MCID: NPH113
MIFTS: 37

Nephroma

Categories: Cancer diseases, Nephrological diseases

Aliases & Classifications for Nephroma

MalaCards integrated aliases for Nephroma:

Name: Nephroma 12 15
Benign Nephroma 12

Classifications:



External Ids:

Disease Ontology 12 DOID:0080615

Summaries for Nephroma

Disease Ontology : 12 A kidney benign neoplasm that is located in the kidney cortex.

MalaCards based summary : Nephroma, also known as benign nephroma, is related to cellular congenital mesoblastic nephroma and congenital mesoblastic nephroma. An important gene associated with Nephroma is DICER1 (Dicer 1, Ribonuclease III), and among its related pathways/superpathways are Cytoskeletal Signaling and Cardiac Progenitor Differentiation. The drugs Fentanyl and Pancreatic Polypeptide have been mentioned in the context of this disorder. Affiliated tissues include kidney cortex, kidney and cortex, and related phenotypes are Decreased shRNA abundance (Z-score < -2) and Decreased shRNA abundance (Z-score < -2)

Related Diseases for Nephroma

Diseases related to Nephroma via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 371)
# Related Disease Score Top Affiliating Genes
1 cellular congenital mesoblastic nephroma 34.4 NTRK3 ETV6
2 congenital mesoblastic nephroma 33.7 WT1 NTRK3 KIT IGF2 ETV6
3 adult cystic nephroma 32.6 PGR MME MEST KIT DES ACTC1
4 infantile myofibromatosis 32.5 NTRK3 ETV6 DES ACTC1
5 pleuropulmonary blastoma 32.4 U2AF1 IGF2 DICER1 DES
6 mixed epithelial stromal tumour 32.1 MME MEST
7 wilms tumor 1 31.3 WT1 U2AF1 PGR KIT IGF2 DICER1
8 congenital fibrosarcoma 31.0 NTRK3 ETV6 DES ACTC1
9 rare tumor 30.9 KIT DES ACTC1
10 nephrogenic adenofibroma 30.8 WT1 TFE3
11 kidney sarcoma 30.8 WT1 DICER1
12 breast secretory carcinoma 30.6 NTRK3 ETV6
13 fibrosarcoma 30.5 NTRK3 KIT ETV6 DES ACTC1
14 clear cell papillary renal cell carcinoma 30.4 TFE3 MME
15 clear cell sarcoma 30.4 WT1 TFE3 KIT IGF2
16 hemihyperplasia, isolated 30.4 WT1 IGF2
17 metanephric adenoma 30.3 WT1 TFE3 MUC1
18 spindle cell sarcoma 30.3 NTRK3 MUC1 DES ACTC1
19 lymphangioleiomyomatosis 30.1 PGR IGF2 DES
20 rhabdoid tumor predisposition syndrome 1 30.1 WT1 MUC1 IGF2 DES ACTC1
21 mesenchymal cell neoplasm 30.1 TFE3 NTRK3 MME KIT
22 cystadenocarcinoma 30.1 PGR MUC1 ACTC1
23 fibroma 30.1 KRT19 DES ACTC1
24 embryonal rhabdomyosarcoma 29.9 IGF2 DICER1 DES ACTC1
25 polycystic liver disease 1 with or without kidney cysts 29.9 MUC1 KRT19 ACTC1
26 adenofibroma 29.7 WT1 MUC1 MME KRT19 ACTC1
27 rhabdomyosarcoma 29.5 WT1 KIT IGF2 DICER1 DES ACTC1
28 myelofibrosis 29.5 WT1 U2AF1 KIT ETV6
29 angiomyolipoma 29.4 TFE3 PGR MME KIT DES ACTC1
30 leiomyoma 29.4 PGR MME KIT IGF2 DES ACTC1
31 renal cell carcinoma, nonpapillary 29.3 WT1 TFE3 MUC1 MME KRT19 KIT
32 sarcoma 29.1 WT1 TFE3 MUC1 KIT DICER1 DES
33 renal cell carcinoma, papillary, 1 29.1 WT1 TFE3 MME KRT19 KIT
34 malignant cystic nephroma 12.4
35 classic congenital mesoblastic nephroma 12.3
36 wilms tumor 5 11.8
37 dicer1 tumor predisposition 11.5
38 childhood multilocular cystic kidney neoplasm 11.5
39 dicer1-related pleuropulmonary blastoma cancer predisposition syndrome 11.5
40 myofibromatosis, infantile, 1 11.2
41 graham boyle troxell syndrome 11.2
42 polyhydramnios 10.6
43 microcystic stromal tumor 10.5 WT1 DICER1
44 lymphangiomatosis 10.5 PGR DES
45 adult fibrosarcoma 10.5 NTRK3 ETV6
46 fibroblastic rheumatism 10.5 DES ACTC1
47 congenital epulis 10.5 PGR DES
48 epulis 10.5 PGR DES
49 embryoma 10.5
50 cervical adenomyoma 10.5 PGR MME

