The basis for constructing a vertically integrated management system in the healthcare sector in the Russian Federation is the model of functioning of national centers. One of the key components in achieving success is the interaction of the national center with a leading national society. The aim of the article is to present the experience of joint work of the National Society of Pediatric Hematologists and Oncologists (NSPHO) with the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology in the field of development of pediatric oncology-hematology in the Russian Federation. In order to accomplish the task of building a system for organizing medical care for children in pediatric oncology-hematology, a comprehensive development program was implemented, which included collecting data on the structure of the service through questionnaires in the regions, obtaining expert opinion and the subsequent creation of a service passport using data visualization. In addition, the experience of creating professional standards and the concept of a unified approach in the field of training in the specialties “Pediatric Oncology” and “Hematology” using the technology of continuing medical education are reflected. The result of the work was the formation of a single plan for the organization of the service of pediatric oncology-hematology, developed by the NSPHO in conjunction with the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology with the support of the National Medical House and under the leadership of the Ministry of Health of Russia.

Introduction. Stratification of patients with germ cell tumors is primary carried out depends on stage and previous operation, at the same time is not considered the tumor markers decline.
The aim of the study was the nomogram creation for diagnostics of the prolonged alfa-fetoprotein (AFP) half-life (HL) in patients with germ cell tumors in order to identify of a patients cohort with chemoresistant malignancy and to find treatment modification with transition to the escalated and intensified modality therapy.
Materials and methods. Seventy-two patients less than 18 years old with extracranial germ cell tumors were treated in N.N. Petron National Medical Research Centre of Oncology between 1996 and 2017, among them with increased AFP were 46 (63.9 %) children. AFP half-life periods were calculated.
Results. AFP half-life varied from 3 to 138 days (the average value was 15.6 days). A cut-off point at 6 days was found. An unfavourable decline in AFP was predictive overall survival (OS). OS rates were 85.2 ± 7.9 % and 50.1 ± 12 % in patients with HL £ 6 days, and with > 6 days, respectively (p = 0.01873). Nomogram for diagnostics of the prolonged AFP HL in patients with childhood extracranial germ cell tumors was built. Sensitivity, specificity and diagnostic accuracy of nomogram were 92.9 %, 63.6 % and 72.3 %, respectively. False negative result was in one case (2.1 %).
Conclusions. Secondary stratification of patients has to be carried out after induction treatment with use of the principles of “response-based” of therapy. The nomogram allowing revealing the cohort of patients with prolongation of the period of AFP HL demanding early escalation and an intensification of medical programs helps with such cases for diagnostics of reduction in the rate of disintegration of AFP.

Currently, two staging systems are used: the International Neuroblastoma Staging System (INSS), which relies on surgical staging, and the International Neroblastoma Risk Group Stading System (INRGSS), which relies on preoperative imaging for staging and image-defined risk. The article presents a review of the literature on image defined risk factors (IDRF) determined during radiological studies (computed tomography and magnetic resonance imaging) in patients with neuroblastoma. The article focuses on the definition of IDRFs, their prevalence and their influence on surgical outcomes and prognosis of patients with neuroblastoma.

Melanotic neuroectodermal tumor of infancy (MNTI) is a rare type of tumor with uncertain clinical behavior. The tumor commonly occurred in the head and neck region. Radical surgical resection is the standard of treatment, which is often associated with high risks of severe postoperative complications due to tumor localization. Alternative methods of treatment including chemotherapy/radiotherapy are used in metastatic, locally advanced and relapsed cases of MNTI. The aim of this study was to analyze clinical and morphological characteristics of the tumor, evaluate the effectiveness of various treatment options, and to describe two clinical cases of MNTI.

Introduction. Frequent bleeding with hemophilia significantly worsens the quality of life of patients. The pathogenesis of hemorrhage in hemophilia has not been studied enough, especially at the vascular level, so it is necessary to study microcirculation in this disease.
The purpose of the study is to assess the mechanisms of regulation of blood tissue perfusion and the adaptive reserves of the microcirculation system in patients with hemophilia.
Materials and methods. Total microcirculation was assessed by laser Doppler flowmetry in 44 patients with hemophilia A between the ages of 14 and 20 years. Severe form of the disease was in 59 % of patients, the average – in 32 %, light – in 9 % of patients. The control group included 26 healthy men aged 14 to 19 years. The sensors recorded blood flow in the index fingers on both sides. In 20 patients with hemophilia А, an occlusion test was performed.
The results of the study. In patients with hemophilia, asymmetric changes in microcirculation parameters were detected when measured in the area of the index fingers. At rest in patients with hemophilia, the prevalence of vasospasm was revealed: a decrease in the perfusion index M, an increased blood bypass due to the predominance of myogenic tone. However, neurogenic tone indicators tended to decrease. During occlusive ischemia, vasospasm is slowed down in the first seconds after the onset of exposure to the stress factor.
Conclusion. The study revealed a dysregulation of the vascular tone of the microvasculature in young hemophilia patients at rest and under the influence of a stress factor in the form of short-term ischemia. Therefore, with hemophilia from a young age, control of microcirculation is necessary for the timely prevention of both bleeding and cardiovascular pathology associated with vasospasm.

