KEGG   DISEASE: OsteosarcomaHelp
Entry
H00036                      Disease                                

Name
Osteosarcoma
Description
Osteosarcoma is the most common type of primary bone cancer. Approximately 900 new cases of osteosarcoma are diagnosed each year in the United States. The presentation of this disease is bimodal, with peaks in adolescence and after the age of 50 years. The tumors typically arise in the metaphyseal regions of long bones, with the distal femur, proximal tibia, and proximal humerus representing the three most common sites. Genomic amplification, especially of both the p53-binding MDM2 gene and the flanking SAS gene, plays an important role in the biology of these tumors. Alterations of Rb1 and c-myc are also common, and mutations have been reported in p53, p16INK4A, and CDKN2B.
Category
Cancer
Brite
Human diseases [BR:br08402]
 Cancers
  Cancers of soft tissues and bone
   H00036  Osteosarcoma
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
     2B51  Osteosarcoma, primary site
      H00036  Osteosarcoma
BRITE hierarchy
Gene
MDM2 (amplification) [HSA:4193] [KO:K06643]
SAS (amplification) [HSA:6302] [KO:K17356]
p53 (losses, rearrangements, point mutation) [HSA:7157] [KO:K04451]
RB1 (losses, rearrangements, point mutation) [HSA:5925] [KO:K06618]
p16/INK4A (mutation) [HSA:1029] [KO:K06621]
CDKN2B (mutation) [HSA:1030] [KO:K04685]
c-MYC (amplification) [HSA:4609] [KO:K04377]
Carcinogen
Radium-224 and its decay products [CPD:C16455]
Radium-226 and its decay products [CPD:C16456]
Radium-228 and its decay products [CPD:C16457]
Drug
Leucovorin calcium [DR:D01211]
Levoleucovorin calcium [DR:D04715]
Sodium levofolinate [DR:D11394]
Comment
ICD-O: 9180/3, Tumor type: Osteosarcoma
Other DBs
ICD-11: 2B51
ICD-10: C41
MeSH: D012516
Reference
  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
Ragland BD, Bell WC, Lopez RR, Siegal GP
  Title
Cytogenetics and molecular biology of osteosarcoma.
  Journal
Lab Invest 82:365-73 (2002)
Reference
PMID:17263592 (description, gene, tumor type)
  Authors
Kansara M, Thomas DM.
  Title
Molecular pathogenesis of osteosarcoma.
  Journal
DNA Cell Biol 26:1-18 (2007)
DOI:10.1089/dna.2006.0505
Reference
  Authors
Papachristou DJ, Papavassiliou AG.
  Title
Osteosarcoma and chondrosarcoma: new signaling pathways as targets for novel therapeutic interventions.
  Journal
Int J Biochem Cell Biol 39:857-62 (2007)
DOI:10.1016/j.biocel.2006.12.003
Reference
PMID:10564927 (carcinogen)
  Authors
Gossner W.
  Title
Pathology of radium-induced bone tumors: new aspects of histopathology and histogenesis.
  Journal
Radiat Res 152:S12-5 (1999)
DOI:10.2307/3580104
Reference
PMID:10564925 (carcinogen)
  Authors
Nekolla EA, Kellerer AM, Kuse-Isingschulte M, Eder E, Spiess H.
  Title
Malignancies in patients treated with high doses of radium-224.
  Journal
Radiat Res 152:S3-7 (1999)
DOI:10.2307/3580102
Reference
PMID:12829666 (marker)
  Authors
Dhaini HR, Thomas DG, Giordano TJ, Johnson TD, Biermann JS, Leu K, Hollenberg PF, Baker LH.
  Title
Cytochrome P450 CYP3A4/5 expression as a biomarker of outcome in osteosarcoma.
  Journal
J Clin Oncol 21:2481-5 (2003)
DOI:10.1200/JCO.2003.06.015
Reference
PMID:17786474 (marker)
  Authors
Salas S, Bartoli C, Deville JL, Gaudart J, Fina F, Calisti A, Bollini G, Curvale G, Gentet JC, Duffaud F, Figarella-Branger D, Bouvier C.
  Title
Ezrin and alpha-smooth muscle actin are immunohistochemical prognostic markers in conventional osteosarcomas.
  Journal
Virchows Arch 451:999-1007 (2007)
DOI:10.1007/s00428-007-0474-8
Reference
PMID:17327929 (marker)
  Authors
Osaka E, Suzuki T, Osaka S, Yoshida Y, Sugita H, Asami S, Tabata K, Hemmi A, Sugitani M, Nemoto N, Ryu J.
  Title
Survivin as a prognostic factor for osteosarcoma patients.
  Journal
Acta Histochem Cytochem 39:95-100 (2006)
DOI:10.1267/ahc.06005

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