KEGG   DISEASE: 滑膜肉腫
エントリ  
H00050                                                             

名称    
滑膜肉腫
  上位グループ
固形腫瘍 [DS:H02421]
軟部肉腫 [DS:H02427]
概要    
Synovial sarcomas account for 7% to 10% of all human soft-tissue sarcomas. The tumors arise at any age, but affect mainly young adults and more commonly males. Clinically, they appear as deep-seated slowly growing masses. In more than half of the cases, metastases develop, primarily to the lungs but also to the lymph nodes and bone marrow. A specific translocation, t(X; 18)(p11.2; q11.2), is found in more than 90% of reported synovial sarcoma, including biphasic, monophasic, and poorly differentiated tumors. The breakpoints of the t(X; 18) have been cloned and shown to involve the fusion of the SYT gene at 18q11 to either of two highly homologous genes at Xp11 called SSX1 and SSX2. The SYT-SSX1 fusion is associated with biphasic morphology and a worse prognosis, whereas the SYT-SSX2 fusion tends to show monophasic morphology and better outcome.
カテゴリ  
がん
階層分類  
ヒト疾患 [BR:jp08402]
 がん
  軟部組織および骨のがん
   H00050  滑膜肉腫
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
    悪性間葉系腫瘍
     2B5A  滑膜肉腫, 原発巣
      H00050  滑膜肉腫
関連パスウェイ
hsa05202  Transcriptional misregulation in cancer
hsa05200  Pathways in cancer
ネットワーク
  エレメント
N00142  SYT-SSX fusion to transcriptional repression
病因遺伝子 
SYT-SSX1 (translocation) [HSA:6756] [KO:K15624]
SYT-SSX2 (translocation) [HSA:6757] [KO:K15625]
IGF-IR (overexpression) [HSA:3480] [KO:K05087]
コメント  
ICD-O: 9040/3, Tumor type: Synovial sarcoma
リンク   
ICD-11: 2B5A
ICD-10: C49
MeSH: D013584
文献    
  著者
dos Santos NR, de Bruijn DR, van Kessel AG
  タイトル
Molecular mechanisms underlying human synovial sarcoma development.
  雑誌
Genes Chromosomes Cancer 30:1-14 (2001)
DOI:10.1002/1098-2264(2000)9999:9999<::AID-GCC1056>3.0.CO;2-G
文献    
PMID:11378650 (gene, tumor type)
  著者
Letson GD, Muro-Cacho CA.
  タイトル
Genetic and molecular abnormalities in tumors of the bone and soft tissues.
  雑誌
Cancer Control 8:239-51 (2001)
DOI:10.1177/107327480100800304
文献    
  著者
Helman LJ, Meltzer P.
  タイトル
Mechanisms of sarcoma development.
  雑誌
Nat Rev Cancer 3:685-94 (2003)
DOI:10.1038/nrc1168
文献    
  著者
Fisher C.
  タイトル
Current aspects of the pathology of soft tissue sarcomas.
  雑誌
Semin Radiat Oncol 9:315-27 (1999)
DOI:10.1053/SRAO00900315
文献    
PMID:10824918 (marker)
  著者
Inagaki H, Nagasaka T, Otsuka T, Sugiura E, Nakashima N, Eimoto T.
  タイトル
Association of SYT-SSX fusion types with proliferative activity and prognosis in synovial sarcoma.
  雑誌
Mod Pathol 13:482-8 (2000)
DOI:10.1038/modpathol.3880083
文献    
PMID:11826189 (marker)
  著者
Bijwaard KE, Fetsch JF, Przygodzki R, Taubenberger JK, Lichy JH.
  タイトル
Detection of SYT-SSX fusion transcripts in archival synovial sarcomas by real-time reverse transcriptase-polymerase chain reaction.
  雑誌
J Mol Diagn 4:59-64 (2002)
DOI:10.1016/S1525-1578(10)60681-2
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