Phase II Study of Selinexor for Children and Young People With Relapsed or Refractory Wilms Tumor and Other Solid Tumors

What this Trial Is About

Researchers are evaluating selinexor as a potential treatment for people with relapsed or refractory Wilms tumor, rhabdoid tumor, malignant peripheral nerve sheath tumors MPNST, BCOR-driven sarcomas, or other solid tumors that produce higher than normal levels of the XPO1 protein or have genetic changes that increase XPO1 activity. This phase II study aims to understand selinexors effectiveness in these specific pediatric solid tumors, sponsored by Memorial Sloan Kettering Cancer Center. The study treats participants with selinexor at a pediatric recommended phase 2 dose of 35 mgm2 once weekly using a liquid form, with a maximum dose of 100 mg per dose. Participants are divided into cohorts based on their tumor type, including Wilms tumor, rhabdoid tumor, MPNST, other solid tumors showing potential benefit from selinexor, and BCOR-driven sarcomas. Each cohort follows the same treatment schedule, and some patients may be enrolled in pharmacokinetic cohorts depending on their age. During the study, participants will undergo assessments to monitor their disease status, including measurable disease evaluations using standardized criteria. Researchers will track the overall response rate six months after treatment begins to determine the treatments impact. Participants must meet specific health criteria, including adequate organ function and performance status. The study includes informed consent, safety monitoring, and follows participants until the end of the treatment period, which may last several months.

A Study of Selinexor in People With Wilms Tumors and Other Solid Tumors