 CTCF
2 condensed chromosome GO:0000793 8.62 MKI67 CTCF

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

# Name GO ID Score Top Affiliating Genes
1 positive regulation of transcription by RNA polymerase II GO:0045944 9.72 WT1 SMARCB1 IGF2 ETV6 CTCF
2 positive regulation of MAPK cascade GO:0043410 9.43 NTRK3 KIT IGF2
3 positive regulation of gene expression GO:0010628 9.26 WT1 NTRK3 KIT CTCF
4 regulation of gene expression by genetic imprinting GO:0006349 9.16 IGF2 CTCF
5 positive regulation of phospholipase C activity GO:0010863 8.62 NTRK3 KIT

Sources for Congenital Mesoblastic Nephroma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 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 TGDB
71 Tocris
72 UMLS
73 UMLS via Orphanet
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