Graphical network of the top 20 diseases related to Nephroma:



Diseases related to Nephroma

Symptoms & Phenotypes for Nephroma

GenomeRNAi Phenotypes related to Nephroma according to GeneCards Suite gene sharing:

26 (show all 15)
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Decreased shRNA abundance (Z-score < -2) GR00366-A-102 9.55 KIT
2 Decreased shRNA abundance (Z-score < -2) GR00366-A-109 9.55 NTRK3
3 Decreased shRNA abundance (Z-score < -2) GR00366-A-125 9.55 MEST
4 Decreased shRNA abundance (Z-score < -2) GR00366-A-130 9.55 NTRK3
5 Decreased shRNA abundance (Z-score < -2) GR00366-A-152 9.55 MEST
6 Decreased shRNA abundance (Z-score < -2) GR00366-A-165 9.55 KIT MEST
7 Decreased shRNA abundance (Z-score < -2) GR00366-A-18 9.55 KIT
8 Decreased shRNA abundance (Z-score < -2) GR00366-A-184 9.55 NTRK3
9 Decreased shRNA abundance (Z-score < -2) GR00366-A-2 9.55 MEST
10 Decreased shRNA abundance (Z-score < -2) GR00366-A-214 9.55 MEST
11 Decreased shRNA abundance (Z-score < -2) GR00366-A-23 9.55 MEST WT1
12 Decreased shRNA abundance (Z-score < -2) GR00366-A-35 9.55 WT1
13 Decreased shRNA abundance (Z-score < -2) GR00366-A-42 9.55 KIT
14 Decreased shRNA abundance (Z-score < -2) GR00366-A-55 9.55 NTRK3
15 Decreased shRNA abundance (Z-score < -2) GR00366-A-92 9.55 MEST

MGI Mouse Phenotypes related to Nephroma:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 10.26 ACTC1 DES DICER1 ETV6 IGF2 KIT
2 cellular MP:0005384 10.2 ACTC1 DES DICER1 ETV6 IGF2 KIT
3 growth/size/body region MP:0005378 10.14 ACTC1 DICER1 ETV6 IGF2 KIT KRT19
4 mortality/aging MP:0010768 10.1 ACTC1 DES DICER1 ETV6 IGF2 KIT
5 embryo MP:0005380 10.03 DICER1 ETV6 IGF2 KIT KRT19 MEST
6 integument MP:0010771 9.97 DICER1 ETV6 IGF2 KIT KRT19 MME
7 muscle MP:0005369 9.91 ACTC1 DES DICER1 IGF2 KIT MEST
8 neoplasm MP:0002006 9.63 DICER1 ETV6 KIT KRT19 PGR WT1
9 no phenotypic analysis MP:0003012 9.56 DICER1 ETV6 IGF2 KIT KRT19 NTRK3
10 normal MP:0002873 9.28 ACTC1 DICER1 ETV6 KIT KRT19 NTRK3

Drugs & Therapeutics for Nephroma

Drugs for 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 Liver Extracts Phase 3
4 Anesthetics Phase 3
5 Narcotics Phase 3
6 Cola Phase 3
7 Analgesics Phase 3
8 Analgesics, Opioid Phase 3
9 Anesthetics, General Phase 3
10 Anesthetics, Intravenous Phase 3
11
Entrectinib Approved, Investigational Phase 1, Phase 2 1108743-60-7 25141092
12
Ifosfamide Approved Phase 1, Phase 2 3778-73-2 3690
13
Dasatinib Approved, Investigational Phase 1, Phase 2 302962-49-8 3062316
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
Tyrosine Approved, Investigational, Nutraceutical Phase 1, Phase 2 60-18-4 6057
17 Protein Kinase Inhibitors Phase 1, Phase 2
18
Isophosphamide mustard Phase 1, Phase 2 0
19 Etoposide phosphate Phase 1, Phase 2
20 Alkylating Agents Phase 1, Phase 2
21
Vincristine Approved, Investigational 57-22-7, 2068-78-2 5978
22
Doxorubicin Approved, Investigational 23214-92-8 31703
23
Dactinomycin Approved, Investigational 50-76-0 457193 2019
24
Cyclophosphamide Approved, Investigational 50-18-0, 6055-19-2 2907
25
Liposomal doxorubicin 31703

