The aim of this trial is to determine preliminary activity of the combination treatment with nivolumab and entinostat in children and adolescents with high risk refractory/relapsed/progressive tumors harboring a high mutational load, focal MYC(N) amplification or ATRT-MYC subgroup as well as tumors with high tumor infiltrating lymphocytes (TILs) or a tertiary lymphoid structure (TLS).
Children and adolescents with refractory/relapsed/progressive high-risk
CNS tumors: medulloblastoma, ependymoma, ATRT, ETMR, pediatric high grade glioma (including DIPG) or other pediatric embryonal CNS tumors OR
solid tumors: neuroblastoma, nephroblastoma, rhabdoid tumor, embryonal or alveolar rhabdomyosarcoma, other embryonal small round blue cell tumors including pediatric type (bone) sarcoma or other pediatric type solid tumors OR
Children and adolescents with newly diagnosed high grade glioma (HGG) in the context of a constitutional mismatch repair deficiency syndrome after maximum safe surgical resection with no established standard of care treatment option with curative intention available. In addition in France: ineligible to radiotherapy
Patients with CNS tumors or metastases who are neurologically unstable despite adequate treatment (e.g. convulsions).
Patients with low-grade gliomas or tumors of unknown malignant potential are not eligible
Evidence of > Grade 1 recent CNS hemorrhage on the baseline MRI scan.
Tumor with any evidence of uncal herniation or severe midline shift
University Hospital Heidelberg
Lead sponsor
German Cancer Research Center
Collaborator