KEGG   DISEASE: 腎芽腫
エントリ  
H02301                                                             
名称    
腎芽腫;
ウィルムス腫瘍
概要    
Nephroblastoma, also called Wilms tumor (WT), is the most common renal tumor of childhood. It can present as a single nodule, as multifocal unilateral lesions or as bilateral tumours. Typically, nephroblastoma comprises three histological components namely blastemal, epithelial and stromal. WT1, a zinc-finger transcription factor, was identified as the first nephroblastoma gene. Several other genes including CTNNB1, WTX, and TP53 have also been implicated in various stages of tumorigenesis of nephroblastoma.
カテゴリ  
がん
階層分類  
ヒト疾患 [BR:jp08402]
 がん
  泌尿器系のがん
   H02301  腎芽腫
ICD-11 による疾患分類 [BR:jp08403]
 02 腫瘍
  悪性腫瘍, ただしリンパ, 造血, 中枢神経系または関連組織の原発腫瘍を除く
   悪性腫瘍, 原発性と確定または推定されるもの, 明示された部位のもの, ただしリンパ, 造血, 中枢神経系, 関連組織を除く
    腎尿路の悪性腫瘍
     2C90  腎盂を除く腎臓の悪性腫瘍
      H02301  腎芽腫
関連パスウェイ
hsa05200  Pathways in cancer
病因遺伝子 
(WT1) WT1 [HSA:7490] [KO:K09234]
(WT5) POU6F2 [HSA:11281] [KO:K09368]
(WT6) REST [HSA:5978] [KO:K09222]
CTNNB1 [HSA:1499] [KO:K02105]
TP53 [HSA:7157] [KO:K04451]
BRCA2 [HSA:675] [KO:K08775]
GPC3 [HSA:2719] [KO:K08109]
治療薬   
イホスファミド [DR:D00343]
アクチノマイシンD [DR:D00214]
ドキソルビシン塩酸塩 [DR:D01275]
ビンクリスチン硫酸塩 [DR:D02197]
エトポシド [DR:D00125]
カルボプラチン [DR:D01363]
リンク   
ICD-11: 2C90.Y
ICD-10: C64
MeSH: D009396
OMIM: 194070 601583 616806
文献    
  著者
Popov SD, Sebire NJ, Vujanic GM
  タイトル
Wilms' Tumour - Histology and Differential Diagnosis
  雑誌
Wilms Tumor (2016)
DOI:10.15586/codon.wt.2016
文献    
  著者
Diniz G
  タイトル
Histopathological and Molecular Characteristics of Wilms Tumor
  雑誌
Wilms Tumor (2016)
DOI:10.15586/codon.wt.2016
文献    
  著者
Dome JS, Huff V
  タイトル
Wilms Tumor Predisposition
  雑誌
GeneReviews (1993)
文献    
  著者
Deng C, Dai R, Li X, Liu F
  タイトル
Genetic variation frequencies in Wilms' tumor: A meta-analysis and systematic review.
  雑誌
Cancer Sci 107:690-9 (2016)
DOI:10.1111/cas.12910
文献    
  著者
Al-Hussain T, Ali A, Akhtar M
  タイトル
Wilms tumor: an update.
  雑誌
Adv Anat Pathol 21:166-73 (2014)
DOI:10.1097/PAP.0000000000000017
文献    
  著者
Royer-Pokora B
  タイトル
Genetics of pediatric renal tumors.
  雑誌
Pediatr Nephrol 28:13-23 (2013)
DOI:10.1007/s00467-012-2146-4
文献    
PMID:3031816 (WT1)
  著者
Weissman BE, Saxon PJ, Pasquale SR, Jones GR, Geiser AG, Stanbridge EJ
  タイトル
Introduction of a normal human chromosome 11 into a Wilms' tumor cell line controls its tumorigenic expression.
  雑誌
Science 236:175-80 (1987)
DOI:10.1126/science.3031816
文献    
PMID:17164647 (POU6F2)
  著者
Di Renzo F, Doneda L, Menegola E, Sardella M, De Vecchi G, Collini P, Spreafico F, Fossati-Bellani F, Giavini E, Radice P, Perotti D
  タイトル
The murine Pou6f2 gene is temporally and spatially regulated during kidney embryogenesis and its human homolog is overexpressed in a subset of Wilms tumors.
  雑誌
J Pediatr Hematol Oncol 28:791-7 (2006)
DOI:10.1097/MPH.0b013e31802d3e65
文献    
PMID:26551668 (REST)
  著者
Mahamdallie SS, Hanks S, Karlin KL, Zachariou A, Perdeaux ER, Ruark E, Shaw CA, Renwick A, Ramsay E, Yost S, Elliott A, Birch J, Capra M, Gray J, Hale J, Kingston J, Levitt G, McLean T, Sheridan E, Renwick A, Seal S, Stiller C, Sebire N, Westbrook TF, Rahman N
  タイトル
Mutations in the transcriptional repressor REST predispose to Wilms tumor.
  雑誌
Nat Genet 47:1471-4 (2015)
DOI:10.1038/ng.3440
文献    
PMID:15689453 (BRCA2)
  著者
Reid S, Renwick A, Seal S, Baskcomb L, Barfoot R, Jayatilake H, Pritchard-Jones K, Stratton MR, Ridolfi-Luthy A, Rahman N
  タイトル
Biallelic BRCA2 mutations are associated with multiple malignancies in childhood including familial Wilms tumour.
  雑誌
J Med Genet 42:147-51 (2005)
DOI:10.1136/jmg.2004.022673
文献    
PMID:12085187 (GPC3)
  著者
White GR, Kelsey AM, Varley JM, Birch JM
  タイトル
Somatic glypican 3 (GPC3) mutations in Wilms' tumour.
  雑誌
Br J Cancer 86:1920-2 (2002)
DOI:10.1038/sj.bjc.6600417
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