MCID: CNG029
MIFTS: 40

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 74 52 58 15 71
Nephroma, Mesoblastic 43
Mesoblastic Nephroma 17

Classifications:

Orphanet: 58  
Rare renal diseases


External Ids:

Disease Ontology 12 DOID:4773
MeSH 43 D018201
NCIt 49 C6569
MESH via Orphanet 44 D018201
ICD10 via Orphanet 33 D41.0
UMLS via Orphanet 72 C1332965
Orphanet 58 ORPHA2665
UMLS 71 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 breast secretory carcinoma. An important gene associated with Congenital Mesoblastic Nephroma is ETV6 (ETS Variant Transcription Factor 6), and among its related pathways/superpathways are p70S6K Signaling and NFAT and Cardiac Hypertrophy. The drugs Fentanyl and Pancreatic Polypeptide have been mentioned in the context of this disorder. Affiliated tissues include kidney, myeloid and liver, and related phenotypes are Increased shRNA abundance (Z-score > 2) and Increased shRNA abundance (Z-score > 2)

Wikipedia : 74 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 157)
# Related Disease Score Top Affiliating Genes
1 cellular congenital mesoblastic nephroma 33.7 VGLL2 SSX2 NTRK3 ETV6
2 breast secretory carcinoma 31.1 NTRK3 ETV6
3 hemihyperplasia, isolated 31.1 WT1 IGF2
4 clear cell sarcoma 31.0 YWHAE WT1 KIT IGF2
5 fibrosarcoma 30.7 SSX2 SSX1 NTRK3 KIT ETV6
6 malignant mesenchymoma 30.6 SSX4B SSX2
7 leiomyoma 30.6 MKI67 KIT IGF2
8 conventional fibrosarcoma 30.6 NRAS KIT
9 wilms tumor 1 30.5 WT1 KIT IGF2 CTCF AMER1
10 mesenchymal cell neoplasm 30.5 SSX2 NTRK3 NTRK1 KIT
11 spindle cell sarcoma 30.4 SSX2 SSX1 NTRK3
12 adult mesoblastic nephroma 30.3 WT1 SSX2 NTRK3 KIT ETV6
13 congenital fibrosarcoma 30.2 VGLL2 NTRK3 NTRK1 ETV6 BTBD1
14 kidney sarcoma 29.9 YWHAE WT1 SSX2 SSX1
15 rhabdomyosarcoma 29.2 VGLL2 SSX2 SSX1 KIT IGF2
16 kidney clear cell sarcoma 29.1 YWHAE WT1 VGLL2 TARID NTRK3 ETV6
17 sarcoma 29.0 WT1 SSX4B SSX4 SSX2 SSX1 NRAS
18 classic congenital mesoblastic nephroma 12.6
19 infantile myofibromatosis 11.8
20 myofibromatosis, infantile, 1 11.5
21 graham boyle troxell syndrome 11.5
22 wilms tumor 5 10.9
23 polyhydramnios 10.8
24 chromosomal triplication 10.7
25 rare tumor 10.6
26 rare renal tumor 10.6
27 congenital heart defects, hamartomas of tongue, and polysyndactyly 10.6
28 hydrops fetalis, nonimmune 10.6
29 rhabdoid tumor predisposition syndrome 1 10.5
30 rhabdoid cancer 10.5
31 neuroblastoma 10.5
32 pregnancy adenoma 10.5 NTRK3 ETV6
33 pax6-related aniridia 10.5 WT1 IGF2
34 testicular gonadoblastoma 10.5 WT1 KIT
35 kidney rhabdoid cancer 10.5
36 adenofibroma 10.5
37 nephrogenic adenofibroma 10.5
38 selection of therapeutic option in melanoma 10.4 NRAS KIT
39 pulmonary vein stenosis 10.4 MKI67 KIT
40 ovarian gonadoblastoma 10.4 WT1 KIT
41 acute myeloid leukemia with abnormal bone marrow eosinophils inv(16)(p13q22) or t(16;16)(p13;q22) 10.4 KIT ETV6
42 malignant anus melanoma 10.4 NRAS KIT
43 hypereosinophilic syndrome, idiopathic 10.4 WT1 KIT
44 myeloid and lymphoid neoplasms associated with pdgfra rearrangement 10.4 KIT ETV6
45 kidney hemangiopericytoma 10.4 SSX2 IGF2
46 liver leiomyosarcoma 10.4 WT1 KIT
47 malignant dermis tumor 10.4 NRAS KIT
48 cerebellar angioblastoma 10.4 WT1 NTRK3 ETV6
49 malignant skin fibrous histiocytoma 10.4 NRAS KIT
50 beckwith-wiedemann syndrome 10.4

