In this review lecture on questions of modern approaches to radiation therapy (RT) at children and adolescents, actual concepts on volumes of RT, ways of its’ determination and correction, possible ways of decreasing of side effects are given. Lecture demonstrates new methods of RT including tomotherapy, which was firstly introduced for children in Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, presented the possibilities of multicenter and international collaboration to improve the situation in the field of radiology for children and adolescents.

Neuroblastoma, a malignant embryonal tumor of early childhood, has the unique feature of regression after mild or even no chemotherapy in low risk patients. In contrast, most high-risk patients die of disease despite intensive multimodality treatments. It is, therefore, an ideal model tumor for establishing individualized therapies. For many years, neuroblastoma patients have undergone risk adapted treatment according to clinical and molecular characteristics of the patient and the tumor, respectively, at the time of diagnosis. Recently, other approaches such as treatment modifications based on response to treatment as well as targeted molecular therapies directed against distinct abnormal pathways are becoming increasingly important. Every approach must rely on prospectively evaluated treatment strategies.

The results of treatment children with acute myeloid leukemia (AML) are not satisfied yet. The standard chemotherapy allows achieve complete remission in 92–96 % of patients, but disease free survival (DFS) and event free survival (EFS) are not good yet.
In a new study – NII DOG AML 2012 – the specific aim was to explore effectiveness demethylating drug (Decitabine) and inhibitors of histon deacetylase (HDAC) to find the place in standard chemotherapy.
From 01.2013 to 09.2016 26 patients were enrolled into NII DOG AML 2012 study. The average was 6.5 ± 1.24 years (6 mo – 16 years): 15 males and 11 females, standard (SR) – 2 (7.7 %), intermediate (IR) – 7 (26.9 %) and high (HR) – 17 (65.4 %) risk groups.
Chemotherapy consisted on 5 courses for HR and IR (AIE, HAM, AI, hAM, HAE) and 4 courses for SR (AIE, HAM, hAM, HAE). Epigenetic therapy consisted of Valproic acid (VA) weeks 1–78, All Trans Retinoic Acid (ATRA) 1–43 days and from the day one to day 14 of the every course chemotherapy and Decitabine in “window” regime before induction (5 pts) and on day 16 after beginning of induction.
Decitabine was given as a demethylating drug 20 mg/m2 for 5 days in “window” regime before induction (AIE). There were no any toxicity and we did not check decrease of blasts in BM and peripheral blood after Decitobine, one of them developed relapse and one died from severe infection, 3 pts are alive, but two of them underwent haploidentical HSCT. Decitobine was moved on the day 16. Now, 21 pts got this therapy, all of them achieved CR after AIE with VA, ATRA and Decitobine, two pts did not respond to induction and achieved CR just after Decitobine. Three years DFS and EFS were the same – 67.9 ± 12 % with median follow up 28.1 ± 3.1 mo, OS – 81.6 ± 9.6 % with median follow up 31.0 ± 2.6 mo. None of the patients underwent HSCT.
Thus, epigenetic therapy increases rate of CR, DFS and EFS in children with AML. Demethylating drug has to be used during aplasia and HDAC inhibitors – during the whole chemotherapy program.

This article presents modern situation in childhood cancer treatment in high- and low/middle-income countries and position of WHO on this question. Authors suggest that it is very important to collaborate to reach success. Work of WHO on improvement of care and treatment for children with cancer presented. WHO strategy to control childhood cancer presented and to improve access to essential medicine for all affected children

Creation of monoclonal antibodies for leukaemia treatment is rapidly developing area of science. A number of compounds were effective for treatment of acute lymphoblastic leukaemia (ALL) at children. While unconjugated humanized antibodies have good tolerability and can be combined with chemotherapy, immunoconjugates, delivering the toxic compounds directly to the target cells, have more serious adverse effects.
Antigens with high and selective expression are the ideal targets for usage with antibodies and suitable for studies of phases I/II and III in case of pediatric ALL. Antigens with stable expression on the cells surfaces, suitable both for unconjugated antibodies, leading to antibodies-dependent cell and complement-dependent cytotoxicity, and for bispecific antibodies with participation of T-cells. Monoclonal antibodies have quite different mechanism of action in comparison with routine chemotherapy and, of course, can change the strategy of ALL treatment in future.

Manuscript presents actuality of question of import of unregistered drugs on the territory of Russian Federation, clear step-by-step instructions to get these medications given. Main complexity and features of process organization presented, supplement is presented. This manuscript was prepared by the materials of symposia held during the 2nd Petersburg Oncological Forum “White nights” June 22–24 2016.

Paroxysmal nocturnal hemoglobinuria (PNH) at children presents at children rarely in comparison with adults, but clinical presentation characterized by the same symptoms and complications like at the adult population of patients. Children present with chronic hemolysis, thrombosis, abdominal pain, infection complications, fatigue, decreasing of quality of life. In this case, the feature of PNH at children is predominant combination of diseases with bone marrow failure (most common, aplastic anemia) and relatively rare hemoglobinuria, what can complicate in-time diagnosing. Blood testing with the help of flow cytometry allows establishing diagnosis and risking groups for PNH determination presented in the international guidelines. In-time diagnostics of diseases is very important for treatment tactics chosen and prognosis, and gain the high importance with the appearance of possibility of pathogenetic therapy by eculizumab medication. Eculizumab is the humanized monoclonal antibody which inhibits the activity of complement system and prevents development of chronic intravascular hemolysis in case of PNH. Effectiveness and safety of eculizumab were proven in clinical studies both for children and adults. This article presents own experience of treatment of 2 children with PNH on the base of Uliyanovsk Regional Clinical Hospital.

Paroxysmal nocturnal hemoglobinuria (PNH) can be found at children and adolescents, but often diseases not diagnosed in time and patients cannot receive adequate treatment for a long period. Thus, in presented clinical case the diagnosis of PNH was firstly established after 4 years from the moment of first admission to the hospital and after 6 years from the debut of disease. Since the PNH-clone can be found mainly at patients of child age with aplastic anemia (AA) or with myelodisplastic syndrome, screening is required for such patients to identify the PNH. For children with AA, even in case of absence of hemolysis clinical presentation, screening is recommended at least 1 time per year during the subsequent observation.
Before appearance of eculizumab the main methods of PNH treatment in complex with bone marrow failure were blood transfusions and symptomatic therapy, as well as allogenic bone marrow transplantation (BMT). Though the above-mentioned method allows achieving healing of PNH, BMT can be associated with high risk of complications and death. In presented case, mother of girl refuse of BMT. Treatment if AA lead to partial recovery of hematopoiesis, but the severity of patients’ health determined by severe course of chronic intravascular hemolysis and hemolytic crises due to PNH. Eculizumab treatment allows to effectively control of hemolysis, significantly decrease transfusion dependent and improve the quality of life of child.

The article presents a case report describing the difficulty of radiation and intraoperative differential diagnosis of nephroblastoma and multicystic dysplastic kidney in a child.

Nephroblastoma is one of the most common tumours of children. For correct clinical management and determine of tactics of chemotherapy it is required to set histological type of nephroblastoma and risk group. In this clinical example presented the case of stromal type of nephroblastoma of children of first year of life.

The article describes the clinical case and the complexity of the staging of the disease the patient’s first year of life with neuroblastoma (NB) of the neck. Shows diagnostic search aimed at identifying distant metastasis of the tumor. Highlighted perform scintigraphy with 123I metaiodobenzylguanidine to detect distant metastases in patients with NB and correct stratification of patients into risk groups.