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Childhood Rhabdomyosarcoma Treatment (PDQ®)
Patient VersionHealth Professional VersionEn españolLast Modified: 11/06/2009



Purpose of This PDQ Summary






General Information






Cellular Classification






Stage Information






Treatment Option Overview






Previously Untreated Childhood Rhabdomyosarcoma






Recurrent Childhood Rhabdomyosarcoma






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Changes to This Summary (11/06/2009)






More Information



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Changes to This Summary (11/06/2009)

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 the prognosis of rhabdomyosarcoma is also related to the widest diameter of the tumor.

Revised text to state that primary sites with more favorable prognoses include the orbit and nonparameningeal head and neck, paratestis, vulva, vagina, uterus (nonbladder, nonprostate genitourinary), and biliary tract.

Added text about a retrospective review of soft tissue sarcomas in children and adolescents that suggested that the 5 cm cutoff used for adults with soft tissue sarcoma may not be ideal for smaller children, especially infants; the review identified an interaction between tumor diameter and body surface area (BSA).

Revised text to state that the Clinical Group is now referred to as the Surgico-pathologic Group.

Revised text to state that tumor tissue should be reviewed by pathologists and cytogeneticists/molecular geneticists.

Cellular Classification

Added text to state that around 22% of cases have no detectable PAX gene translocation.

Stage Information

This section was extensively revised.

Previously Untreated Childhood Rhabdomyosarcoma

Revised text to state that the Children’s Oncology Group Soft Tissue Sarcoma Committee (COG-STS) recommends inguinal and femoral triangle node sampling for patients with lower-extremity primary tumors, even with clinically and radiographically negative nodes.

Added Raney et al. as reference 44.

Added Corpron et al. as reference 49.

Added Cribbs et al. as reference 53.

Added text to state that the total cyclophosphamide dose used in recently completed COG protocols was 28.6 gm/M2.

Added Martelli et al. as reference 93.

Added text about the COG-ARST0331 study, where there was an unacceptably high rate of local recurrence in girls with Group III vaginal tumors who did not receive RT. Also added text to state that the COG-STS is now recommending that RT be administered to patients with residual viable vaginal tumor, beginning at week 24.

Recurrent Childhood Rhabdomyosarcoma

Added text to state that a retrospective review of rhabdomyosarcoma patients from German soft tissue sarcoma trials, identified time to recurrence as an important independent prognostic factor. Also added text to state that a shorter time to recurrence was associated with higher risk of mortality from recurrent rhabdomyosarcoma (cited Mattke et al. as reference 4 and level of evidence 3iiB).

Added text that treatment with topotecan, vincristine, and doxorubicin are under clinical investigation for recurrent rhabdomyosarcoma (cited Meazza as reference 18 and level of evidence 3iiiDiii).

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