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 ¹

Revised text ² to state that the prognosis of rhabdomyosarcoma is also related to the widest diameter of the tumor.

Revised text ³ 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 ³ 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 ³ to state that the Clinical Group is now referred to as the Surgico-pathologic Group.

Revised text ⁴ to state that tumor tissue should be reviewed by pathologists and cytogeneticists/molecular geneticists.

Cellular Classification ⁵

Added text ⁶ to state that around 22% of cases have no detectable PAX gene translocation.

Stage Information ⁷

This section was extensively revised.

Previously Untreated Childhood Rhabdomyosarcoma ⁸

Revised text ⁹ 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 ¹⁰.

Added Corpron et al. as reference 49 ¹¹.

Added Cribbs et al. as reference 53 ¹².

Added text ¹³ 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 ¹⁴.

Added text about the COG-ARST0331 ¹⁵ 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 ¹⁶

Added text ¹⁷ 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 ¹⁸ that treatment with topotecan, vincristine, and doxorubicin are under clinical investigation for recurrent rhabdomyosarcoma (cited Meazza as reference 18 and level of evidence 3iiiDiii).