MCID: CYS007
MIFTS: 39

Cystic Nephroma

Categories: Cancer diseases, Nephrological diseases

Aliases & Classifications for Cystic Nephroma

MalaCards integrated aliases for Cystic Nephroma:

Name: Cystic Nephroma 12 15 17
Benign Cystic Nephroma 74

Classifications:



External Ids:

Disease Ontology 12 DOID:2673
UMLS 74 C1266138

Summaries for Cystic Nephroma

MalaCards based summary : Cystic Nephroma, also known as benign cystic nephroma, is related to embryonal rhabdomyosarcoma and rhabdomyosarcoma. An important gene associated with Cystic Nephroma is DICER1 (Dicer 1, Ribonuclease III), and among its related pathways/superpathways are Dendritic Cells Developmental Lineage Pathway and Striated Muscle Contraction. The drugs Ifosfamide and Carboplatin have been mentioned in the context of this disorder. Affiliated tissues include kidney, liver and lung, and related phenotypes are Increased shRNA abundance (Z-score > 2) and Increased shRNA abundance (Z-score > 2)

Wikipedia : 77 A cystic nephroma, also known as multilocular cystic nephroma, mixed epithelial stromal tumour (MEST)... more...

Related Diseases for Cystic Nephroma

Diseases in the Cystic Nephroma family:

Malignant Cystic Nephroma

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

(show top 50) (show all 162)
# Related Disease Score Top Affiliating Genes
1 embryonal rhabdomyosarcoma 30.0 DES DICER1
2 rhabdomyosarcoma 29.9 DES DICER1 VIM
3 angiomyolipoma 29.8 DES KIT PGR
4 sarcoma 29.6 KIT VIM WT1
5 renal cell carcinoma, nonpapillary 29.5 KIT MME VIM WT1
6 malignant mesenchymoma 29.5 DES KIT WT1
7 malignant cystic nephroma 12.4
8 pleuropulmonary blastoma 11.7
9 dicer1-related pleuropulmonary blastoma cancer predisposition syndrome 11.6
10 mixed epithelial stromal tumour 11.4
11 childhood multilocular cystic kidney neoplasm 11.2
12 multiloculated renal cyst 10.3
13 reproductive organ benign neoplasm 10.2 DICER1 PGR
14 myxoid leiomyosarcoma 10.2 MME PGR
15 cystadenoma 10.2 MME PGR
16 cervical carcinosarcoma 10.2 MME PGR
17 wilms tumor 5 10.2
18 wilms tumor 6 10.2
19 intravenous leiomyomatosis 10.2 DES MME
20 endometrial stromal nodule 10.2 MME PGR
21 spermatocele 10.2 MME PGR
22 malignant leydig cell tumor 10.2 KIT MME
23 breast benign neoplasm 10.2 MME PGR
24 rete testis adenocarcinoma 10.2 KIT MME
25 thoracic benign neoplasm 10.2 MME PGR
26 pulmonary blastoma 10.2 DES DICER1
27 endometrial stromal tumor 10.2 MME PGR
28 gynandroblastoma 10.2 DICER1 VIM
29 cribriform carcinoma 10.2 KIT PGR
30 pancreatic mucinous cystadenoma 10.2 MME PGR
31 adenoid basal cell carcinoma 10.2 KIT PGR
32 breast adenoid cystic carcinoma 10.2 KIT PGR
33 rete testis neoplasm 10.2 KIT MME
34 ovarian fibrothecoma 10.1 DES KIT
35 gallbladder sarcoma 10.1 DES KIT
36 adenofibroma 10.1 MME VIM
37 pulmonary vein stenosis 10.1 DES KIT
38 breast fibroadenoma 10.1 MME PGR
39 renal oncocytoma 10.1 KIT MME
40 endometrial mucinous adenocarcinoma 10.1 PGR VIM
41 clear cell meningioma 10.1 PGR VIM
42 adenomyosis 10.1 MME PGR
43 infantile digital fibromatosis 10.1 DES VIM
44 angiolipoma 10.1 DES VIM
45 primitive neuroectodermal tumor of the cervix uteri 10.1 DES VIM
46 mast-cell sarcoma 10.1 KIT VIM
47 spindle cell lipoma 10.1 DES VIM
48 parachordoma 10.1 DES VIM
49 chordoma 10.1 DES VIM
50 syringocystadenoma papilliferum 10.1 DES VIM

