How are nonoperative treatments selected for pediatric rhabdomyosarcoma (RMS)?

Updated: Mar 28, 2019
  • Author: Roshni Dasgupta, MD, MPH; Chief Editor: Eugene S Kim, MD, FACS, FAAP  more...
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Answer

Current recommendations stem from IRS-V, in which treatment is stratified according to risk—low, intermediate, or high—on the basis of risk of disease recurrence and overall survival. Patients are then staged on the basis of the primary tumor site. Favorable sites include the orbit, nonparameningeal head and neck sites, and genitourinary nonbladder/nonprostate sites; all other sites are unfavorable. [13]

Staging is further refined on the basis of primary tumor size (< 5 cm or ≥5 cm), regional lymph node involvement, distant metastases, and histologic subtype (see Staging). Alveolar subtypes are now stratified according to gene fusion status.


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