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 24)
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Increased shRNA abundance (Z-score > 2) GR00366-A-100 9.84 SSX2 WT1
2 Increased shRNA abundance (Z-score > 2) GR00366-A-102 9.84 KIT
3 Increased shRNA abundance (Z-score > 2) GR00366-A-122 9.84 WT1
4 Increased shRNA abundance (Z-score > 2) GR00366-A-137 9.84 NTRK3
5 Increased shRNA abundance (Z-score > 2) GR00366-A-149 9.84 SSX2
6 Increased shRNA abundance (Z-score > 2) GR00366-A-152 9.84 KIT NTRK3 WT1
7 Increased shRNA abundance (Z-score > 2) GR00366-A-157 9.84 KIT
8 Increased shRNA abundance (Z-score > 2) GR00366-A-170 9.84 KIT
9 Increased shRNA abundance (Z-score > 2) GR00366-A-191 9.84 SSX2
10 Increased shRNA abundance (Z-score > 2) GR00366-A-214 9.84 WT1
11 Increased shRNA abundance (Z-score > 2) GR00366-A-23 9.84 WT1
12 Increased shRNA abundance (Z-score > 2) GR00366-A-30 9.84 KIT NTRK3 SSX2 WT1
13 Increased shRNA abundance (Z-score > 2) GR00366-A-35 9.84 WT1
14 Increased shRNA abundance (Z-score > 2) GR00366-A-41 9.84 NTRK3
15 Increased shRNA abundance (Z-score > 2) GR00366-A-42 9.84 KIT NTRK3
16 Increased shRNA abundance (Z-score > 2) GR00366-A-43 9.84 WT1
17 Increased shRNA abundance (Z-score > 2) GR00366-A-47 9.84 WT1
18 Increased shRNA abundance (Z-score > 2) GR00366-A-60 9.84 NTRK3
19 Increased shRNA abundance (Z-score > 2) GR00366-A-76 9.84 SSX2
20 Increased shRNA abundance (Z-score > 2) GR00366-A-77 9.84 SSX2
21 Increased shRNA abundance (Z-score > 2) GR00366-A-81 9.84 SSX2
22 Increased shRNA abundance (Z-score > 2) GR00366-A-85 9.84 KIT NTRK3 WT1
23 Increased shRNA abundance (Z-score > 2) GR00366-A-9 9.84 KIT
24 Increased shRNA abundance (Z-score > 2) GR00366-A-99 9.84 KIT

MGI Mouse Phenotypes related to Congenital Mesoblastic Nephroma:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 no phenotypic analysis MP:0003012 9.23 ETV6 IGF2 KIT MKI67 NRAS NTRK1

Drugs & Therapeutics for Congenital Mesoblastic Nephroma

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

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

40
Kidney, Myeloid, Liver, Breast, Bone, Lung, Heart

Publications for Congenital Mesoblastic Nephroma

Articles related to Congenital Mesoblastic Nephroma:

