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 rhabdomyosarcoma and embryonal 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 Primary Focal Segmental Glomerulosclerosis FSGS. The drugs Ifosfamide and Dasatinib have been mentioned in the context of this disorder. Affiliated tissues include kidney, breast and liver, and related phenotypes are cardiovascular system and cellular

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 155)
# Related Disease Score Top Affiliating Genes
1 rhabdomyosarcoma 30.0 ACTC1 DES DICER1
2 embryonal rhabdomyosarcoma 30.0 DES DICER1
3 renal cell carcinoma, nonpapillary 30.0 KIT MME WT1
4 malignant mesenchymoma 29.4 DES KIT WT1
5 angiomyolipoma 29.3 ACTC1 DES KIT PGR
6 malignant cystic nephroma 12.5
7 pleuropulmonary blastoma 11.7
8 dicer1-related pleuropulmonary blastoma cancer predisposition syndrome 11.6
9 mixed epithelial stromal tumour 11.4
10 childhood multilocular cystic kidney neoplasm 11.2
11 embryoma 10.4
12 wilms tumor 1 10.3
13 multiloculated renal cyst 10.3
14 reproductive organ benign neoplasm 10.3 DICER1 PGR
15 cystadenoma 10.2 MME PGR
16 cervical carcinosarcoma 10.2 MME PGR
17 endometrial stromal nodule 10.2 MME PGR
18 wilms tumor 5 10.2
19 wilms tumor 6 10.2
20 spermatocele 10.2 MME PGR
21 breast benign neoplasm 10.2 MME PGR
22 thoracic benign neoplasm 10.2 MME PGR
23 adenofibroma 10.2 ACTC1 MME
24 malignant leydig cell tumor 10.2 KIT MME
25 endometrial stromal tumor 10.2 MME PGR
26 rete testis adenocarcinoma 10.2 KIT MME
27 pancreatic mucinous cystadenoma 10.2 MME PGR
28 calcifying aponeurotic fibroma 10.2 ACTC1 PGR
29 bizarre leiomyoma 10.2 ACTC1 PGR
30 cribriform carcinoma 10.2 KIT PGR
31 adenoid basal cell carcinoma 10.2 KIT PGR
32 rete testis neoplasm 10.2 KIT MME
33 breast adenoid cystic carcinoma 10.2 KIT PGR
34 glycogen-rich clear cell breast carcinoma 10.2 ACTC1 PGR
35 intravenous leiomyomatosis 10.1 DES MME
36 breast fibroadenoma 10.1 MME PGR
37 pulmonary blastoma 10.1 DES DICER1
38 dermoid cyst 10.1 KIT PGR
39 lymphangiomatosis 10.1 DES PGR
40 renal oncocytoma 10.1 KIT MME
41 oncocytoma 10.1 KIT MME
42 sarcoma 10.1
43 chickenpox 10.1 ACTC1 PGR
44 epulis 10.1 DES PGR
45 adenomyosis 10.1 MME PGR
46 fibroblastic rheumatism 10.1 ACTC1 DES
47 angiolipoma 10.1 ACTC1 DES
48 cutaneous leiomyosarcoma 10.1 ACTC1 DES
49 botryoid rhabdomyosarcoma 10.1 ACTC1 DES
50 gallbladder sarcoma 10.1 DES KIT

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:

47
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 9.85 ACTC1 DES DICER1 KIT PGR WT1
2 cellular MP:0005384 9.8 ACTC1 DES DICER1 KIT PGR WT1
3 homeostasis/metabolism MP:0005376 9.8 ACTC1 DES DICER1 KIT MME PGR
4 muscle MP:0005369 9.63 ACTC1 DES DICER1 KIT PGR WT1
5 neoplasm MP:0002006 9.26 DICER1 KIT PGR WT1
6 normal MP:0002873 9.02 ACTC1 DICER1 KIT PGR 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
Dasatinib Approved, Investigational Phase 1, Phase 2 302962-49-8 3062316
3
Mechlorethamine Approved, Investigational Phase 1, Phase 2 51-75-2 4033
4
Etoposide Approved Phase 1, Phase 2 33419-42-0 36462
5
Carboplatin Approved Phase 1, Phase 2 41575-94-4 10339178 38904 498142
6
Isophosphamide mustard Phase 1, Phase 2 0
7 Liver Extracts Phase 1, Phase 2
8 Protein Kinase Inhibitors Phase 1, Phase 2
9 Alkylating Agents Phase 1, Phase 2
10 Antineoplastic Agents, Alkylating Phase 1, Phase 2
11 Etoposide phosphate Phase 1, Phase 2
12 Antineoplastic Agents, Phytogenic Phase 1, Phase 2
13 Topoisomerase Inhibitors Phase 1, Phase 2
14
Dactinomycin Approved, Investigational 50-76-0 457193 2019
15
Vincristine Approved, Investigational 2068-78-2, 57-22-7 5978
16
Doxorubicin Approved, Investigational 23214-92-8 31703
17
Cyclophosphamide Approved, Investigational 50-18-0, 6055-19-2 2907
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, Breast, Liver, Heart, Ovary, Testis, Myeloid

