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Objective: Nivolumab is a human monoclonal antibody to programmed cell death receptor 1 (PD-1) that acts as an immune checkpoint inhibitor and is used in the immunotherapy of various types of advanced or metastatic cancer. The aim of this study is to evaluate the efficacy of nivolumab in pediatric patients with various highly malignant tumors and to share the experience of Ankara University Pediatric Oncology Department. Methodology: Eight patients were included in the study. Median age at diagnosis is 11.3 years (min 4.9- max 13.9). Treatment indications were malignant mesothelioma (1), rectal adenocarcinoma (1), malignant melanoma (1), ewing sarcoma (2), osteosarcoma (1), non-hodgkin lymphoma (1) and hodgkin lymphoma (1). Results: Four patients died due to progressive disease. Complete remission was achieved in four patients diagnosed with malignant mesothelioma, rectal adenocarcinoma, malignant melanoma and Hodgkin lymphoma. Conclusion: Immune checkpoint inhibitors are one of the greatest advances in oncological therapy and improve the overall survival of patients with advanced and resistant malignancies. More studies are needed to evaluate the efficacy of immune checkpoint inhibitors in pediatric tumors.

Case report In Langerhans cell histiocytosis thyroid involvement is rarely seen. Here, we would like to present a 12-year-old male patient with lung, external auditory canal skin and lymph node involvement in diagnosis. Disease relapse occurred with thyroid involvement 19 months after remission. In molecular analysis, BRAF p.N 486 _P490del was detected and he reccived MEK inhibitor Trametinib monotherapy. He is still in remission for 16 months.

Case report Gastrointestinal stromal tumors (GISTs) occur exceedingly rare in children and adolescents. Eighty five percent of pediatric GISTs and 15 % of adult GISTs lack oncogenic mutations in KIT and PDGFRA. The results of tyrosine kinase inhibitor therapy in GIST cases with SDH deficiency are limited and controversial. Here, we would like to present a pediatric SDH deficient GIST case treated with surgery and Imatinib Mesylate. We obtained a good response with Imatinib Mesylate.