Optimal Multimodal Treatment for Desmoplastic Small Round Cell Tumors—Reply

In Reply We thank Mir and colleagues for their thoughtful comments and appreciate the opportunity to respond. First and foremost, our article is an overview of DSRCT in general, not intended for management of the case specifically. Management of desmoplastic small round cell tumor (DSRCT) has been challenging because of its rarity and limited prospective data. Hyperthermic intraperitoneal chemotherapy (HIPEC) has been investigated following cytoreductive surgery. We agree that the use of HIPEC is probably not indicated in patients with extraperitoneal disease. Hayes-Jordan et al conducted a phase 2 study of HIPEC in pediatric patients with sarcoma including DSRCT in which patients with the extra-abdominal extension who could not achieve complete cytoreduction at the time of enrollment were excluded. Adjuvant whole-abdominal radiation therapy (WART) combined with cytoreductive surgery and HIPEC provides adequate local control of DSRCT. And yet it is imperative to recognize the risks of severe complications due to this extensive irradiation. Osborne et al reviewed 32 patients with DSRCT treated with chemotherapy, cytoreductive surgery with HIPEC, followed by WART in which 84% of patients had grade 3 or higher toxic effects. It was concluded that WART following chemotherapy, surgical cytoreduction, and HIPEC is an aggressive treatment for patients with DSRCT and can result in severe adverse effects.

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