MCID: CYS007
MIFTS: 37

Cystic Nephroma

Categories: Nephrological diseases, Cancer diseases

Aliases & Classifications for Cystic Nephroma

MalaCards integrated aliases for Cystic Nephroma:

Name: Cystic Nephroma 12 14
Benign Cystic Nephroma 69

Classifications:



External Ids:

Disease Ontology 12 DOID:2673
UMLS 69 C1266138

Summaries for Cystic Nephroma

MalaCards based summary : Cystic Nephroma, also known as benign cystic nephroma, is related to embryonal rhabdomyosarcoma and renal cell carcinoma, nonpapillary. 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 Primary Focal Segmental Glomerulosclerosis FSGS. 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 cardiovascular system and cellular

Wikipedia : 72 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 145)
# Related Disease Score Top Affiliating Genes
1 embryonal rhabdomyosarcoma 29.9 DES DICER1
2 renal cell carcinoma, nonpapillary 29.6 KIT MME WT1
3 malignant mesenchymoma 29.1 DES KIT WT1
4 angiomyolipoma 28.8 ACTC1 DES KIT PGR
5 malignant cystic nephroma 12.3
6 pleuropulmonary blastoma 11.6
7 dicer1-related pleuropulmonary blastoma cancer predisposition syndrome 11.4
8 mixed epithelial stromal tumour 11.2
9 sertoli-leydig cell tumor 10.3 DICER1 PGR
10 cervical carcinosarcoma 10.3 MME PGR
11 reproductive organ benign neoplasm 10.3 DICER1 PGR
12 breast benign neoplasm 10.3 MME PGR
13 thoracic benign neoplasm 10.3 MME PGR
14 endometrial stromal nodule 10.3 MME PGR
15 spermatocele 10.3 MME PGR
16 intravenous leiomyomatosis 10.3 DES MME
17 pulmonary blastoma 10.3 DES DICER1
18 malignant leydig cell tumor 10.3 KIT MME
19 rete testis adenocarcinoma 10.3 KIT MME
20 pancreatic mucinous cystadenoma 10.2 MME PGR
21 lymphangiomatosis 10.2 DES PGR
22 breast metaplastic carcinoma 10.2 MME PGR
23 epulis 10.2 DES PGR
24 rete testis neoplasm 10.2 KIT MME
25 cribriform carcinoma 10.2 KIT PGR
26 malignant mixed mullerian tumor 10.2 KIT PGR
27 breast papillary carcinoma 10.2 MME PGR
28 blastoma 10.2
29 adenoid basal cell carcinoma 10.2 KIT PGR
30 breast angiosarcoma 10.2 KIT PGR
31 uterine body mixed cancer 10.2 KIT PGR
32 breast adenoid cystic carcinoma 10.2 KIT PGR
33 sweat gland cancer 10.2 MME PGR
34 cystadenoma 10.2 MME PGR
35 wilms tumor 6 10.2
36 gallbladder sarcoma 10.2 DES KIT
37 dermoid cyst 10.2 KIT PGR
38 renal oncocytoma 10.2 KIT MME
39 adenofibroma 10.2 ACTC1 MME
40 congenital epulis 10.2 DES PGR
41 breast fibroadenoma 10.2 MME PGR
42 calcifying aponeurotic fibroma 10.2 ACTC1 PGR
43 uterine carcinosarcoma 10.2 KIT PGR
44 bizarre leiomyoma 10.2 ACTC1 PGR
45 myxoid leiomyosarcoma 10.2 ACTC1 PGR
46 glycogen-rich clear cell breast carcinoma 10.1 ACTC1 PGR
47 renal cell carcinoma, papillary, 1 10.1 KIT MME
48 embryonal sarcoma 10.1 DES KIT
49 fibroblastic rheumatism 10.1 ACTC1 DES
50 angiolipoma 10.1 ACTC1 DES

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



Diseases related to Cystic Nephroma

Symptoms & Phenotypes for Cystic Nephroma

MGI Mouse Phenotypes related to Cystic Nephroma:

