MCID: CNG029
MIFTS: 43

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 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 beckwith-wiedemann syndrome. 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, bone and liver, 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 89)
# Related Disease Score Top Affiliating Genes
1 cellular congenital mesoblastic nephroma 33.9 ETV6 NRAS NTRK3
2 beckwith-wiedemann syndrome 30.3 CTCF IGF2 WT1
3 wilms tumor 1 30.2 CTCF IGF2 WT1
4 clear cell sarcoma 30.1 IGF2 KIT WT1
5 leiomyoma 30.0 IGF2 KIT MKI67
6 classic congenital mesoblastic nephroma 12.4
7 polyhydramnios 10.5
8 wilms tumor 6 10.4
9 fibrosarcoma 10.4
10 hydrops fetalis, nonimmune, and/or atrial septal defect 10.4
11 renal hypertension 10.4
12 sarcoma 10.4
13 nephrocalcinosis 10.4
14 fetal edema 10.4
15 hydrops fetalis 10.4
16 adult mesoblastic nephroma 10.2 ETV6 NTRK3
17 disseminated intravascular coagulation 10.2
18 oligohydramnios 10.2
19 von willebrand's disease 10.2
20 hypertrophic pyloric stenosis 10.2
21 pyloric stenosis 10.2
22 pediatric fibrosarcoma 10.2 ETV6 NTRK3
23 congenital fibrosarcoma 10.2 ETV6 NTRK3
24 kidney clear cell sarcoma 10.2 ETV6 WT1
25 breast secretory carcinoma 10.2 ETV6 NTRK3
26 hemihyperplasia, isolated 10.2 IGF2 WT1
27 hereditary wilms' tumor 10.1 IGF2 WT1
28 iris disease 10.1 IGF2 WT1
29 wilms tumor, aniridia, genitourinary anomalies, and mental retardation syndrome 10.1 IGF2 WT1
30 adult fibrosarcoma 10.1 ETV6 NTRK3 WT1
31 melanomatosis 10.1 NRAS WT1
32 rhabdoid tumor predisposition syndrome 1 10.1 IGF2 WT1
33 wilson-turner x-linked mental retardation syndrome 10.0 IGF2 WT1
34 testicular gonadoblastoma 10.0 KIT WT1
35 gonadoblastoma 10.0 KIT WT1
36 acute myeloid leukemia with abnormal bone marrow eosinophils inv(16)(p13q22) or t(16;16)(p13;q22) 10.0 ETV6 KIT
37 peritoneal benign neoplasm 10.0 KIT WT1
38 diffuse peritoneal leiomyomatosis 10.0 KIT WT1
39 small intestine leiomyosarcoma 10.0 IGF2 KIT
40 hypereosinophilic syndrome, idiopathic 10.0 KIT WT1
41 small intestinal sarcoma 10.0 IGF2 KIT
42 cystic nephroma 10.0 KIT WT1
43 gastric leiomyosarcoma 10.0 IGF2 KIT
44 aniridia 1 10.0 IGF2 WT1
45 mixed germ cell-sex cord neoplasm 10.0 KIT WT1
46 spindle cell liposarcoma 10.0 KIT WT1
47 heart sarcoma 10.0 KIT WT1
48 chronic eosinophilic leukemia 10.0 KIT WT1
49 differentiated thyroid carcinoma 10.0 ETV6 NRAS NTRK3
50 embryoma 10.0 IGF2 KIT

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 31)
# 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 9.83 IGF2 NTRK3
21 Decreased shRNA abundance (Z-score < -2) GR00366-A-134 9.83 IGF2
22 Decreased shRNA abundance (Z-score < -2) GR00366-A-148 9.83 IGF2 NRAS NTRK3
23 Decreased shRNA abundance (Z-score < -2) GR00366-A-184 9.83 NTRK3
24 Decreased shRNA abundance (Z-score < -2) GR00366-A-204 9.83 NRAS
25 Decreased shRNA abundance (Z-score < -2) GR00366-A-28 9.83 NRAS
26 Decreased shRNA abundance (Z-score < -2) GR00366-A-55 9.83 NTRK3
27 Decreased shRNA abundance (Z-score < -2) GR00366-A-60 9.83 NTRK3
28 Decreased shRNA abundance (Z-score < -2) GR00366-A-66 9.83 IGF2
29 Decreased shRNA abundance (Z-score < -2) GR00366-A-72 9.83 NRAS
30 Decreased shRNA abundance (Z-score < -2) GR00366-A-87 9.83 IGF2
31 Decreased shRNA abundance (Z-score < -2) GR00366-A-91 9.83 IGF2