Graphical network of the top 20 diseases related to Cystic Nephroma:



Diseases related to Cystic Nephroma

Symptoms & Phenotypes for Cystic Nephroma

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

27 (show all 24)
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Increased shRNA abundance (Z-score > 2) GR00366-A-100 9.78 WT1
2 Increased shRNA abundance (Z-score > 2) GR00366-A-102 9.78 KIT
3 Increased shRNA abundance (Z-score > 2) GR00366-A-105 9.78 VIM
4 Increased shRNA abundance (Z-score > 2) GR00366-A-122 9.78 WT1
5 Increased shRNA abundance (Z-score > 2) GR00366-A-146 9.78 VIM
6 Increased shRNA abundance (Z-score > 2) GR00366-A-152 9.78 KIT WT1
7 Increased shRNA abundance (Z-score > 2) GR00366-A-157 9.78 KIT
8 Increased shRNA abundance (Z-score > 2) GR00366-A-170 9.78 KIT
9 Increased shRNA abundance (Z-score > 2) GR00366-A-176 9.78 VIM
10 Increased shRNA abundance (Z-score > 2) GR00366-A-177 9.78 VIM
11 Increased shRNA abundance (Z-score > 2) GR00366-A-189 9.78 VIM
12 Increased shRNA abundance (Z-score > 2) GR00366-A-190 9.78 VIM
13 Increased shRNA abundance (Z-score > 2) GR00366-A-214 9.78 WT1
14 Increased shRNA abundance (Z-score > 2) GR00366-A-23 9.78 WT1
15 Increased shRNA abundance (Z-score > 2) GR00366-A-29 9.78 VIM
16 Increased shRNA abundance (Z-score > 2) GR00366-A-30 9.78 KIT VIM WT1
17 Increased shRNA abundance (Z-score > 2) GR00366-A-35 9.78 WT1
18 Increased shRNA abundance (Z-score > 2) GR00366-A-42 9.78 KIT
19 Increased shRNA abundance (Z-score > 2) GR00366-A-43 9.78 WT1
20 Increased shRNA abundance (Z-score > 2) GR00366-A-47 9.78 WT1
21 Increased shRNA abundance (Z-score > 2) GR00366-A-63 9.78 VIM
22 Increased shRNA abundance (Z-score > 2) GR00366-A-85 9.78 KIT VIM WT1
23 Increased shRNA abundance (Z-score > 2) GR00366-A-9 9.78 KIT
24 Increased shRNA abundance (Z-score > 2) GR00366-A-99 9.78 KIT

MGI Mouse Phenotypes related to Cystic Nephroma:

47
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 9.91 DES DICER1 KIT PGR VIM WT1
2 cellular MP:0005384 9.88 DES DICER1 KIT PGR VIM WT1
3 homeostasis/metabolism MP:0005376 9.87 DES DICER1 KIT MME PGR VIM
4 embryo MP:0005380 9.77 DICER1 KIT PGR VIM WT1
5 immune system MP:0005387 9.73 DICER1 KIT MME PGR VIM WT1
6 muscle MP:0005369 9.63 DES DICER1 KIT PGR VIM WT1
7 neoplasm MP:0002006 9.26 DICER1 KIT PGR WT1
8 reproductive system MP:0005389 9.02 DICER1 KIT PGR VIM WT1

Drugs & Therapeutics for Cystic Nephroma

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

(show all 18)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Ifosfamide Approved Phase 1, Phase 2 3778-73-2 3690
2
Carboplatin Approved Phase 1, Phase 2 41575-94-4 38904 10339178 498142
3
Dasatinib Approved, Investigational Phase 1, Phase 2 302962-49-8 3062316
4
Etoposide Approved Phase 1, Phase 2 33419-42-0 36462
5
Mechlorethamine Approved, Investigational Phase 1, Phase 2 51-75-2 4033
6
Isophosphamide mustard Phase 1, Phase 2 0
7 Protein Kinase Inhibitors Phase 1, Phase 2
8 Antineoplastic Agents, Alkylating Phase 1, Phase 2
9 Etoposide phosphate Phase 1, Phase 2
10 Topoisomerase Inhibitors Phase 1, Phase 2
11 Alkylating Agents Phase 1, Phase 2
12 Liver Extracts Phase 1, Phase 2
13 Antineoplastic Agents, Phytogenic Phase 1, Phase 2
14
Dactinomycin Approved, Investigational 50-76-0 457193 2019
15
Doxorubicin Approved, Investigational 23214-92-8 31703
16
Cyclophosphamide Approved, Investigational 6055-19-2, 50-18-0 2907
17
Vincristine Approved, Investigational 2068-78-2, 57-22-7 5978
18
Doxil Approved June 1999 31703