43
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 9.85 DES DICER1 KIT PGR WT1 ACTC1
2 cellular MP:0005384 9.8 DES DICER1 KIT PGR WT1 ACTC1
3 homeostasis/metabolism MP:0005376 9.8 DES DICER1 KIT MME PGR WT1
4 muscle MP:0005369 9.63 DICER1 KIT PGR WT1 ACTC1 DES
5 neoplasm MP:0002006 9.26 DICER1 KIT PGR WT1
6 normal MP:0002873 9.02 DICER1 KIT PGR WT1 ACTC1

Drugs & Therapeutics for Cystic Nephroma

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

(show all 17)
# 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 10339178 38904 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 Alkylating Agents Phase 1, Phase 2
8 Antineoplastic Agents, Phytogenic Phase 1, Phase 2
9 Etoposide phosphate Phase 1, Phase 2
10 Liver Extracts Phase 1, Phase 2
11 Protein Kinase Inhibitors Phase 1, Phase 2
12 Topoisomerase Inhibitors Phase 1, Phase 2
13
Cyclophosphamide Approved, Investigational 50-18-0, 6055-19-2 2907
14
Dactinomycin Approved, Investigational 50-76-0 457193 2019
15
Doxorubicin Approved, Investigational 23214-92-8 31703
16
Vincristine Approved, Investigational 2068-78-2, 57-22-7 5978
17
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 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:

38
Kidney, Liver, Lung

Publications for Cystic Nephroma

Articles related to Cystic Nephroma:

