Desmoplastic small round cell tumor: an update of current management practices

Desmoplastic small round cell tumor (DSRCT) poses a diagnostic challenge, initiating with imaging techniques like ultrasound, CT, MRI, and PET scans, with CT being the primary choice for abdominal tumor visualization. Despite advances, the absence of a standardized staging system complicates the diagnostic process.

A comprehensive literature review and search strategy, encompassing databases like PubMed and Cochrane was performed.

Systemic chemotherapy, notably the P6 regimen, is the cornerstone of DSRCT treatment, while other options, including CDK4/6 inhibitors, antibody-drug conjugates, and anlotinib present varying efficacy. A novel chemotherapeutic agent, ONC-201, exhibits promise, inhibiting tumor growth in preclinical models. Surgical management, encompassing cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (HIPEC), reveals improved survival rates, particularly when combined with chemotherapy. Whole abdomen radiotherapy (WART) emerges as a post-chemotherapy treatment option, despite potential complications. A study employing whole abdominopelvic intensity-modulated radiation therapy (WAP-IMRT) suggests reduced radiation toxicity compared to conventional WART. Immunotherapy, targeting receptors such as B7-H3, GD2, EGFR, HER2, tyrosine kinase inhibitors, and androgen receptors is a promising avenue, especially in younger populations, given its favorable long-term effects. Additionally, the inclusion of CCDN1 genomic alterations further informs potential targeted therapies for DSRCT.

This comprehensive review provides a current understanding of DSRCT diagnosis and treatment modalities, highlighting the ongoing challenges and promising avenues for future research. The integration of personalized approaches, novel chemotherapeutic agents, and evolving immunotherapy strategies holds the potential to enhance outcomes for individuals with DSRCT.