What is the standard of care (if any) for pediatric desmoplastic round cell tumor?

I am an adult oncologist and will defer to a pediatric oncologist if one is available to specifically address the question asked. Multidisciplinary evaluation in a sarcoma center is essential. For adults with this diagnosis, we manage desmoplastic small round cell tumors along the lines of Ewing sarcoma. In general, we treat these with neoadjuvant chemotherapy with a Ewing sarcoma regimen and if possible cytoreductive surgery. Occasionally other modalities such as HIPEC or radiotherapy can be incorporated depending on the extent of disease. A recent article by Subbiah et al (PMID 29871905) discusses this approach.

I am not aware of a role for immunotherapy for DSRCT outside the setting of a clinical trial.