(show top 50) (show all 136)
# Title Authors Year
1
A Case Report of Largest Documented Multilocular Cystic Nephroma Removed by Thoracoabdominal Approach. ( 28892971 )
2017
2
Pediatric Cystic Nephroma Is Morphologically, Immunohistochemically, and Genetically Distinct From Adult Cystic Nephroma. ( 28177962 )
2017
3
A rare presentation of cystic nephroma in a young adult. ( 29264218 )
2017
4
Ultrasound Imaging of Cystic Nephroma. ( 28752022 )
2017
5
Identification of somatic and germ-line DICER1 mutations in pleuropulmonary blastoma, cystic nephroma and rhabdomyosarcoma tumors within a DICER1 syndrome pedigree. ( 28222777 )
2017
6
Pediatric cystic nephromas: distinctive features and frequent DICER1 mutations. ( 26772403 )
2016
7
Imagistic and histopathological description of a cystic nephroma during early second trimester of gestation. Case report. ( 28845501 )
2016
8
Tumor Progression in DICER1-mutated Cystic Nephroma - witnessing the genesis of Anaplastic Sarcoma of the Kidney. ( 27036314 )
2016
9
Multilocular Cystic Renal Cell Carcinoma or Cystic Nephroma? ( 28074169 )
2016
10
Cystic Nephroma in Adults: A Clinicopathologic Study of 46 Cases. ( 27831959 )
2016
11
An Unusual Presentation of Cystic Nephroma in an Adult Man. ( 26266015 )
2015
12
Multilocular Cystic Nephroma: A Systematic Literature Review of the Radiologic and Clinical Findings. ( 26587924 )
2015
13
A rare case of bilateral cystic nephroma associated with embryonal rhabdomyosarcoma of the penile urethra. ( 25829672 )
2015
14
Cystic nephroma of the kidney. ( 25444959 )
2015
15
Cystic Nephroma/Mixed Epithelial Stromal Tumor: A Benign Neoplasm With Potential for Recurrence. ( 25525149 )
2014
16
Multilocular cystic nephroma treated with laparoscopic nephron-sparing surgery: A case report. ( 25210560 )
2014
17
Uretheral invagination of multilocular cystic nephroma; a case report of a new pathologic variant. ( 25197409 )
2014
18
Laparoscopic Partial Nephrectomy for the Treatment of Large Cystic Nephroma in Children. ( 25265482 )
2014
19
DICER1 mutations in childhood cystic nephroma and its relationship to DICER1-renal sarcoma. ( 24481001 )
2014
20
Multilocular cystic nephroma: A clinical case. ( 24892395 )
2014
21
Quiz page JULY 2014: bilateral abdominal masses in an infant. Diagnosis: Bilateral cystic nephroma. ( 24954459 )
2014
22
Case report: Multimodality imaging of a cystic nephroma. ( 23861515 )
2013
23
Renal metastasis of an ovarian granulosa cell tumour inducing growth of a cystic nephroma. ( 23843415 )
2013
24
Adult multilocular cystic nephroma: Report of six cases with clinical, radio-pathologic correlation and review of literature. ( 23662002 )
2013
25
Can multilocular cystic nephroma be a harbinger of multilocular cystic renal cell carcinoma? ( 23032854 )
2012
26
Coexistence of cystic nephroma and neuroblastoma: a rare case of a childhood collision tumor. ( 22395847 )
2012
27
Asymptomatic infantile cystic nephroma: a diagnostic dilemma. ( 24293912 )
2012
28
Cystic nephroma masquerading as hydatid cyst of the kidney. ( 21045463 )
2010
29
Bilateral progressive cystic nephroma in a 9-month-old male infant requiring renal replacement therapy. ( 20414683 )
2010
30
Cystic nephroma: a fine needle aspiration diagnostic pitfall. ( 20391987 )
2010
31
Cystic nephroma: a diagnostic dilemma. ( 20427881 )
2010
32
What is a cystic nephroma? ( 20035151 )
2010
33
Germline DICER1 mutations and familial cystic nephroma. ( 21036787 )
2010
34
Multilocular cystic nephroma and multicystic clear cell carcinoma: two faces of the Roman god Janus? ( 18611925 )
2009
35
Adult cystic nephroma: a case report and a review of the literature. ( 18555707 )
2009
36
Adult cystic nephroma and mixed epithelial and stromal tumor of the kidney are the same disease entity: molecular and histologic evidence. ( 18971776 )
2009
37
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
38
[Clinicopathologic analysis of mixed epithelial and stromal tumor of kidney and adult cystic nephroma]. ( 19781188 )
2009
39
Cystic nephroma in childhood. ( 19661676 )
2009
40
Cystic nephroma, cystic partially differentiated nephroblastoma and cystic Wilms' tumor in children: a spectrum with therapeutic dilemmas. ( 19172100 )
2009
41
Best cases from the AFIP: Multilocular cystic renal tumor: cystic nephroma. ( 18635639 )
2008
42
Pleuropulmonary blastema with cystic nephroma - A rare presentation and surgical dilemma. ( 19057859 )
2008
43
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
44
Failure of percutaneous endoscopic resection of a renal cystic nephroma on longer-term follow-up. ( 18613780 )
2008
45
Cystic nephroma and mixed epithelial and stromal tumour of the kidney: opposite ends of the spectrum of the same entity? ( 18006141 )
2008
46
Sonographic diagnosis of an unusual case of multilocular cystic nephroma mimicking polycystic kidney disease. ( 19034827 )
2008
47
Cystic nephroma: a case report and review of the literature. ( 18947428 )
2008
48
Adult cystic nephroma and mixed epithelial and stromal tumor of the kidney: clinical, radiographic, and pathologic characteristics. ( 18313107 )
2008
49
Cytology of cystic nephroma: a case report. ( 18323282 )
2008
50
Multilocular cystic nephroma of the kidney. ( 19008600 )
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 11.13 MME WT1
3 11.01 ACTC1 DES
4 10.68 KIT MME
5 10.35 ACTC1 KIT

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 ACTC1 DES DICER1 KIT MME PGR

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

# Name GO ID Score Top Affiliating Genes
1 muscle filament sliding GO:0030049 8.62 ACTC1 DES

Sources for Cystic Nephroma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
27 GO
28 GTR
29 HGMD
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 MedGen
41 MeSH
42 MESH via Orphanet
43 MGI
45 NCI
46 NCIt
47 NDF-RT
50 NINDS
51 Novoseek
53 OMIM
54 OMIM via Orphanet
58 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
Content
Loading form....