KEGG   DISEASE: Myxoid liposarcomaHelp
Entry
H00049                      Disease                                

Name
Myxoid liposarcoma
Description
Liposarcoma(LS) represents the most common soft-tissue sarcoma of adults and occurs most often in the thigh and retroperitoneum. LSs are subclassified into well-differentiated, myxoid, round cell, and pleomorphic types. The myxoid type, the most common subtype, accounts for approximately 40% to 54% of all liposarcomas. The karyotypic hallmark of myxoid LS is the t(12;16)(q13;p11), present cytogenetically in >90% of the cases. The translocation leads to the fusion of the DDIT3(CHOP) and FUS(TLS) genes at 12q13 and 16p11, respectively, and the generation of a FUS-DDIT3 hybrid protein. In 4 cases of myxoid LS, a variant chromosomal translocation has been described, t(12;22), in which DDIT3 fuses instead with EWSR1(EWS), a gene highly related to TLS.
Category
Cancer
Brite
Human diseases [BR:br08402]
 Cancers
  Cancers of soft tissues and bone
   H00049  Myxoid liposarcoma
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
     2B59  Liposarcoma, primary site
      H00049  Myxoid liposarcoma
BRITE hierarchy
Pathway
hsa05202  Transcriptional misregulation in cancer
Network
N00139  FUS-DDIT3 fusion to CEBPB-mediated transcription
N00140  FUS-DDIT3 fusion to NFKB-mediated transcription
Gene
FUS-DDIT3 (translocation) [HSA:1649] [KO:K04452]
EWSR1-DDIT3 (translocation) [HSA:1649] [KO:K04452]
Comment
ICD-O: 8852/3, Tumor type: Myxoid liposarcoma
Other DBs
ICD-11: 2B59
ICD-10: C49
MeSH: D018208
Reference
  Authors
Antonescu CR, Elahi A, Humphrey M, Lui MY, Healey JH, Brennan MF, Woodruff JM, Jhanwar SC, Ladanyi M
  Title
Specificity of TLS-CHOP rearrangement for classic myxoid/round cell liposarcoma: absence in predominantly myxoid well-differentiated liposarcomas.
  Journal
J Mol Diagn 2:132-8 (2000)
DOI:10.1016/S1525-1578(10)60628-9
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
PMID:12890805 (marker)
  Authors
Tateishi U, Hasegawa T, Beppu Y, Kawai A, Moriyama N.
  Title
Prognostic significance of grading (MIB-1 system) in patients with myxoid liposarcoma.
  Journal
J Clin Pathol 56:579-82 (2003)
DOI:10.1136/jcp.56.8.579
Reference
PMID:7817937 (marker)
  Authors
Gibas Z, Miettinen M, Limon J, Nedoszytko B, Mrozek K, Roszkiewicz A, Rys J, Niezabitowski A, Debiec-Rychter M.
  Title
Cytogenetic and immunohistochemical profile of myxoid liposarcoma.
  Journal
Am J Clin Pathol 103:20-6 (1995)
DOI:10.1093/ajcp/103.1.20
Reference
PMID:7805034 (marker)
  Authors
Knight JC, Renwick PJ, Cin PD, Van den Berghe H, Fletcher CD.
  Title
Translocation t(12;16)(q13;p11) in myxoid liposarcoma and round cell liposarcoma: molecular and cytogenetic analysis.
  Journal
Cancer Res 55:24-7 (1995)

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