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 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
2 Gene Mutation in Samples From Young Patients With Pleuropulmonary Blastoma Syndrome at Risk for Developing Cancer Completed NCT01353300
3 DICER1-related Pleuropulmonary Blastoma Cancer Predisposition Syndrome: A Natural History Study Recruiting NCT01247597
4 International PPB Registry for PPB, DICER1 and Associated Conditions Recruiting NCT03382158
5 Elucidating the Genetic Basis of the Pleuropulmonary Blastoma (PPB) Familial Cancer Syndrome Active, not recruiting NCT00565903

Search NIH Clinical Center for Cystic Nephroma

Genetic Tests for Cystic Nephroma

Anatomical Context for Cystic Nephroma

MalaCards organs/tissues related to Cystic Nephroma:

42
Kidney, Liver, Lung

Publications for Cystic Nephroma

Articles related to Cystic Nephroma:

(show top 50) (show all 137)
# Title Authors Year
1
Cystic nephroma: A case report in adult patients. ( 29799412 )
2018
2
Ultrasound Imaging of Cystic Nephroma. ( 28752022 )
2017
3
Identification of somatic and germ-line DICER1 mutations in pleuropulmonary blastoma, cystic nephroma and rhabdomyosarcoma tumors within a DICER1 syndrome pedigree. ( 28222777 )
2017
4
A Case Report of Largest Documented Multilocular Cystic Nephroma Removed by Thoracoabdominal Approach. ( 28892971 )
2017
5
Pediatric Cystic Nephroma Is Morphologically, Immunohistochemically, and Genetically Distinct From Adult Cystic Nephroma. ( 28177962 )
2017
6
A rare presentation of cystic nephroma in a young adult. ( 29264218 )
2017
7
Cystic Nephroma in Adults: A Clinicopathologic Study of 46 Cases. ( 27831959 )
2016
8
Imagistic and histopathological description of a cystic nephroma during early second trimester of gestation. Case report. ( 28845501 )
2016
9
Tumor Progression in DICER1-mutated Cystic Nephroma - witnessing the genesis of Anaplastic Sarcoma of the Kidney. ( 27036314 )
2016
10
Multilocular Cystic Renal Cell Carcinoma or Cystic Nephroma? ( 28074169 )
2016
11
Cystic nephroma of the kidney. ( 25444959 )
2015
12
A rare case of bilateral cystic nephroma associated with embryonal rhabdomyosarcoma of the penile urethra. ( 25829672 )
2015
13
Multilocular Cystic Nephroma: A Systematic Literature Review of the Radiologic and Clinical Findings. ( 26587924 )
2015
14
An Unusual Presentation of Cystic Nephroma in an Adult Man. ( 26266015 )
2015
15
Laparoscopic Partial Nephrectomy for the Treatment of Large Cystic Nephroma in Children. ( 25265482 )
2014
16
Quiz page JULY 2014: bilateral abdominal masses in an infant. Diagnosis: Bilateral cystic nephroma. ( 24954459 )
2014
17
Case 211: Pleuropulmonary Blastoma in Association with Cystic Nephroma-DICER1 Syndrome. ( 25340273 )
2014
18
Multilocular cystic nephroma: A clinical case. ( 24892395 )
2014
19
Uretheral invagination of multilocular cystic nephroma; a case report of a new pathologic variant. ( 25197409 )
2014
20
Multilocular cystic nephroma treated with laparoscopic nephron-sparing surgery: A case report. ( 25210560 )
2014
21
Cystic Nephroma/Mixed Epithelial Stromal Tumor: A Benign Neoplasm With Potential for Recurrence. ( 25525149 )
2014
22
DICER1 mutations in childhood cystic nephroma and its relationship to DICER1-renal sarcoma. ( 24481001 )
2014
23
Case report: Multimodality imaging of a cystic nephroma. ( 23861515 )
2013
24
Renal metastasis of an ovarian granulosa cell tumour inducing growth of a cystic nephroma. ( 23843415 )
2013
25
Adult multilocular cystic nephroma: Report of six cases with clinical, radio-pathologic correlation and review of literature. ( 23662002 )
2013
26
Can multilocular cystic nephroma be a harbinger of multilocular cystic renal cell carcinoma? ( 23032854 )
