World cancer congress was held in Paris October 31 – November 3 2016 and organized by the Union for International Cancer Control (UICC). Questions of treatment of children with cancer were discussed at November 2. This article presents data on results of work of the session organized by the secretariat of the International Society of Paediatric Oncology (SIOP). The following questions were discussed during this session: accessibility of help for children with cancer in different parts of the World with an accent on developing countries, advocacy for children affected by cancer, pharmacoeconomics in pediatric oncology and work of SIOP PODC.

Acute myeloid leukemia (AML) occurs in frequency 1.5 per 100 000 children and occupy 15–20 % in the structure of pediatric acute leukemia. In comparison with adults, pediatric AML usually a primary disease; clonal evolution of myeloproliferative disorders is rare situation in clinical course of the disease. Pediatric AML classification based on combination of morphology, cytochemistry, immune phenotyping and molecular genetics and situated in the process of permanent revision. Doctrine of AML treatment, excluding acute promyelocytic leukemia, was not changed during last 30 years, but the survival rate increased from 40 to 60–65 % because of stratification of patients on risk groups, intensification of chemotherapy, application of epigenetic/target therapy, improvement of supportive care and widening of indications for hematopoietic stem cell transplantation in the clinical program of treatment.

Children in the age under 2 years old with AML is the unique group of patients with different biology of process, genetic aberrations, concentration of unfavorable risk factors and expressed predisposition to therapeutic toxicity. Progress of the treatment for this subgroup with usage of risk-based protocols of treatment during the last 10 years leads to change of prognosis – now the prognosis is the same in comparison with more adult groups of patients.

Thus, 25–30 % of patients with AML in the age of 0–18 years old can be faced with relapse of leukemia, 5–10 % will die because of complications of disease and/or side effects of therapy. Prospects of treatment optimization for children are connected with target therapy of different genetic variants of AML, like for promyelocytic leukemia or chronic myeloid leukemia, as well as in decreasing of the toxicity of therapy. Review of international studies for AML treatment for children is presented in this article as well as discussion on prospect of optimization of treatment.

Improvement of morphological techniques, molecular genetics, radiation diagnosis defined the modern hystomolecular classification of the brain tumors in children. Many tumors of the central nervous system (CNS) are associated with cancer predisposition syndromes. Future strategy for treatment of patients with CNS tumors becomes increasingly personalized.

This article provides recommendations for the brain and spinal cord magnetic resonance imaging (MRI) technique in children with neoplasms of the central nervous system (CNS). These guidelines are made up on the great first-hand experience of such studies in the Dmitry Rogachev National Research Center of Pediatric Hematology, Oncology and Immunology, experience in MRI reference of the CNS made in other medical institutions, and European experience in MRI of the CNS and reference analysis in the framework of international multicentre studies.

Introduction. The way of placing of Ommaya reservoir for patients with neurooncological and oncohematological diseases by the new gadget Thomale Guide without costly navigation equipment described in this article.

Materials and methods. Nineteenth surgeries with usage of Thomale Guide were done from April 2015 to August 2016 in Dmitry Rogachev NRC PHOI.

Results. In all 19 cases ventricular catheter was successfully placed to the lateral ventricle that was confirmed by successful functioning of Ommaya reservoir.

Conclusion. Simple and easy available Thomale Guide technique allows to implement the concept “to deliver equipment to a patient, but not a patient to equipment”. Nowadays specialist of Dmitry Rogachev NRC PHOI can place Ommaya reservoir in Regions of Russia in the clinics where the intraventricular chemotherapy must be done, but there is no technical possibility to place the Ommaya reservoir.

Actuality. Malignant tumors (MT) in adolescents are rare that critically impedes research on dynamics and structure of cancer pathology. An even greater challenge is to develop comprehensive base of patients’ contingents since special treatment is only possible in large federal centers and extracts from case histories in most cases do not reach cancer institutions in patients’ residency.

Such problems affect St. Petersburg smaller as treatment of patients of this group is carried out in the city in a number of specialized clinics, research institutes, medical universities and academies but even here there is a loss.

To obtain more accurate data on MT prevalence among children and adolescents it is primarily necessary to create children’s populationbased cancer registry in the scale of the Federal district with giving him the status of the Population-based Cancer Registry with the right of use of personalized data and close mutual work with existing cancer registries of all Population-based cancer registries of administrative territories of the Federal district. The purpose of the study is to compare dynamics and structure of MT prevalence among adolescents and to estimate in dynamics of observed and relative survival rates of adolescents with MT.

