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.