2012
27
Coexistence of cystic nephroma and neuroblastoma: a rare case of a childhood collision tumor. ( 22395847 )
2012
28
Asymptomatic infantile cystic nephroma: a diagnostic dilemma. ( 24293912 )
2012
29
Bilateral cystic nephroma with pleuropulmonary blastoma. ( 22688934 )
2011
30
Cystic nephroma: a diagnostic dilemma. ( 20427881 )
2010
31
Cystic nephroma: a fine needle aspiration diagnostic pitfall. ( 20391987 )
2010
32
Germline DICER1 mutations and familial cystic nephroma. ( 21036787 )
2010
33
Bilateral progressive cystic nephroma in a 9-month-old male infant requiring renal replacement therapy. ( 20414683 )
2010
34
What is a cystic nephroma? ( 20035151 )
2010
35
Cystic nephroma masquerading as hydatid cyst of the kidney. ( 21045463 )
2010
36
Re: Rodolfo Montironi, Roberta Mazzuccelli, Antonio Lopez-Beltran, et al. Cystic nephroma and mixed epithelial and stromal tumour of the kidney: opposite ends of the spectrum of the same entity? Eur Urol 2008;54:1237-46. ( 19167806 )
2009
37
Adult cystic nephroma: a case report and a review of the literature. ( 18555707 )
2009
38
Adult cystic nephroma and mixed epithelial and stromal tumor of the kidney are the same disease entity: molecular and histologic evidence. ( 18971776 )
2009
39
Cystic nephroma, cystic partially differentiated nephroblastoma and cystic Wilms' tumor in children: a spectrum with therapeutic dilemmas. ( 19172100 )
2009
40
Cystic nephroma in childhood. ( 19661676 )
2009
41
Multilocular cystic nephroma and multicystic clear cell carcinoma: two faces of the Roman god Janus? ( 18611925 )
2009
42
Best cases from the AFIP: Multilocular cystic renal tumor: cystic nephroma. ( 18635639 )
2008
43
Multilocular cystic nephroma of the kidney. ( 19008600 )
2008
44
Cystic nephroma: a case report and review of the literature. ( 18947428 )
2008
45
Sonographic diagnosis of an unusual case of multilocular cystic nephroma mimicking polycystic kidney disease. ( 19034827 )
2008
46
Cystic nephroma and mixed epithelial and stromal tumour of the kidney: opposite ends of the spectrum of the same entity? ( 18006141 )
2008
47
Failure of percutaneous endoscopic resection of a renal cystic nephroma on longer-term follow-up. ( 18613780 )
2008
48
Cystic nephroma and localized renal cystic disease in children: diagnostic clues and management. ( 18970929 )
2008
49
Pleuropulmonary blastema with cystic nephroma - A rare presentation and surgical dilemma. ( 19057859 )
2008
50
Editorial comment on: cystic nephroma and mixed epithelial and stromal tumour of the kidney: opposite ends of the spectrum of the same entity? ( 18006142 )
2008

Variations for Cystic Nephroma

Expression for Cystic Nephroma

Search GEO for disease gene expression data for Cystic Nephroma.

Pathways for Cystic Nephroma

Pathways related to Cystic Nephroma according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1 11.19 KIT MME
2 11.11 DES VIM
3 10.88 KIT MME
4 10.67 MME VIM WT1
5
Show member pathways
10.61 DES VIM

GO Terms for Cystic Nephroma

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

# Name GO ID Score Top Affiliating Genes
1 cytoplasm GO:0005737 9.17 DES DICER1 KIT MME PGR VIM

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

# Name GO ID Score Top Affiliating Genes
1 positive regulation of gene expression GO:0010628 9.33 KIT VIM WT1
2 muscle filament sliding GO:0030049 8.96 DES VIM
3 intermediate filament organization GO:0045109 8.62 DES VIM

Molecular functions related to Cystic Nephroma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 double-stranded RNA binding GO:0003725 8.62 DICER1 VIM

Sources for Cystic Nephroma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
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