The Potential Role of Immunotherapy in Wilms’ Tumor: Opportunities and Challenges

Abstract: Wilms' tumor (WT) is the most common renal malignancy in children, accounting for more than 90% of all pediatric renal cancers. Although this tumor is generally responsive to treatment, relapses and deaths still occur in a significant proportion of patients. The genetic alterations commonly found in WT and also its unique histological features and the tumor microenvironment suggest that the immune system may play a crucial role in the disease's development and progression. The limitations of conventional therapies, including surgery, chemotherapy, and radiotherapy, in preventing recurrence in WT patients and their potential for exerting long-term side effects, necessitate the application of novel therapeutic strategies, like immunotherapy, in this disease. Immunotherapy is an emerging cancer treatment approach based on the concept of harnessing the patient's immune system to fight tumor cells. This approach has demonstrated promising results in various types of cancers due to its relatively high specificity, efficacy, and tolerability. However, the precise effects of immunotherapy in WT remain to be explored. For this purpose, this review highlights the potential implication of different immunotherapy approaches, like monoclonal antibodies, adoptive cell therapy, and immune checkpoint inhibitors, in patients with WT, with a particular emphasis on the tumor's genetic and histological features. Although much remains to be learned about the optimal use of immunotherapy for this disease, the available evidence suggests that immunotherapy has the potential to significantly improve outcomes for patients with WT. However, there is still a substantial need for conducting further studies, especially randomized controlled trials, to determine the most effective immunotherapy strategy for this tumor. Moreover, the potential beneficiary roles of the combination of immunotherapy and conventional treatments should be investigated in future research.