How should young patients with rhabdomyosarcoma be managed in the absence of a clinical trial?

-Are there settings where group II disease does not need RT? -Are there additional genetic alterations in fusion negative RMS patients that can be used to further risk-adapt therapy? -Should we uniformly apply RT guidelines regardless of age (what about infants)? -Should RT doses change in light of the higher failure w/low dose cyclophosphamide or should we use higher doses of cyclo?

1 Answer

Mednet MemberInvited Expert

Radiation Oncology · University of Florida College of Medicine

Answered on · Updated on

The guiding principles of disease control while maintaining form, function, and quality of life drive the decision-making and management for patients with rhabdomyosarcoma.

Regarding patients with paratesticular primary, inguinal orchiectomy with no scrotal invasion but tumor on the surface of the removed testes is classified as group II. There are data to support a high local control rate in the absence of radiation in this setting. Therefore, we recommend the omission of radiation for this specific cohort of group II disease (Routh et al; PMID 32525556). Radiotherapy is recommended for all other Group II disease, as well as for patients who have Group III disease at diagnosis and undergo a DPE, even with an R0 or R1 resection.

While MYOD1 m...

Join for free or sign in to see the full answer

How should young patients with rhabdomyosarcoma be managed in the absence of a clinical trial?

What dose of RT should be used for group 3 RMS following chemotherapy w/a CR?