MCID: CYS007
MIFTS: 35

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 72

Classifications:



External Ids:

Disease Ontology 12 DOID:2673
UMLS 72 C1266138

Summaries for Cystic Nephroma

MalaCards based summary : Cystic Nephroma, also known as benign cystic nephroma, is related to kidney sarcoma 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 Primary Focal Segmental Glomerulosclerosis FSGS and Dendritic Cells Developmental Lineage Pathway. The drugs Ifosfamide and Etoposide 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 : 75 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 132)
# Related Disease Score Top Affiliating Genes
1 kidney sarcoma 30.3 WT1 DICER1
2 renal cell carcinoma, nonpapillary 29.9 WT1 MME KIT
3 congenital mesoblastic nephroma 29.6 WT1 KIT
4 malignant mesenchymoma 29.5 WT1 KIT
5 angiomyolipoma 29.5 PGR KIT ACTC1
6 renal cell carcinoma, papillary, 1 29.1 WT1 MME KIT
7 malignant cystic nephroma 12.6
8 pleuropulmonary blastoma 11.9
9 dicer1-related disorders 11.7
10 childhood multilocular cystic kidney neoplasm 11.7
11 dicer1-related pleuropulmonary blastoma cancer predisposition syndrome 11.7
12 mixed epithelial stromal tumour 11.5
13 wilms tumor 1 10.7
14 wilms tumor 5 10.6
15 embryoma 10.5
16 multilocular cystic renal neoplasm of low malignant potential 10.4
17 reproductive organ benign neoplasm 10.4 PGR DICER1
18 cervical carcinosarcoma 10.3 PGR MME
19 acute cystitis 10.3
20 cystic kidney disease 10.3
21 endometrial stromal nodule 10.3 PGR MME
22 spermatocele 10.3 PGR MME
23 endometrial stromal tumor 10.3 PGR MME
24 thoracic benign neoplasm 10.3 PGR MME
25 adenofibroma 10.3 MME ACTC1
26 breast benign neoplasm 10.3 PGR MME
27 pancreatic mucinous cystadenoma 10.3 PGR MME
28 cystadenoma 10.3 PGR MME
29 calcifying aponeurotic fibroma 10.3 PGR ACTC1
30 bizarre leiomyoma 10.3 PGR ACTC1
31 benign metastasizing leiomyoma 10.3 PGR ACTC1
32 juvenile type testicular granulosa cell tumor 10.2 WT1 DICER1
33 testicular granulosa cell tumor 10.2 WT1 DICER1
34 multiloculated renal cyst 10.2
35 glycogen-rich clear cell breast carcinoma 10.2 PGR ACTC1
36 male reproductive organ benign neoplasm 10.2 WT1 DICER1
37 chickenpox 10.2 PGR ACTC1
38 breast fibroadenoma 10.2 PGR MME
39 endosalpingiosis 10.2 WT1 PGR
40 female reproductive endometrioid cancer 10.1 WT1 PGR
41 congenital heart defects, hamartomas of tongue, and polysyndactyly 10.1
42 sarcoma 10.1
43 rhabdomyosarcoma 10.1
44 spindle cell sarcoma 10.1
45 sertoli cell tumor 10.1 WT1 PGR
46 spindle cell carcinoma 10.1 PGR ACTC1
47 adenomyosis 10.1 PGR MME
48 nephrogenic adenofibroma 10.1 WT1 MME
49 malignant leydig cell tumor 10.0 MME KIT
50 rete testis adenocarcinoma 10.0 MME 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:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 9.93 ACTC1 DICER1 KIT MEST PGR WT1
2 cellular MP:0005384 9.91 ACTC1 DICER1 KIT MEST PGR WT1
3 homeostasis/metabolism MP:0005376 9.85 ACTC1 DICER1 KIT MME PGR WT1
4 embryo MP:0005380 9.8 DICER1 KIT MEST PGR WT1
5 muscle MP:0005369 9.73 ACTC1 DICER1 KIT MEST PGR WT1
6 liver/biliary system MP:0005370 9.62 DICER1 KIT MME WT1
7 neoplasm MP:0002006 9.46 DICER1 KIT PGR WT1
8 no phenotypic analysis MP:0003012 9.26 DICER1 KIT PGR WT1
9 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
Etoposide Approved Phase 1, Phase 2 33419-42-0 36462
3
Dasatinib Approved, Investigational Phase 1, Phase 2 302962-49-8 3062316
4
Mechlorethamine Approved, Investigational Phase 1, Phase 2 51-75-2 4033
5
Carboplatin Approved Phase 1, Phase 2 41575-94-4 10339178 498142 38904
6
Isophosphamide mustard Phase 1, Phase 2 0
7 Antineoplastic Agents, Phytogenic Phase 1, Phase 2
8 Alkylating Agents Phase 1, Phase 2
9 Protein Kinase Inhibitors Phase 1, Phase 2
10 Topoisomerase Inhibitors Phase 1, Phase 2
11 Liver Extracts Phase 1, Phase 2
12 Etoposide phosphate Phase 1, Phase 2
13 Antineoplastic Agents, Alkylating Phase 1, Phase 2
14
Cyclophosphamide Approved, Investigational 50-18-0, 6055-19-2 2907
15
Vincristine Approved, Investigational 57-22-7, 2068-78-2 5978
16
Dactinomycin Approved, Investigational 50-76-0 457193 2019
17
Doxorubicin Approved, Investigational 23214-92-8 31703
18
Liposomal doxorubicin 31703

