Irinotecan and vincristine for the treatment of refractory desmoplastic small round cell tumor in a developing country: a case report

• Main Authors: Natalia Brenneken Duarte Ambar, Maria Teresa de Seixas Alves, Henrique Manoel Lederman, Simone Abib, Alexandre Alberto Barros Duarte, Eliana Monteiro Caran
• Format: Article
• Language: English
• Published: BMC 2019-03-01
• Series: Journal of Medical Case Reports
• Subjects: Desmoplastic small round cell tumor (DSRCT); Irinotecan; Chemotherapy
• Online Access: http://link.springer.com/article/10.1186/s13256-019-1985-z

Abstract Introduction Desmoplastic small round cell tumor is an extremely rare and aggressive cancer that affects mainly adolescents and young adults. Despite multiple therapeutic strategies, most patients have resistant disease with very poor survival rates. Case presentation We present a case of a 10-year-old Caucasian boy with a desmoplastic small round cell tumor refractory to conventional treatment who exhibited a good response to alternative treatment. With use of irinotecan and vincristine in association with radiation therapy, a reduction of 96.9% of the dimensions of the target lesions compared with the initial image was observed. Conclusion This chemotherapy regimen, in association with radiation therapy, demonstrated efficacy for refractory desmoplastic small round cell tumor in our patient, and it is cost-effective.