Immunotherapy and Radioimmunotherapy for Desmoplastic Small Round Cell Tumor.

Desmoplastic small round cell tumor (DRSCT) is a highly aggressive primitive sarcoma that primarily affects adolescent and young adult males. The 5-year survival rate is 15-30% and few curative treatment options exist. Although there is no standard treatment for DSRCT, patients are most often treated with a combination of aggressive chemotherapy, radiation, and surgery. Targeted therapy inhibitors of PDGFA and IGF-1R, which are almost uniformly overexpressed in DSRCT, have largely failed in clinical trials. As in cancer in general, interest in immunotherapy to treat DSRCT has increased in recent years. To that end, several types of immunotherapy are now being tested clinically, including monoclonal antibodies, radionuclide-conjugated antibodies, chimeric antigen receptor T cells, checkpoint inhibitors, and bispecific antibodies (BsAbs). These types of therapies may be particularly useful in DSRCT, which is frequently characterized by widespread intraperitoneal implants, which are difficult to completely remove surgically and are the frequent cause of relapse. Successful treatment with immunotherapy or radioimmunotherapy following debulking surgery could eradiate these micrometasteses and prevent relapse. Although there has been limited success to date for immunotherapy in pediatric solid tumors, the significant improvements in survival seen in the treatment of other pediatric solid tumors, such as metastatic neuroblastoma and its CNS spread, suggest a potential of immunotherapy and specifically compartmental immunotherapy in DSRCT.
Competing Interests: N-KC reports receiving commercial research grants from Y-mAbs Therapeutics and Abpro-Labs Inc.; holding ownership interest/equity/options in Y-mAbs Therapeutics Inc., and in Abpro-Labs, and owning stock options in Eureka Therapeutics. N-KC is the inventor of pending and issued patents filed by MSK, including hu3F8 and 8H9 licensed to Y-mAbs Therapeutics, beta-glucan to Biotec Pharmacon, and HER2 bispecific antibody to Abpro-labs. N-KC is an advisory board member for Abpro-Labs and Eureka Therapeutics. The remaining author declares that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.
(Copyright © 2021 Espinosa-Cotton and Cheung.)