KEGG   DISEASE: 骨巨細胞腫
エントリ  
H01470                                                             
名称    
骨巨細胞腫
概要    
Giant-cell tumor of bone (GCTB) is a rare osteolytic tumor of the bone. Although classified as a benign tumor, GCTB is characterized by local aggressiveness and risk of local recurrence. Its name is derived from the numerous multinucleated giant cells found within the tumor, which are principally responsible for the extensive bone resorption that is characteristic of GCTB. However, the neoplastic components of GCTB are the spindle-like stromal cells, which produce RANKL that recruits monocytic osteoclast precursors from blood to the tumor, and stimulates differentiation into multinucleated giant cells. Recently, denosumab (RANKL inhibitor) has become a new treatment option for locally advanced GCTB.
カテゴリ  
がん
階層分類  
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
    悪性間葉系腫瘍
     2B51  骨肉腫, 原発部位
      H01470  骨巨細胞腫
パスウェイ 
hsa04380  Osteoclast differentiation
病因遺伝子 
RANKL (overexpression) [HSA:8600] [KO:K05473]
H3F3A (mutation) [HSA:3020] [KO:K11253]
TP53 (mutation) [HSA:7157] [KO:K04451]
HRAS (mutation) [HSA:3265] [KO:K02833]
治療薬   
デノスマブ [DR:D03684]
リンク   
ICD-11: 2B51
MeSH: D018212
文献    
  著者
van der Heijden L, Dijkstra PD, van de Sande MA, Kroep JR, Nout RA, van Rijswijk CS, Bovee JV, Hogendoorn PC, Gelderblom H
  タイトル
The clinical approach toward giant cell tumor of bone.
  雑誌
Oncologist 19:550-61 (2014)
DOI:10.1634/theoncologist.2013-0432
文献    
  著者
Dufresne A, Derbel O, Cassier P, Vaz G, Decouvelaere AV, Blay JY
  タイトル
Giant-cell tumor of bone, anti-RANKL therapy.
  雑誌
Bonekey Rep 1:149 (2012)
DOI:10.1038/bonekey.2012.149
文献    
  著者
Cowan RW, Singh G
  タイトル
Giant cell tumor of bone: a basic science perspective.
  雑誌
Bone 52:238-46 (2013)
DOI:10.1016/j.bone.2012.10.002
文献    
  著者
Skubitz KM
  タイトル
Giant cell tumor of bone: current treatment options.
  雑誌
Curr Treat Options Oncol 15:507-18 (2014)
DOI:10.1007/s11864-014-0289-1
LinkDB    

» English version

DBGET integrated database retrieval system