Desmoplastic small round cell tumour (DSRCT) is a rare and highly aggressive neoplasm, typically diagnosed in adolescents and young adults. With fewer than 500 cases described worldwide, treatment is multimodal and challenging, involving intensive chemotherapy, cytoreductive surgery, and radiotherapy. We present two clinical cases of adolescents diagnosed with advanced abdominal DSRCT, exhibiting distinct clinical courses. Emphasis is placed on the role of whole abdominal radiotherapy in locoregional control within a multidisciplinary approach. Therapeutic options, associated complications, and clinical management challenges are discussed.
Catalo et al. (2026) studied this question.