Materials and methods. The study materials were taken from database of the Population-based cancer registry of St. Petersburg and there were used a set of standard methods of statistical analysis and the method of estimation of survival by constructing tables of expectancy.

Analysis of data obtained. There was performed a precise analysis of comparative data of MT prevalence among young men and girls of St. Petersburg for 1994–2014. There were estimated rates of a 5-year observed and relative survival of adolescents. The findings suggest a substantial increase in morbidity rates and particularly in their survival.

Conclusion. The study showed positive dynamics of MT morbidity among adolescents, stable structure of cancer pathology among young males, some changes in the structure of cancer pathology among females, a significant increase of a 5-year observed and relative survival.

Effectiveness and safety of domestic recombinant factor VIII (FVIII) with removed B-domain (moroctocogum alfa, Octofactor®, JC “Generium”) usage for preventive treatment of 12 adolescents at age 12–17 years old with severe hemophilia A described in this article. Octofactor infused in dose 35 ± 5 ME/kg 3 times per week with the interval of 48 hours during the period of 21 ± 1 weeks. Effectiveness of therapy estimated at 11 patients because 1 patient was excluded after the first injection of Octofactor due to anaphylactic reaction. Seventeen episodes of bleeding at 6 patients were registered on the background of treatment with the help of Octofactor. Mean number of episodes of bleeding was 1.55 ± 2.12. Fourteen (82.4 %) hemorrhagic episodes of 17 were posttraumatic and 3 (17.6 %) were spontaneous. Mean number of spontaneous episodes (main criterion of effectiveness) was 0.27 ± 0.65, which confirms the low frequency of exacerbations of hemorrhagic syndrome on a background of prophylactic treatment by Octafactor. Among all registered hemorrhagic episodes, 10 (58.8 %) were mild, 5 (29.4 %) observations were intermediate and 2 (11.8 %) episodes were severe. Overwhelming majority of hemorrhagic episodes (15 or 88.2 %) were stopped after 1–2 infusions of Octafactor medication. Mean number of infusions of Octofactor medication for stopping of hemorrhagic episodes was 1.6 ± 0.86. It was needed mean 3808.8 ± 2022.4 МЕ of Octofactor medication to stop 1 episode of bleeding. Residual activity of FVIII after 48 hours after the Octofactor infusion was 2 % and more at overwhelming majority (63.6–90.9 %) of adolescents during the observation (3 visits). Common number of Octofactor medication infused for bleeding prevention was 1 500 500 МЕ, for bleeding stopping – 64 750 МЕ. Safety of therapy estimated at 12 patients. Nine adverse events (AE) were registered at 7 patients, clinical AE were predominant – 5 (55.6 %) cases. One patient had an anaphylactic reaction on the first infusion of Octofactor medication which was estimated as serious AE of mild severity. All other AE were not serious or mild and resolved without after-effects. Trombembolic complications and immunogenic reactions were not registered. Thus, received data demonstrate the effectiveness and safety of Octofactor medication both for prevention and stopping of bleeding at adolescents with severe hemophilia A.

Primary intracranial germ cell tumors are rare heterogeneous group of neoplasms presented several histological types with different biological profile and response to therapy. Pure germinomas make up no more than 5 % of malignant tumors of the central nervous system in children, bifocal germinomas account for only 5–10 % of all germinomas. Radiotherapy and chemotherapy are usually used in intracranial germ cell tumors. The choice of initial therapy options is dependent on the histological variants of tumors, and the prevalence of the process (M-stage). Vascular malformations of the brain is rare in childhood, in a large proportion of cases, which is a random finding and can significantly affect the treatment policy in the presence of a brain disease (eg, neoplasia).

We present a rare case of a combination of clinical bifocal germinomas and arteriovenous malformations of the brain in a boy 13 years old

Now the problem thrombocytopenia and thrombocytopathy and pathology of hemostasis in general is an important issue, which is an interesting topic, relevant for all children’s hematology and oncology. One of the leading trends in the study of this issue is Timon Hospital in Marseilles (France), of which the best practices presented in the article, the winner for the best presentation of clinical cases, held by the National Society of Pediatric Hematology and Oncology.

4S stage is unique and heterogeneous metastatic neuroblastoma - the most common extracranial solid tumor of childhood. Extremely important is the multidisciplinary approach to the management of this group of patients, from diagnosis to the development of a therapeutic plan and scheme of dynamic observation.