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

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.

Added text to state that the International Society of Pediatric Oncology Malignant Mesenchymal Tumor group found that RT improved local control in patients with localized pelvic rhabdomyosarcoma whose initial surgical procedure was biopsy only, leaving macroscopic residual tumor. Age older than 10 years and lymph node involvement were unfavorable prognostic factors (cited Réguerre et al. as reference 73 and level of evidence 2A).

Added text to state that the Soft Tissue Sarcoma Committee of the COG recommends that RT be administered to patients with residual viable vaginal tumor, beginning at week 24.

Added text to state that 12 of 14 girls with primary cervical embryonal rhabdomyosarcoma were disease-free following VAC chemotherapy and conservative surgery; of note, two girls also had a pleuropulmonary blastoma and another had Sertoli-Leydig cell tumor (cited Dehner et al. as reference 98).

Added text to state that in a European trial of 457 patients with incompletely resected embryonal rhabdomyosarcoma, alveolar rhabdomyosarcoma, undifferentiated sarcoma, or soft tissue primitive neuroectodermal tumor, the addition of carboplatin, epirubicin, and etoposide to standard ifosfamide, vincristine, and dactinomycin therapy did not improve outcome (cited Oberlin et al. as reference 118).

Added text to state that the pilot 1 and pilot 3 studies of the COG-ARST08P1 trial are now closed.

This summary is written and maintained by the PDQ Pediatric Treatment Editorial Board, which is editorially independent of NCI. The summary reflects an independent review of the literature and does not represent a policy statement of NCI or NIH. More information about summary policies and the role of the PDQ Editorial Boards in maintaining the PDQ summaries can be found on the About This PDQ Summary and PDQ NCI's Comprehensive Cancer Database pages.