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Dasatinib With Ifosfamide, Carboplatin, Etoposide: A Pediatric Phase I/II Trial Active, not recruiting NCT00788125 Phase 1, Phase 2 carboplatin;dasatinib;etoposide phosphate;ifosfamide
2 Investigation of DICER1 in Cystic Nephroma and Cystic Partially Differentiated Nephroblastoma Completed NCT01353300
3 DICER1-Related Pleuropulmonary Blastoma Cancer Predisposition Syndrome: A Natural History Study Recruiting NCT01247597
4 International Pleuropulmonary Blastoma 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
6 Renal Tumors Classification, Biology, and Banking Study Suspended NCT00898365

Search NIH Clinical Center for Cystic Nephroma

Genetic Tests for Cystic Nephroma

Anatomical Context for Cystic Nephroma

MalaCards organs/tissues related to Cystic Nephroma:

41
Kidney, Liver, Lung, Testes, Lymph Node, Thyroid, Neutrophil

Publications for Cystic Nephroma

Articles related to Cystic Nephroma:

(show top 50) (show all 350)
# Title Authors PMID Year
1
Open Radical Nephrectomy for Suspected Renal Malignancy-Tips and Tricks. 38
31419431 2019
2
An update on the central nervous system manifestations of DICER1 syndrome. 38
30953130 2019
3
Neoplasm Risk Among Individuals With a Pathogenic Germline Variant in DICER1. 38
30715996 2019
4
[Cystic nephroma : a rare kidney neoplasm]. 38
30897310 2019
5
Contemporary update on imaging of cystic renal masses with histopathological correlation and emphasis on patient management. 38
30314810 2019
6
Identification of two 14q32 deletions involving DICER1 associated with the development of DICER1-related tumors. 38
29698806 2019
7
DICER1 Syndrome. 38
31409088 2019
8
Structural renal abnormalities in the DICER1 syndrome: a family-based cohort study. 38
30178239 2018
9
Characterization and management of various renal cystic lesions by sonographic features. 38
30017809 2018
10
Sorting the Alphabet Soup of Renal Pathology: A Review. 38
26928791 2018
11
Magnetic resonance imaging (MRI) of the renal sinus. 38
29632991 2018
12
Contrast-enhanced ultrasound of the kidney: a single-institution experience. 38
29218489 2018
13
Wilms tumor, pleuropulmonary blastoma, and DICER1: case report and literature review. 38
30097050 2018
14
Primary intracranial spindle cell sarcoma with rhabdomyosarcoma-like features share a highly distinct methylation profile and DICER1 mutations. 38
29881993 2018
15
Cystic nephroma: A case report in adult patients. 38
29799412 2018
16
Tubulocystic renal cell carcinoma: a review of literature focused on radiological findings for differential diagnosis. 38
29796844 2018
17
Multiple DICER1-related tumors in a child with a large interstitial 14q32 deletion. 38
29315962 2018
18
DICER1 and Associated Conditions: Identification of At-risk Individuals and Recommended Surveillance Strategies. 38
29343557 2018
19
A case of neuroblastoma in DICER1 syndrome: Chance finding or noncanonical causation? 38
28766837 2018
20
Multiloculated Cystic Renal Tumors of Childhood: Has the Final Word Been Spoken. 38
29386760 2018
21
Primary Renal Sarcomas With BCOR-CCNB3 Gene Fusion: A Report of 2 Cases Showing Histologic Overlap With Clear Cell Sarcoma of Kidney, Suggesting Further Link Between BCOR-related Sarcomas of the Kidney and Soft Tissues. 38
28817404 2017
22
The prevalence of DICER1 pathogenic variation in population databases. 38
28748527 2017
23
Pediatric imaging in DICER1 syndrome. 38
28474256 2017
24
Multimorbidity and Genetic Characteristics of DICER1 Syndrome Based on Systematic Review. 38
27906793 2017
25