The issues concerning quality of life are becoming increasingly important with the improvement of survival rates in children with cancer. The possibility of minimizing the toxicity of antitumor therapy for various organs and systems without reducing the effectiveness of specific therapy, as well as the development of preventive measures, is now an important task of medical specialists. Gonadal toxicity is a frequent adverse event that negatively affects both the somatic and psychological and emotional state of the cured patients. The only prospect of reproductive function preserving in a cohort of prepubertal girls with a high risk of premature ovarian failure is the ovarian tissue cryopreservation.
The main goal of this publication is to emphasize the importance of the reproductive health problem of girls with oncological diseases, with a description of the current data of international literature on the prospects of ovarian tissue cryopreservation in order to preserve fertility. Another goal is to present a multidisciplinary strategy for the management of prepubertal age patients with oncological disease within the framework of the Oncological Fertility Project at Almazov National Medical Research Center.
Based on the data of Russian and international literature, as well as existing guidelines and recommendations on reproductive health, a single algorithm for selecting patients has been developed, considering the expected gonadal toxicity for the use of the ovarian cryopreservation method in prepubertal girls. Carrying out of all the steps is possible in the optimal terms of antitumor therapy in the conditions of one hospital with observance of the stages of medical aid provision. The developed algorithm will allow to identify patients of prepubertal age, requiring the use of modern possibilities of reproductive technologies. In long-term date we are planning to evaluate the effectiveness of the orthotopic reimplantation technique for cryopreserved ovarian tissue in order to realize the reproductive function. A multidisciplinary team of specialists and the possibilities of the federal center fully allow to realize the Oncofertility Program in daily practice for girls and young women who needs to undertake gonadotoxic treatment.

Invasive mycoses caused by fungi of the genus Aspergillus, today remain an urgent clinical problem in oncological patients. In 10–20 % of patients with primary by aspergilloma can be detected in the Central nervous system. The article describes a case of neuroaspergillosis in an immunocompromised patient with choroid carcinoma of the right parietal-occipital lobe of the brain. Correct interpretation of risk factors, clinical symptoms and imaging techniques, early diagnosis with verification the pathological process, the use of fungistatic and fungicidal drugs allowed to avoid the negative consequences of fungal neuroinfection.

Over the past two decades, mucormycosis has firmly taken 3^rd place among invasive mycoses after candidiasis and aspergillosis in patients with oncohematological diseases. The article presents a clinical case of disseminated mucormycosis that developed in a child with nonHodgkin’s lymphoma on the background of deep immunosuppression.

Presented a clinical observation of hemolytic disease of the newborn as a result of fetus immunization with low-frequency erythrocyte antigen (Lutheran). The special aspect of the case is the presence of hyperleukocytosis with evident regenerative shift of neutrophils to the left to singular blastic variants, thrombocytopenia, high reticulocytosis (more than 1000 ‰) and a great number of nucleated red blood cells (normoblasts) in the newborn’s hemogram. In myelogram a sharp expansion of erythroid sprout (90 %) and signs of dizerythropoiesis were determinded. Typical clinical signs and isoserological incompatibility of mother and fetus in erythrocyte antigen provided basis for early diagnostics and adequate treatment of hemolytic disease of the newborn.

This article is devoted to the analysis of problems associated with the state of the musculoskeletal system in patients who survived malignant neoplasms in childhood. The main diseases and pathological conditions that can develop in this population are considered. The risks of pathological changes in the musculoskeletal system, treatment and prevention are described. The issues of long-term consequences after limb amputation, organ-preserving operations, osteonecrosis, scoliosis, osteoporosis are separately analyzed.

Focal nodular hyperplasia (FNH) is a regenerative volumetric liver formation that can occur as a result of the reaction of hepatocytes to local vascular circulatory disorders and is one of the late complications in patients who received high-dose chemotherapy followed by hematopoietic stem cell transplantation. This article presents a description of two clinical cases of development of liver FNH in patients with high-risk neuroblastoma identified in the late stages from the end of specific treatment. A possible reason for the development of liver FNH in these patients could be the use of alkylating drugs (ifosfamide, threosulfan, busulfan, melphalan).