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Childhood Rhabdomyosarcoma Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Previously Untreated Childhood Rhabdomyosarcoma

Table 5. Radiation Therapy (RT) Dose According to Rhabdomyosarcoma Group, Histology, and Site of Disease (Children's Oncology Group [COG]) continued...

Chemotherapy Treatment Options

All children with rhabdomyosarcoma should receive chemotherapy. The intensity and duration of the chemotherapy are dependent on the Risk Group assignment.[105] See Table 4 in the Stage Information section for more information about Risk Groups.

Adolescents treated with therapy for rhabdomyosarcoma experience less hematologic toxicity and more peripheral nerve toxicity than do younger patients.[106]

Low-risk patients

Standard treatment options

  • Low-risk patients have localized (nonmetastatic) embryonal histology tumors in favorable sites that have been grossly resected (Groups I and II), embryonal tumors in the orbit that have not been completely resected (Group III), and localized tumors in an unfavorable site that have been grossly resected (Groups I and II). (See Table 3 in the Stage Information section of this summary.) Certain subgroups of low-risk patients have achieved survival rates higher than 90% when treated with a two-drug chemotherapy regimen that includes vincristine and dactinomycin (VA) plus RT for residual tumor. See Table 6 below.

Table 6. Characteristics of Low-Risk Patients with High Survival Rates Using Two-Drug Therapy with Vincristine and Dactinomycin With or Without Radiation Therapy (Subset A)

SiteSizeGroupNodes
N0 = absence of nodal spread.
FavorableAnyI, IIAN0
OrbitalAnyI, II, IIIN0
Unfavorable≤5 cmIN0

The COG-D9602 study stratified 388 patients with low-risk embryonal rhabdomyosarcoma into two groups.[107] Treatment for subgroup A patients (n = 264; Stage 1 Group I/IIA, Stage 2 Group I, and Stage 1 Group III orbit) consisted of VA with or without RT for 48 weeks. Patients with subgroup B disease (n = 78; Stage 1 Group IIB/C, Stage I Group III nonorbit, Stage 2 Group II, and Stage 3 Group I/II disease) received VAC (total cumulative dose of 28.6 g/m2). Radiation doses were reduced from 41.4 Gy to 36 Gy for Stage 1 Group IIA patients and from 50 Gy or 59 Gy to 45 Gy for Group III orbit patients. For subgroup A patients, the 5-year overall FFS rate was 88% and the OS rate was 97%. For subgroup B patients, the 5-year FFS rate was 85% and the OS rate was 93%.

Other subgroups of low-risk patients have achieved survival rates of at least 90% with three-drug chemotherapy with VAC (total cyclophosphamide dose of 28.6 g/m2) plus RT for residual tumor. See Table 7 below.

Table 7. Characteristics of Low-Risk Patients with High Survival Rates Using Three-Drug Therapy with Vincristine, Dactinomycin, and Cyclophosphamide With or Without Radiation Therapy (Subset B)

SiteSizeGroupNodes
N0 = absence of nodal spread; N1 = presence of regional nodal spread beyond the primary site.
Favorable (orbital or non-orbital)AnyIIB, IIC, IIIN0, N1
Unfavorable≤5 cmIIN0
Unfavorable>5 cmI, IIN0, N1
1|2|3|4|5|6|7|8|9|10|11|12|13

WebMD Public Information from the National Cancer Institute

Last Updated: February 25, 2014
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