Embryonal rhabdomyosarcoma in a patient with a heterozygous frameshift variant in the DICER1 gene and additional manifestations of the DICER1 syndrome. 38
27896549 2017
26
A Case Report of Largest Documented Multilocular Cystic Nephroma Removed by Thoracoabdominal Approach. 38
28892971 2017
27
PTEN, DICER1, FH, and Their Associated Tumor Susceptibility Syndromes: Clinical Features, Genetics, and Surveillance Recommendations in Childhood. 38
28620008 2017
28
A rare presentation of cystic nephroma in a young adult. 38
29264218 2017
29
Pediatric Cystic Nephroma Is Morphologically, Immunohistochemically, and Genetically Distinct From Adult Cystic Nephroma. 38
28177962 2017
30
DICER1 mutation and tumors associated with a familial tumor predisposition syndrome: practical considerations. 38
27830405 2017
31
Identification of somatic and germ-line DICER1 mutations in pleuropulmonary blastoma, cystic nephroma and rhabdomyosarcoma tumors within a DICER1 syndrome pedigree. 38
28222777 2017
32
Mixed Epithelial and Stromal Tumor of the Kidney: Mutation Analysis of the DICER 1 Gene in 29 Cases. 38
26509905 2017
33
Ultrasound Imaging of Cystic Nephroma. 38
28752022 2017
34
Retrospective evaluation of ultrasound-indeterminate renal multilocular cystic masses by using neutrophil-lymphocyte ratio and computed tomography. 38
27671994 2017
35
[Cystic nephroma. Report of two cases and bibliographic review.] 38
28042792 2016
36
Cystic Nephroma in Adults: A Clinicopathologic Study of 46 Cases. 38
27831959 2016
37
Imagistic and histopathological description of a cystic nephroma during early second trimester of gestation. Case report. 38
28845501 2016
38
Pediatric renal and genitourinary tract tumors and the contributions of Dr. Louis "Pepper" Dehner therewith. 38
27720562 2016
39
Multilocular cystic renal cell carcinoma in a 23 year old female. 38
28058005 2016
40
Tumor progression in DICER1-mutated cystic nephroma-witnessing the genesis of anaplastic sarcoma of the kidney. 38
27036314 2016
41
Evolution of Renal Cysts to Anaplastic Sarcoma of Kidney in a Child With DICER1 Syndrome. 38
26928971 2016
42
A Case of Bilateral Cystic Partially Differentiated Nephroblastoma vs Cystic Wilms' Tumor: Highlighting a Diagnostic Dilemma. 38
26850814 2016
43
Hodgkin lymphoma as a novel presentation of familial DICER1 syndrome. 38
26526666 2016
44
Uncommon renal tumors in children: A single center experience. 38
27046976 2016
45
DICER1 pleuropulmonary blastoma familial tumour predisposition syndrome: What the paediatric urologist needs to know. 38
26454454 2016
46
Ovarian tumors related to intronic mutations in DICER1: a report from the international ovarian and testicular stromal tumor registry. 38
26289771 2016
47
DICER1 Mutations and Differentiated Thyroid Carcinoma: Evidence of a Direct Association. 38
26555935 2016
48
Multilocular Cystic Renal Cell Carcinoma or Cystic Nephroma? 38
28074169 2016
49
DICER1 syndrome can mimic different genetic tumor predispositions. 38
26577641 2016
50
Multilocular Cystic Nephroma: A Systematic Literature Review of the Radiologic and Clinical Findings. 38
26587924 2015

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 11.13 WT1 MME
2 11.02 MME KIT
3 10.68 KIT ACTC1
4 10.35 MME KIT

GO Terms for Cystic Nephroma

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 8.8 WT1 KIT ACTC1

Sources for Cystic Nephroma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 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 TGDB
71 Tocris
72 UMLS
73 UMLS via Orphanet
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