Interventional clinical trials:

(show all 12)
# 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 A Phase 1/2, Open-Label, Dose-Escalation And Expansion Study Of Entrectinib (Rxdx-101) In Pediatrics With Locally Advanced Or Metastatic Solid Or Primary CNS Tumors And/Or Who Have No Satisfactory Treatment Options Recruiting NCT02650401 Phase 1, Phase 2 Entrectinib
3 Dasatinib With Ifosfamide, Carboplatin, Etoposide: A Pediatric Phase I/II Trial Active, not recruiting NCT00788125 Phase 1, Phase 2 carboplatin;dasatinib;etoposide phosphate;ifosfamide
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 Investigation of DICER1 in Cystic Nephroma and Cystic Partially Differentiated Nephroblastoma Completed NCT01353300
6 The Project: EveryChild Protocol: A Registry, Eligibility Screening, Biology and Outcome Study Recruiting NCT02402244
7 DICER1-Related Pleuropulmonary Blastoma Cancer Predisposition Syndrome: A Natural History Study Recruiting NCT01247597
8 International Pleuropulmonary Blastoma Registry for PPB, DICER1 and Associated Conditions Recruiting NCT03382158
9 Elucidating the Genetic Basis of the Pleuropulmonary Blastoma (PPB) Familial Cancer Syndrome Active, not recruiting NCT00565903
10 Renal Tumors Classification, Biology, and Banking Study Suspended NCT00898365
11 Protocol for the Enrollment on the Official COG Registry, The Childhood Cancer Research Network (CCRN) Children s Oncology Group Terminated NCT01117168
12 Focal Adhesion Kinase Expression in Pediatric Renal Tumors Withdrawn NCT01642095

Search NIH Clinical Center for Nephroma

Genetic Tests for Nephroma

Anatomical Context for Nephroma

The Foundational Model of Anatomy Ontology organs/tissues related to Nephroma:

19
Kidney Cortex

MalaCards organs/tissues related to Nephroma:

40
Kidney, Cortex, Liver, Lung, Bone, Brain, Testes

Publications for Nephroma

Articles related to Nephroma:

(show top 50) (show all 967)
# Title Authors PMID Year
1
Cystic partially differentiated nephroblastoma: a rare pediatric renal tumor-case report. 61
32528602 2020
2
Recurrent EGFR alterations in NTRK3 fusion negative congenital mesoblastic nephroma. 61
32490123 2020
3
NTRK-rearranged mesenchymal tumours: diagnostic challenges, morphological patterns and proposed testing algorithm. 61
32278476 2020
4
Cellular mesoblastic nephroma in infants and children: Report of four cases and review of the literature. 61
31120379 2020
5
DICER1-Related Embryonal Rhabdomyosarcoma of the Uterine Corpus in a Prepubertal Girl. 61
31838154 2020
6
An update on the central nervous system manifestations of DICER1 syndrome. 61
30953130 2020
7
Can contrast enhanced ultrasound differentiate benign cystic nephroma from malignant multicystic renal lesions? A case report. 61
31867215 2020
8
The fungal community and its interaction with the concentration of short-chain fatty acids in the faeces of Chenghua, Yorkshire and Tibetan pigs. 61
31691493 2020
9
DICER1 screening in 15 paediatric paratesticular sarcomas unveils an unusual DICER1-associated sarcoma. 61
32222066 2020
10
Twenty children with non-Wilms renal tumors from a reference center in Central Anatolia, Turkey 61
31655501 2020
11
Congenital mesoblastic nephroma: a single-centre series. 61
31508997 2020
12
Diagnostic Utility of BCOR Antibody in Clear Cell Sarcomas of Kidney. 61
31875746 2019
13
Lessons learned from the developmental origins of childhood renal cancer. 61
31738020 2019
14
[Rare childhood kidney tumors]. 61
31338565 2019
15
Open Radical Nephrectomy for Suspected Renal Malignancy-Tips and Tricks. 61
31419431 2019
16
Re: Recurrent and Metastatic Congenital Mesoblastic Nephroma: Where Does the Evidence Stand? 61
31343939 2019
17
Imaging of DICER1 syndrome. 61
31620849 2019
18
Laparoscopic management of congenital mesoblastic nephroma- case report. 61
31388496 2019
19
Prenatal diagnosis of congenital mesoblastic nephroma: a case with poor prognosis. 61
31466985 2019
20
Unusual Case of Concurrent Retroperitoneal Congenital Infantile Fibrosarcoma and Cellular Type Congenital Mesoblastic Nephroma. 61
31343481 2019
21
Paraneoplastic disorders associated with miscellaneous neoplasms with focus on selected soft tissue and Undifferentiated/ rhabdoid malignancies. 61
30819530 2019
22
Pleuropulmonary Blastoma: More Than a Lung Neoplasm of Childhood. 61
31527943 2019
23
Pediatric Renal Neoplasms:: MR Imaging-Based Practical Diagnostic Approach. 61
30910098 2019
24
The clinical presentation, imaging features and differential diagnoses of congenital Wilms tumour. 61
31151974 2019
25
Multiple Abusive Fractures in an Infant With a Concurrent Parathyroid Hormone-Related Peptide-Secreting Renal Tumor: Abusive Fractures Accompanying a Parathyroid Hormone-Related Peptide-Secreting Tumor. 61
30973495 2019
26
Anaplastic sarcoma of the kidney: Case report and literature review. 61
31007495 2019
27
Antiproliferative and antibiofilm potentials of endolichenic fungi associated with the lichen Nephroma laevigatum. 61
30597735 2019
28
[Cystic nephroma : a rare kidney neoplasm]. 61
30897310 2019
29
Neoplasm Risk Among Individuals With a Pathogenic Germline Variant in DICER1. 61
30715996 2019
30
Neurotrophic tropomyosin or tyrosine receptor kinase (NTRK) genes. 61
30636697 2019
31
TRK Fusion Cancers in Children: A Clinical Review and Recommendations for Screening. 61
30592640 2019
32
Contemporary update on imaging of cystic renal masses with histopathological correlation and emphasis on patient management. 61
30314810 2019
33
Expression of cyclin D1 in clear cell sarcoma of kidney. Is it useful in differentiating it from its histological mimics? 61
30736802 2019
34
Identification of two 14q32 deletions involving DICER1 associated with the development of DICER1-related tumors. 61
29698806 2019
35
DICER1 Syndrome. 61
31409088 2019
36
Reprint of Efficient fungal UV-screening provides a remarkably high UV-B tolerance of photosystem II in lichen photobionts. 61
30337144 2019
37
A Case Report: Neonatal Torsional Ovarian Cyst. 61
32377121 2019
38
[WT1-positive congential mesoblastic nephroma: report of a case]. 61
30522186 2018
39
Characterization and management of various renal cystic lesions by sonographic features. 61
30017809 2018
40
Structural renal abnormalities in the DICER1 syndrome: a family-based cohort study. 61
30178239 2018
41
Specific computed tomography imaging characteristics of congenital mesoblastic nephroma and correlation with ultrasound and pathology. 61
30145031 2018
42
Sorting the Alphabet Soup of Renal Pathology: A Review. 61
26928791 2018
43
Climate-woodland effects on population genetics for two congeneric lichens with contrasting reproductive strategies. 61
30107505 2018
44
Magnetic resonance imaging (MRI) of the renal sinus. 61
29632991 2018
45
Efficient fungal UV-screening provides a remarkably high UV-B tolerance of photosystem II in lichen photobionts. 61
30176432 2018
46
Dramatic bone remodeling following larotrectinib administration for bone metastasis in a patient with TRK fusion congenital mesoblastic nephroma. 61
29893456 2018
47
Estimated glomerular filtration rate after nephrectomy for Wilms tumor. 61
30003640 2018
48
Wilms tumor, pleuropulmonary blastoma, and DICER1: case report and literature review. 61
30097050 2018
49
Primary intracranial spindle cell sarcoma with rhabdomyosarcoma-like features share a highly distinct methylation profile and DICER1 mutations. 61
29881993 2018
50
A retrospective cohort study of risk factors and pregnancy outcomes in 14,014 Chinese pregnant women. 61
30113460 2018

Variations for Nephroma

Expression for Nephroma

Search GEO for disease gene expression data for Nephroma.

Pathways for Nephroma

Pathways related to Nephroma according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1 11.75 MUC1 KRT19 DES ACTC1
2 10.52 KIT IGF2 ACTC1

GO Terms for Nephroma

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

# Name GO ID Score Top Affiliating Genes
1 extracellular exosome GO:0070062 9.23 THBS4 MUC1 MME MEST KRT19 DICER1

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

# Name GO ID Score Top Affiliating Genes
1 positive regulation of MAPK cascade GO:0043410 9.13 NTRK3 KIT IGF2
2 positive regulation of phospholipase C activity GO:0010863 8.62 NTRK3 KIT

Sources for 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
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