Key result
Targeted and immunotherapy can inhibit DSRCT progression and prolong survival despite variable and limited efficacy.
Why the study?
Desmoplastic small round cell tumor is a rare, highly malignant, and invasive tumor with poor prognosis, making its treatment a major clinical challenge.
Targeted and immunotherapy offer potential benefits in inhibiting tumor progression and prolonging survival in DSRCT, though efficacy remains limited and highly variable.
Answers are grounded in this paper and cite their sources.
Targeted therapies support multimodal consensus in DSRCT; leaves open need for biomarkers to address variable, limited efficacy.
Desmoplastic small round cell tumor (DSRCT) is a rare and highly malignant tumor that mostly occurs in young males. Due to its extremely strong invasiveness and poor prognosis, the treatment of DSRCT remains a major challenge in current medical research. The comprehensive treatment strategy based on surgery, combined with chemotherapy, targeted therapy, immunotherapy has become a clinical consensus. This review summarizes the main pathogenic mechanisms of DSRCT, as well as the targets involved in treatment and their applications, including targeted therapy targets (PDGF, VEGFR, FGFR4, IGF1R, HER2, c-KIT, mTOR, AR), immunotherapy targets (PD-1, PD-L1, B7H3, GD2), and treatments related to DNA damage response. Studies have shown that treatments targeting specific targets can inhibit tumor progression and prolong patient survival to a certain extent, but the efficacy has individual differences and is still limited. Therefore, future research still needs to further explore the molecular mechanism of DSRCT and discover more accurate and effective therapeutic targets.
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Wei et al. (2026) conducted a review in Desmoplastic small round cell tumor (DSRCT). Targeted therapy and immunotherapy was evaluated. Targeted and immunotherapy for DSRCT can inhibit tumor progression and prolong patient survival, though efficacy shows individual differences and remains limited.
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