MGI Mouse Phenotypes related to Congenital Mesoblastic Nephroma:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 9.8 ETV6 IGF2 KIT NRAS NTRK3 WT1
2 embryo MP:0005380 9.73 CTCF ETV6 IGF2 KIT NRAS WT1
3 endocrine/exocrine gland MP:0005379 9.63 CTCF ETV6 IGF2 KIT NRAS WT1
4 no phenotypic analysis MP:0003012 9.5 ETV6 IGF2 KIT MKI67 NRAS NTRK3
5 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 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 Cola Phase 3
4 Central Nervous System Depressants Phase 3
5 Adjuvants, Anesthesia Phase 3
6 Liver Extracts Phase 3,Phase 1,Phase 2
7 Anesthetics, Intravenous Phase 3
8 Analgesics, Opioid Phase 3
9 Peripheral Nervous System Agents Phase 3
10 Anesthetics, General Phase 3
11 Analgesics Phase 3
12 Narcotics Phase 3
13 Anesthetics Phase 3
14
Mechlorethamine Approved, Investigational Phase 1, Phase 2 51-75-2 4033
15
Carboplatin Approved Phase 1, Phase 2 41575-94-4 10339178 38904 498142
16
Ifosfamide Approved Phase 1, Phase 2 3778-73-2 3690
17
Etoposide Approved Phase 1, Phase 2 33419-42-0 36462
18
Dasatinib Approved, Investigational Phase 1, Phase 2 302962-49-8 3062316
19 Topoisomerase Inhibitors Phase 1, Phase 2
20 Etoposide phosphate Phase 1, Phase 2
21 Alkylating Agents Phase 1, Phase 2
22
Isophosphamide mustard Phase 1, Phase 2 0
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 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 Project: Every Child for Younger Patients With Cancer Recruiting NCT02402244
9 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, Bone, Liver, Breast, Myeloid, Bone Marrow, Small Intestine

Publications for Congenital Mesoblastic Nephroma

Articles related to Congenital Mesoblastic Nephroma:

(show top 50) (show all 158)
# 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
Specific computed tomography imaging characteristics of congenital mesoblastic nephroma and correlation with ultrasound and pathology. ( 30145031 )
2018
5
A rare cause of neonatal hypertension: Congenital mesoblastic nephroma. ( 30325129 )
2018
6
Congenital Mesoblastic Nephroma Presenting with Hematuria in a Neonate. ( 30464149 )
2018
7
Congenital mesoblastic nephroma 50 years after its recognition: A narrative review. ( 28124468 )
2017
8
Cellular Congenital Mesoblastic Nephroma in a Newborn. ( 28770142 )
2017
9
Congenital Mesoblastic Nephroma Presenting With Refractory Hypertension in a Premature Neonate: A Case Study. ( 28137351 )
2017
10
Management of mixed type congenital mesoblastic nephroma: Case series and review of the literature. ( 28282819 )
2017
11
Targeting NTRK fusions for the treatment of congenital mesoblastic nephroma. ( 28440051 )
2017
12
Recurrent EML4-NTRK3 fusions in infantile fibrosarcoma and congenital mesoblastic nephroma suggest a revised testing strategy. ( 29099503 )
2017
13
Recurrent and metastatic congenital mesoblastic nephroma: where does the evidence stand? ( 28856451 )
2017
14
Congenital Mesoblastic Nephroma Presenting With Hematuria in a Neonate: A Case Report. ( 26616096 )
2016
15
Congenital mesoblastic nephroma: a study of 19 cases using immunohistochemistry and ETV6-NTRK3 fusion gene rearrangement. ( 27020209 )
2016
16
The Value of Mitotic Count and Ki67 Proliferation Index in Congenital Mesoblastic Nephroma. ( 27484189 )
2016
17
Congenital mesoblastic nephroma: Its diverse clinical features - A literature review with a case report. ( 26467634 )
2015
18
Prenatal diagnosis of congenital mesoblastic nephroma. ( 26430667 )
2015
19
Congenital mesoblastic nephroma with focal anaplastic lesion. ( 25818639 )
2015
20
Mixed subtype of congenital mesoblastic nephroma with poor evolution: a case report and literature review. ( 26811558 )
2015
21
Congenital mesoblastic nephroma: Clinical analysis of eight cases and a review of the literature. ( 25295083 )
2014
22
Maternal manifestation of Ballantyne's syndrome occurring concomitantly with the development of fetal congenital mesoblastic nephroma. ( 24428571 )
2014
23
Congenital mesoblastic nephroma in a premature neonate: a case report and review of literature. ( 24553043 )
2014
24
Cytodiagnosis of congenital mesoblastic nephroma: a case report. ( 23417966 )
2013
25
Congenital mesoblastic nephroma: A rare cause of recurrent hematuria beyond infancy. ( 23960358 )
2013
26
Prenatal sonographic diagnosis of multicystic congenital mesoblastic nephroma. ( 23280386 )
2013
27
Prenatal diagnosis of congenital mesoblastic nephroma. ( 22354312 )
2013
28
Congenital mesoblastic nephroma: a rare pediatric neoplasm. ( 23354798 )
2013
29
AIRP best cases in radiologic-pathologic correlation: congenital mesoblastic nephroma. ( 22236896 )
2012
30
Congenital mesoblastic nephroma in a young basset hound dog. ( 23020172 )
2012
31
Intrauterine demise due to congenital mesoblastic nephroma in a fetus conceived by assisted reproductive technology. ( 21075369 )
2011
32
Cellular congenital mesoblastic nephroma: case report. ( 21541470 )
2011
33
Parathyroid-hormone-related protein-mediated hypercalcemia in benign congenital mesoblastic nephroma. ( 21161280 )
2011
34
Pathology in practice. Congenital mesoblastic nephroma. ( 21961632 )
2011
35
Congenital mesoblastic nephroma. ( 20096866 )
2010
36
Elevated levels of aldosterone in the amniotic fluid in two cases of congenital mesoblastic nephroma. ( 20455206 )
2010
37
Regression of a congenital mesoblastic nephroma. ( 20582939 )
2010
38
Imaging of congenital mesoblastic nephroma with pathological correlation. ( 19629463 )
2009
39
Pamidronate therapy for hypercalcemia and congenital mesoblastic nephroma: a case report. ( 20062638 )
2009
40
WT1 expression and hemihypertrophy in congenital mesoblastic nephroma. ( 19011477 )
2008
41
Congenital mesoblastic nephroma: a case report. ( 23012173 )
2008
42
Congenital mesoblastic nephroma rich from mitosis after in vitro fertilization: a case report. ( 18485932 )
2008
43
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
44
Atypical congenital mesoblastic nephroma presenting in the perinatal period. ( 17093992 )
2007
45
A case of fetal congenital mesoblastic nephroma with oligohydramnios. ( 17449950 )
2007
46
Congenital mesoblastic nephroma presenting with massive hematuria and hemorrhagic shock: report of one case. ( 17078466 )
2006
47
Expression of ETV6-NTRK in classical, cellular and mixed subtypes of congenital mesoblastic nephroma. ( 16681692 )
2006
48
Prenatal diagnosis of congenital mesoblastic nephroma associated with renal hypertension in a premature child. ( 15711357 )
2005
49
Congenital mesoblastic nephroma presenting with neonatal hypertension. ( 15858607 )
2005
50
Cellular congenital mesoblastic nephroma with contralateral medullary nephrocalcinosis. ( 15121709 )
2004

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

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 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 positive regulation of MAPK cascade GO:0043410 9.33 IGF2 KIT NTRK3
3 regulation of gene expression by genetic imprinting GO:0006349 9.26 CTCF IGF2
4 positive regulation of phospholipase C activity GO:0010863 8.96 KIT NTRK3
5 positive regulation of gene expression GO:0010628 8.92 CTCF KIT NTRK3 WT1

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
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
Content
Loading form....