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Majority of cases are sporadic and the etiology is unknown.

Increased incidence of rhabdomyosarcoma (RMS) associated with:


Li-Fraumeni familial cancer syndrome:

  • An autosomal dominant disorder associated with a significantly increased risk of:
    • RMS
    • Early onset breast cancer
    • Osteogenic sarcoma
    • Adrenocortical carcinoma
    • Glioblastomas and other neoplasms. 
  • In 1990 a germline mutant p53 tumor suppresser gene was identified in families with this syndrome.


Rhabdomyosarcoma (RMS) also associated with:

Maternal or paternal use of marijuana and cocaine also increases the risk of rhabdomyosarcoma in their child.

Congenital abnormalities may be associated with an increased risk of RMS, mostly involving the CNS, GI tract, GU tract and cardiovascular system.








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