(show top 50) (show all 323)
# Title Authors PMID Year
1
WT1 expression and hemihypertrophy in congenital mesoblastic nephroma. 54 61
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. 54 61
17639583 2007
3
KIT expression in normal and neoplastic renal tissues: immunohistochemical and molecular genetic analysis. 54 61
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. 54 61
12165445 2002
5
ETV6 rearrangements in patients with infantile fibrosarcomas and congenital mesoblastic nephromas by fluorescence in situ hybridization. 54 61
11743047 2001
6
Insulin-like growth factor II gene expression by congenital mesoblastic nephroma. 54 61
8634785 1995
7
Good prognosis of cellular mesoblastic nephroma with hyperdiploidy and relaxation of imprinting of the maternal IGF2 gene. 54 61
8597855 1995
8
Congenital mesoblastic nephroma: a single-centre series. 61
31508997 2020
9
Diagnostic Utility of BCOR Antibody in Clear Cell Sarcomas of Kidney. 61
31875746 2019
10
Lessons learned from the developmental origins of childhood renal cancer. 61
31738020 2019
11
Re: Recurrent and Metastatic Congenital Mesoblastic Nephroma: Where Does the Evidence Stand? 61
31343939 2019
12
Laparoscopic management of congenital mesoblastic nephroma- case report. 61
31388496 2019
13
Prenatal diagnosis of congenital mesoblastic nephroma: a case with poor prognosis. 61
31466985 2019
14
Unusual Case of Concurrent Retroperitoneal Congenital Infantile Fibrosarcoma and Cellular Type Congenital Mesoblastic Nephroma. 61
31343481 2019
15
Paraneoplastic disorders associated with miscellaneous neoplasms with focus on selected soft tissue and Undifferentiated/ rhabdoid malignancies. 61
30819530 2019
16
The clinical presentation, imaging features and differential diagnoses of congenital Wilms tumour. 61
31151974 2019
17
Anaplastic sarcoma of the kidney: Case report and literature review. 61
31007495 2019
18
Neurotrophic tropomyosin or tyrosine receptor kinase (NTRK) genes. 61
30636697 2019
19
TRK Fusion Cancers in Children: A Clinical Review and Recommendations for Screening. 61
30592640 2019
20
Specific computed tomography imaging characteristics of congenital mesoblastic nephroma and correlation with ultrasound and pathology. 61
30145031 2018
21
Estimated glomerular filtration rate after nephrectomy for Wilms tumor. 61
30003640 2018
22
Dramatic bone remodeling following larotrectinib administration for bone metastasis in a patient with TRK fusion congenital mesoblastic nephroma. 61
29893456 2018
23
Recurrent intragenic rearrangements of EGFR and BRAF in soft tissue tumors of infants. 61
29915264 2018
24
Evaluation of renal tumors in children. 61
29733801 2018
25
Fetal biparametric MR imaging in the diagnosis of congenital mesoblastic nephroma. 61
29733803 2018
26
ETV6-NTRK3 in congenital mesoblastic nephroma: A report of the SIOP/GPOH nephroblastoma study. 61
29286563 2018
27
Use of Pretherapy Core Biopsy in the Diagnosis of Pediatric Renal Tumors. 61
29681695 2018
28
Recurrent EML4-NTRK3 fusions in infantile fibrosarcoma and congenital mesoblastic nephroma suggest a revised testing strategy. 61
29099503 2018
29
Neonatal solid tumors. 61
28736178 2018
30
Congenital Mesoblastic Nephroma Presenting with Hematuria in a Neonate. 61
30464149 2018
31
Targeting NTRK fusions for the treatment of congenital mesoblastic nephroma. 61
28440051 2018
32
A rare cause of neonatal hypertension: Congenital mesoblastic nephroma. 61
30325129 2018
33
Recurrent and metastatic congenital mesoblastic nephroma: where does the evidence stand? 61
28856451 2017
34
Unusual Presentation of Metanephric Stromal Tumor in a Neonate. 61
29200690 2017
35
Congenital mesoblastic nephroma 50 years after its recognition: A narrative review. 61
28124468 2017
36
Cellular Congenital Mesoblastic Nephroma in a Newborn. 61
28770142 2017
37
Renal Tumors in Children Younger Than 12 Months of Age: A 65-Year Single Institution Review. 61
27820132 2017
38
Congenital Mesoblastic Nephroma Presenting With Refractory Hypertension in a Premature Neonate: A Case Study. 61
28137351 2017
39
Management of mixed type congenital mesoblastic nephroma: Case series and review of the literature. 61
28282819 2017
40
Cytological diagnosis of mesoblastic nephroma: A report of three cases with summary of prior published cases. 61
27324404 2016
41
[Congenital mesoblastic nephroma: a clinicopathologic analysis of 17 cases]. 61
27646897 2016
42
Uncommon renal tumors in children: A single center experience. 61
27046976 2016
43
Congenital Mesoblastic Nephroma Presenting With Hematuria in a Neonate: A Case Report. 61
26616096 2016
44
The Value of Mitotic Count and Ki67 Proliferation Index in Congenital Mesoblastic Nephroma. 61
27484189 2016
45
Congenital mesoblastic nephroma: Its diverse clinical features - A literature review with a case report. 61
26467634 2016
46
Congenital mesoblastic nephroma: a study of 19 cases using immunohistochemistry and ETV6-NTRK3 fusion gene rearrangement. 61
27020209 2016
47
Mixed subtype of congenital mesoblastic nephroma with poor evolution: a case report and literature review. 61
26811558 2015
48
Congenital mesoblastic nephroma with focal anaplastic lesion. 61
25818639 2015
49
Diffuse and strong cyclin D1 immunoreactivity in clear cell sarcoma of the kidney. 61
25556515 2015
50
Prenatal diagnosis of congenital mesoblastic nephroma. 61
26430667 2015

