How would you manage relapsed desmoplastic small round cell tumor?

Unfortunately, recurrent DSRCT has a dismal prognosis and management is generally with palliative intent. In cases where recurrence is localized, consideration of aggressive local control should be given when possible. There are no established, reliable chemotherapeutic regimens for relapsed DSRCT and thus I would encourage clinical trial enrollment when possible. Topotecan or irinotecan-based regimens are commonly used (if these agents were not included in the original therapy). Other agents with reported cases of clinical benefit include vinorelbine and cyclophosphamide (PO or IV) with or without temsirolimus, pazopanib, sunitinib, and eribulin. It is very important in these cases to work closely with the patient and their family to estab...