Table 3.

Immunotherapy trials including Wilms tumor patients

Study/trial	Immunotherapeutic agent	Targeted molecule	Phase	Patients enrolled	No. of Wilms tumor patients	Results
Hont et al. J. Clin. Oncol. 201932	TAA-Ts	WT1 PRAME survivin	phase I	relapsed and refractory solid tumors	9	safe and immunogenic; response rate: 73%
Dohnal et al. Cytotherapy 200736	DC-based vaccine	–	phase I	advanced solid pediatric malignancies	2	safe and immunogenic
Geoerger et al. Lancet Oncol. 20206/KEYNOTE-051 trial	pembrolizumab (mAb)	PD-L1	phase I–II	PD-L1+, advanced relapsed or refractory solid pediatric tumor or lymphoma	3	ongoing trial; response only in Hodgkin’s lymphoma
Merchant et al. Clin. Cancer Res. 2016108	ipilimumab (mAb)	CTLA4	phase I	advanced solid pediatric malignancies	3	no objective tumor regressions
Modak et al. Cancer Res. 2018 NCT0109964445	IP 131I-8H9 (radio-conjugated mAb)	B7-H3	phase I	desmoplastic round cell tumors	not disclosed	satisfactory safety profile and promising antitumor activity; a phase II is ongoing.
NCT02982941	enoblituzumab (mAb)	B7-H3	phase I	B7-H3+ relapsed or refractory malignant solid tumors	not disclosed	ongoing trial; no results published
Cacciavillano et al. Pediatr. Blood Cancer 201554	racotumomab (mAb)	NeuGc-GM	phase I	pediatric refractory malignancies	1	safe and immunogenic; no regression reported.
Tsuchiya et al. OncoImmunology 201757	GPC3-peptide vaccination	GPC3	phase I	pediatric refractory solid malignancies	1	safe and immunogenic; induced response in 1/2 WT patients
Seibel et al. J. Immunother. Emphasis Tumor Immunol. 199478	rTNF-α + actinomycin	TNF-α	phase I	pediatric refractory malignancies	6	tumor response in 1/6 WT patients
Meany et al. J. Immunother. 200879	rTNF-α + actinomycin	TNF-α	phase II	recurrent Wilms tumor	19	safe, complete tumor response in 3/19 patients

Main immunotherapy clinical trials including WT patients are listed. TAA-Ts, tumor-associated antigen cytotoxic T cells; mAb, monoclonal antibody.