KEGG   DISEASE: Osteosarcoma
Entry
H00036                      Disease                                
Name
Osteosarcoma
  Supergrp
Solid tumor [DS:H02421]
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 primary neoplasms 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
Cancer-associated carbohydrates [br08441.html]
 H00036
Related
pathway
hsa05200  Pathways in cancer
hsa04110  Cell cycle
hsa04218  Cellular senescence
Gene
MDM2 (amplification) [HSA:4193] [KO:K06643]
TSPAN31 (amplification) [HSA:6302] [KO:K17356]
MYC (amplification) [HSA:4609] [KO:K04377]
TP53 [HSA:7157] [KO:K04451]
RB1 [HSA:5925] [KO:K06618]
CDKN2A [HSA:1029] [KO:K06621]
CDKN2B [HSA:1030] [KO:K04685]
CHEK2 [HSA:11200] [KO:K06641]
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]
Calcium levofolinate hydrate [DR:D11555]
Sodium levofolinate [DR:D11394]
Other DBs
ICD-11: 2B51
ICD-10: C41
MeSH: D012516
OMIM: 259500
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)
DOI:10.1038/labinvest.3780431
Reference
  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:8893761 (TSPAN31)
  Authors
Noble-Topham SE, Burrow SR, Eppert K, Kandel RA, Meltzer PS, Bell RS, Andrulis IL
  Title
SAS is amplified predominantly in surface osteosarcoma.
  Journal
J Orthop Res 14:700-5 (1996)
DOI:10.1002/jor.1100140504
Reference
PMID:11746983 (CHEK2)
  Authors
Miller CW, Ikezoe T, Krug U, Hofmann WK, Tavor S, Vegesna V, Tsukasaki K, Takeuchi S, Koeffler HP
  Title
Mutations of the CHK2 gene are found in some osteosarcomas, but are rare in breast, lung, and ovarian tumors.
  Journal
Genes Chromosomes Cancer 33:17-21 (2002)
DOI:10.1002/gcc.1207
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