The use of interval-compressed chemotherapy with the addition of vincristine, irinotecan, and temozolomide for pediatric patients with newly diagnosed desmoplastic small round cell tumor

Liu KX, Collins NB, Greenzang KA, Furutani E, Campbell K, Groves A, Mullen EA, Shusterman S, Spidle J, Marcus KJ, Weil BR, Weldon CB, Frazier AL, Janeway KA, O’Neill AF, Mack JW, DuBois SG, Shulman DS. The use of interval-compressed chemotherapy with the addition of vincristine, irinotecan, and temozolomide for pediatric patients with newly diagnosed desmoplastic small round cell tumor. Pediatr Blood Cancer. 2020 Oct;67(10):e28559. doi: 10.1002/pbc.28559. Epub 2020 Jul 19. PubMed PMID: 32686305; PubMed Central PMCID: PMC7721987.

Desmoplastic small round cell tumor (DSRCT) is a rare aggressive sarcoma that affects children and young adults and portends poor outcomes despite intensive multimodal treatment approaches. We reporttoxicity, response and outcomes of patients with DSRCT treated with the addition of vincristine, irinotecan, and temozolomide (VIT) to interval-compressed chemotherapy as per Children’s Oncology GroupARST08P1. All newly diagnosed pediatric patients with DSRCT treated at Dana-Farber Cancer Institute and Boston Children’s Hospital between 2014 and 2019as per ARST08P1, Arm P2 with replacement of VAC cycles with VIT, were identified.Medical records were reviewed for clinical and disease characteristics, and treatment response and outcomes.