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
Pediatric Cystic Nephroma Is Morphologically, Immunohistochemically, and Genetically Distinct From Adult Cystic Nephroma. ( 28177962 )
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
Ultrasound Imaging of Cystic Nephroma. ( 28752022 )
2017
5
A Case Report of Largest Documented Multilocular Cystic Nephroma Removed by Thoracoabdominal Approach. ( 28892971 )
2017
6
A rare presentation of cystic nephroma in a young adult. ( 29264218 )
2017
7
Tumor progression in DICER1-mutated cystic nephroma-witnessing the genesis of anaplastic sarcoma of the kidney. ( 27036314 )
2016
8
Cystic Nephroma in Adults: A Clinicopathologic Study of 46 Cases. ( 27831959 )
2016
9
Multilocular Cystic Renal Cell Carcinoma or Cystic Nephroma? ( 28074169 )
2016
10
Imagistic and histopathological description of a cystic nephroma during early second trimester of gestation. Case report. ( 28845501 )
2016
11
Cystic nephroma of the kidney. ( 25444959 )
2015
12
Cystic nephroma/mixed epithelial stromal tumor: a benign neoplasm with potential for recurrence. ( 25525149 )
2015
13
A rare case of bilateral cystic nephroma associated with embryonal rhabdomyosarcoma of the penile urethra. ( 25829672 )
2015
14
An Unusual Presentation of Cystic Nephroma in an Adult Man. ( 26266015 )
2015
15
Multilocular Cystic Nephroma: A Systematic Literature Review of the Radiologic and Clinical Findings. ( 26587924 )
2015
16
DICER1 mutations in childhood cystic nephroma and its relationship to DICER1-renal sarcoma. ( 24481001 )
2014
17
Multilocular cystic nephroma: A clinical case. ( 24892395 )
2014
18
Quiz page JULY 2014: bilateral abdominal masses in an infant. Diagnosis: Bilateral cystic nephroma. ( 24954459 )
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
Laparoscopic partial nephrectomy for the treatment of large cystic nephroma in children. ( 25265482 )
2014
22
Case 211: pleuropulmonary blastoma in association with cystic nephroma-DICER1 syndrome. ( 25340273 )
2014
23
Adult multilocular cystic nephroma: Report of six cases with clinical, radio-pathologic correlation and review of literature. ( 23662002 )
2013
24
Renal metastasis of an ovarian granulosa cell tumour inducing growth of a cystic nephroma. ( 23843415 )
2013
25
Case report: Multimodality imaging of a cystic nephroma. ( 23861515 )
2013
26
Coexistence of cystic nephroma and neuroblastoma: a rare case of a childhood collision tumor. ( 22395847 )
2012
27
Can multilocular cystic nephroma be a harbinger of multilocular cystic renal cell carcinoma? ( 23032854 )
2012
28
Asymptomatic infantile cystic nephroma: a diagnostic dilemma. ( 24293912 )
2012
29
Bilateral cystic nephroma with pleuropulmonary blastoma. ( 22688934 )
2011
30
What is a cystic nephroma? ( 20035151 )
2010
31
Cystic nephroma: a fine needle aspiration diagnostic pitfall. ( 20391987 )
2010
32
Bilateral progressive cystic nephroma in a 9-month-old male infant requiring renal replacement therapy. ( 20414683 )
2010
33
Cystic nephroma: a diagnostic dilemma. ( 20427881 )
2010
34
Germline DICER1 mutations and familial cystic nephroma. ( 21036787 )
2010
35
Cystic nephroma masquerading as hydatid cyst of the kidney. ( 21045463 )
2010
36
Adult cystic nephroma: a case report and a review of the literature. ( 18555707 )
2009
37
Multilocular cystic nephroma and multicystic clear cell carcinoma: two faces of the Roman god Janus? ( 18611925 )
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
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
40
Cystic nephroma, cystic partially differentiated nephroblastoma and cystic Wilms' tumor in children: a spectrum with therapeutic dilemmas. ( 19172100 )
2009
41
Cystic nephroma in childhood. ( 19661676 )
2009
42
Cystic nephroma and mixed epithelial and stromal tumour of the kidney: opposite ends of the spectrum of the same entity? ( 18006141 )
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
Adult cystic nephroma and mixed epithelial and stromal tumor of the kidney: clinical, radiographic, and pathologic characteristics. ( 18313107 )
2008
45
Cytology of cystic nephroma: a case report. ( 18323282 )
2008
46
Failure of percutaneous endoscopic resection of a renal cystic nephroma on longer-term follow-up. ( 18613780 )
2008
47
Best cases from the AFIP: Multilocular cystic renal tumor: cystic nephroma. ( 18635639 )
2008
48
Cystic nephroma: a case report and review of the literature. ( 18947428 )
2008
49
Cystic nephroma and localized renal cystic disease in children: diagnostic clues and management. ( 18970929 )
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 positive regulation of gene expression GO:0010628 9.13 ACTC1 KIT WT1
2 muscle filament sliding GO:0030049 8.62 ACTC1 DES

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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