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
12.52 YWHAE NTRK3 NTRK1 NRAS KIT IGF2
2
Show member pathways
12.5 YWHAE NTRK3 NTRK1 NRAS IGF2
3
Show member pathways
12.04 YWHAE NTRK3 NTRK1 NRAS
4
Show member pathways
11.97 NTRK3 NTRK1 KIT IGF2
5
Show member pathways
11.95 NTRK3 NTRK1 NRAS KIT IGF2
6
Show member pathways
11.89 YWHAE NTRK3 NTRK1 NRAS
7 11.62 WT1 SSX2 SSX1 NTRK1 ETV6
8 11.42 NTRK3 NTRK1 NRAS IGF2
9 10.76 NTRK3 NTRK1 NRAS KIT

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 transmembrane receptor protein tyrosine kinase signaling pathway GO:0007169 9.63 NTRK3 NTRK1 KIT
2 positive regulation of phosphatidylinositol 3-kinase signaling GO:0014068 9.5 NTRK3 NTRK1 KIT
3 regulation of gene expression by genetic imprinting GO:0006349 9.43 IGF2 CTCF
4 regulation of protein kinase B signaling GO:0051896 9.4 NTRK3 NTRK1
5 positive regulation of phospholipase C activity GO:0010863 9.37 NTRK3 KIT
6 regulation of transcription, DNA-templated GO:0006355 9.28 WT1 VGLL2 SSX4B SSX4 SSX2 SSX1
7 mechanoreceptor differentiation GO:0042490 9.26 NTRK3 NTRK1
8 positive regulation of MAPK cascade GO:0043410 9.26 NTRK3 NTRK1 KIT IGF2
9 neurotrophin signaling pathway GO:0038179 9.16 NTRK3 NTRK1

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

# Name GO ID Score Top Affiliating Genes
1 nucleic acid binding GO:0003676 9.8 WT1 SSX4B SSX4 SSX2 SSX1 CTCF
2 protein tyrosine kinase activity GO:0004713 9.54 NTRK3 NTRK1 KIT
3 transmembrane receptor protein tyrosine kinase activity GO:0004714 9.33 NTRK3 NTRK1 KIT
4 GPI-linked ephrin receptor activity GO:0005004 9.26 NTRK3 NTRK1
5 neurotrophin binding GO:0043121 8.96 NTRK3 NTRK1
6 neurotrophin receptor activity GO:0005030 8.62 NTRK3 NTRK1

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 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
Content
Loading form....