Current Status and Progress of Targeted and Immunotherapy for DSRCT

Area of Science:

• Oncology
• Molecular Biology
• Genetics

Background:

• Desmoplastic small round cell tumor (DSRCT) is a rare, highly malignant neoplasm predominantly affecting young males.
• DSRCT presents significant clinical challenges due to its aggressive nature, invasiveness, and poor prognosis.

Purpose of the Study:

• To summarize the pathogenic mechanisms of DSRCT.
• To review current and emerging therapeutic targets for DSRCT, including targeted therapies and immunotherapies.
• To discuss the application of these targets in clinical treatment strategies.

Main Methods:

• Literature review of studies on DSRCT pathogenesis and treatment.
• Analysis of targeted therapy targets (e.g., PDGF, VEGFR, AR) and immunotherapy targets (e.g., PD-1, B7H3).
• Examination of treatments targeting the DNA damage response pathway.

Main Results:

• Comprehensive treatment involving surgery, chemotherapy, targeted therapy, and immunotherapy is the current clinical consensus.
• Targeted therapies and immunotherapies show potential in inhibiting tumor progression and extending survival, but with variable efficacy.
• DNA damage response-related treatments are also being investigated for DSRCT.

Conclusions:

• While current treatments offer some benefit, their efficacy is limited and varies among patients.
• Further research into DSRCT's molecular mechanisms is crucial for discovering more effective therapeutic targets.
• Future efforts should focus on identifying novel strategies to overcome treatment resistance and improve survival rates for DSRCT patients.