PMID- 23036192
OWN - NLM
STAT- PubMed-not-MEDLINE
DCOM- 20121119
LR  - 20211021
IS  - 2045-3329 (Electronic)
IS  - 2045-3329 (Linking)
VI  - 2
IP  - 1
DP  - 2012 Oct 4
TI  - Sarcomas in hereditary retinoblastoma.
PG  - 15
LID - 10.1186/2045-3329-2-15 [doi]
AB  - Children diagnosed with the hereditary form of retinoblastoma (Rb), a rare eye 
      cancer caused by a germline mutation in the RB1 tumor suppressor gene, have 
      excellent survival, but face an increased risk of bone and soft tissue sarcomas. 
      This predisposition to sarcomas has been attributed to genetic susceptibility due 
      to inactivation of the RB1 gene as well as past radiotherapy for Rb. The majority 
      of bone and soft tissue sarcomas among hereditary Rb survivors occur in the head, 
      within the radiation field, but they also occur outside the radiation field. 
      Sarcomas account for almost half of the second primary cancers in hereditary Rb 
      survivors, but they are very rare following non-hereditary Rb. Sarcomas among 
      hereditary Rb survivors arise at ages similar to the pattern of occurrence in the 
      general population. There has been a trend over the past two decades to replace 
      radiotherapy with chemotherapy and other focal therapies (laser or cryosurgery), 
      and most recently, chemosurgery in order to reduce the incidence of sarcomas and 
      other second cancers in Rb survivors. Given the excellent survival of most Rb 
      patients treated in the past, it is important for survivors, their families and 
      health care providers to be aware of the heightened risk for sarcomas in 
      hereditary patients.
FAU - Kleinerman, Ruth A
AU  - Kleinerman RA
AD  - Epidemiology and Biostatistics Program Division of Cancer Epidemiology and 
      Genetics, National Cancer Institute, National Institutes of Health, 6120 
      Executive Boulevard, Rockville, MD 20852, USA. kleinerr@mail.nih.gov.
FAU - Schonfeld, Sara J
AU  - Schonfeld SJ
FAU - Tucker, Margaret A
AU  - Tucker MA
LA  - eng
PT  - Journal Article
DEP - 20121004
PL  - England
TA  - Clin Sarcoma Res
JT  - Clinical sarcoma research
JID - 101577890
PMC - PMC3499233
EDAT- 2012/10/06 06:00
MHDA- 2012/10/06 06:01
PMCR- 2012/10/04
CRDT- 2012/10/06 06:00
PHST- 2011/10/07 00:00 [received]
PHST- 2012/01/23 00:00 [accepted]
PHST- 2012/10/06 06:00 [entrez]
PHST- 2012/10/06 06:00 [pubmed]
PHST- 2012/10/06 06:01 [medline]
PHST- 2012/10/04 00:00 [pmc-release]
AID - 2045-3329-2-15 [pii]
AID - 10.1186/2045-3329-2-15 [doi]
PST - epublish
SO  - Clin Sarcoma Res. 2012 Oct 4;2(1):15. doi: 10.1186/2045-3329-2-15.