KEGG   DISEASE: Synovial sarcomaHelp
Entry
H00050                      Disease                                

Name
Synovial sarcoma
Description
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.
Category
Cancer
Brite
Human diseases [BR:br08402]
 Cancers
  Cancers of soft tissues and bone
   H00050  Synovial sarcoma
Human diseases in ICD-11 classification [BR:br08403]
 02 Neoplasms
  Malignant neoplasms, except of lymphoid, haematopoietic, central nervous system or related tissues
   Malignant neoplasms, stated or presumed to be primary, of specified sites, except of lymphoid, haematopoietic, central nervous system or related tissues
    Malignant mesenchymal neoplasms
     2B5A  Synovial sarcoma, primary site
      H00050  Synovial sarcoma
BRITE hierarchy
Pathway
hsa05202  Transcriptional misregulation in cancer
Network
N00142  SYT-SSX fusion to transcriptional repression
Gene
SYT-SSX1 (translocation) [HSA:6756] [KO:K15624]
SYT-SSX2 (translocation) [HSA:6757] [KO:K15625]
IGF-IR (overexpression) [HSA:3480] [KO:K05087]
Comment
ICD-O: 9040/3, Tumor type: Synovial sarcoma
Other DBs
ICD-11: 2B5A
ICD-10: C49
MeSH: D013584
Reference
  Authors
dos Santos NR, de Bruijn DR, van Kessel AG
  Title
Molecular mechanisms underlying human synovial sarcoma development.
  Journal
Genes Chromosomes Cancer 30:1-14 (2001)
DOI:10.1002/1098-2264(2000)9999:9999<::AID-GCC1056>3.0.CO;2-G
Reference
PMID:11378650 (gene, tumor type)
  Authors
Letson GD, Muro-Cacho CA.
  Title
Genetic and molecular abnormalities in tumors of the bone and soft tissues.
  Journal
Cancer Control 8:239-51 (2001)
DOI:10.1177/107327480100800304
Reference
  Authors
Helman LJ, Meltzer P.
  Title
Mechanisms of sarcoma development.
  Journal
Nat Rev Cancer 3:685-94 (2003)
DOI:10.1038/nrc1168
Reference
  Authors
Fisher C.
  Title
Current aspects of the pathology of soft tissue sarcomas.
  Journal
Semin Radiat Oncol 9:315-27 (1999)
DOI:10.1053/SRAO00900315
Reference
PMID:10824918 (marker)
  Authors
Inagaki H, Nagasaka T, Otsuka T, Sugiura E, Nakashima N, Eimoto T.
  Title
Association of SYT-SSX fusion types with proliferative activity and prognosis in synovial sarcoma.
  Journal
Mod Pathol 13:482-8 (2000)
DOI:10.1038/modpathol.3880083
Reference
PMID:11826189 (marker)
  Authors
Bijwaard KE, Fetsch JF, Przygodzki R, Taubenberger JK, Lichy JH.
  Title
Detection of SYT-SSX fusion transcripts in archival synovial sarcomas by real-time reverse transcriptase-polymerase chain reaction.
  Journal
J Mol Diagn 4:59-64 (2002)
DOI:10.1016/S1525-1578(10)60681-2

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