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Changes to This Summary (04 / 01 / 2013)

    The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.

    General Information

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    Revised text to state that genetic conditions associated with rhabdomyosarcoma include Li-Fraumeni cancer susceptibility syndrome (with germline p53 mutations), pleuropulmonary blastoma (with DICER1 mutations), neurofibromatosis type I, Costello syndrome (with germline HRAS mutations), Beckwith-Wiedemann syndrome (with which Wilms tumor and hepatoblastoma are more commonly associated), and Noonan syndrome (cited Dehner et al., Doros et al., Kratz et al., and Hasle as references 15, 16, 21, and 25, respectively).

    Added Streby et al. and Van Gaal et al. as references 40 and 41, respectively.

    Cellular Classification

    Added Dumont et al. as reference 12.

    Added Missiaglia et al. and Duan et al. as references 19 and 20, respectively.

    Added text to state that in another study, gene expression signature did not appear to add additional prognostic information beyond that available from the contribution of the PAX3/FOX01 fusion status.

    Treatment Option Overview

    Revised text to state that differences in outcome were most striking for patients with extremity and head and neck nonparameningeal tumors. Also added text to state that failure-free survival was lower for patients with bladder/prostate primary tumors who did not receive radiation therapy (RT) as part of their initial treatment, but there was no difference in overall survival between the two strategies for these patients (cited Rodeberg et al. as reference 4).

    Previously Untreated Childhood Rhabdomyosarcoma

    Added Yang et al. as reference 16.

    Added text to state that a retrospective review of patients with intermediate-risk rhabdomyosarcoma compared conformal RT and intensity-modulated radiation therapy (IMRT); IMRT improved the target coverage but did not show a difference in local failure rate or event-free survival (cited Lin et al. as reference 19 and level of evidence 2B).

    Added Table 5 on Children's Oncology Group (COG) RT doses according to rhabdomyosarcoma group, histology, and site of disease.

    Added text to state that low-risk patients treated on a COG study had local control with 36 Gy, which was comparable to historic controls who received 41.4 Gy.

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