Malignant neoplasms are the second most common cause of infant mortality worldwide. Nutritional deficiency occurs in approximately 60 % of children with cancer and contributes to a decrease in immune function, delayed wound healing, impaired drug metabolism, and a severe and recurrent course of infectious complications. Malnutrition has a particularly adverse effect on outcomes and is one of the main preventable causes of death in infants. Given the growing trend in the incidence of cancer in children of the first years of life, the lack of standards and recommendations for the therapeutic nutrition of these patients, the organization of nutritional support for infants in the context of pediatric oncology is becoming increasingly important. Our article presents current trends in the assessment and correction of the nutritional status of infants with cancer.

Обоснование . Взаимосвязь развития ряда злокачественных опухолей у детей с вирусами хорошо известна. Хорошо изучена этиологическая роль вируса Эпштейна–Барр в развитии недифференцированного рака носоглотки (НРН). Влияние вируса папилломы человека (ВПЧ) на возникновение и течение НРН у детей изучено недостаточно. Цель . Описание клинического случая инфицирования вирусом Эпштейна–Барр и ВПЧ пациента 16 лет с последовательным развитием НРН и папилломатоза ротоглотки. Описание клинического случая . Клиническое наблюдение мальчика, у которого выявлен НРН. После проведения химиолучевой терапии отмечен полный клинический эффект в виде сокращения первичной опухоли и редукции метастазов в лимфатических узлах шеи. Через 18 мес после излечения по данным позитронной эмиссионной томографии, совмещенной с компьютерной томографией, с 18F-дезоксиглюкозой отмечено накопление радиофармпрепарата в области небных и язычной миндалин слева. При осмотре выявлены клинические признаки папилломатоза мягкого неба. Проведена плоскостная резекция мягкого неба. Морфологическое исследование подтвердило плоскоклеточную папиллому. В материале папилломы обнаружена ДНК ВПЧ 16-го типа. Заключение . Накопление информации о поражениях глотки в детском возрасте, обусловленных ВПЧ, позволят расширить представления о наиболее распространенных типах этого вируса в детской популяции. На основании обнаружения высококанцерогенных типов ВПЧ возможно выделение группы риска по развитию злокачественных опухолей.

Background . Therapeutic approach to the treatment of localized parameningeal sarcoma in children is based on both chemoradiotherapy and surgery. In modern treatment protocols, there are no clear criteria for transnasal endoscopic surgery of tumors localized in the parameningeal region in children. Objective. The aim is to report the experience of endoscopic transnasal surgery as a part of complex treatment of localized parameningeal sarcomas in children. Materials and methods . Fifteen transnasal endoscopic surgeries were performed in 12 patients at N.N. Blokhin Institute of Pediatric Oncology and Hematology during 2012–2017. The mean age of patients at the time of surgery was 9.5 years. The morphological diagnosis of rhabdomyosarcoma (RMS) was confirmed in 10 (83.3%) patients, osteosarcoma and hemangiopericytoma — in 1 (8.3%) case each. The transnasal endoscopic biopsy was performed in 7 (58.3%) cases. Transnasal endoscopic removal of the tumor was performed in 8 (66.7%) patients, 3 (25.0%) of them underwent a transnasal endoscopic biopsy previously. The main complication was liquorrhea registered in 4 (33.3%) patients with RMS. Results . At the time of analysis 6 (50.0%) patients have completed full course of treatment, 6 (50%) continue the therapy. The observation period after treatment was 5 up to 22 months. 4 patients are still alive without evidence of disease, and 2 died of tumor progression. Conclusion . Transnasal endoscopic removal of the tumor can be considered as a part of complex treatment in pediatric patients by modern therapeutic protocols.

The Russian clinical practice guidelines for diagnosis and treatment of differentiated thyroid cancer is dedicated to the management of patients with differentiated thyroid cancer. The guideline modifications 2016 include the following matters: indication for fine-needle aspiration biopsy, calcitonin screening, standards for biopsy results, new positions of postoperative risk stratification, indication for suppressive therapy and thyroid replacement therapy, targeted therapy in patients with radioiodine-refractory differentiated thyroid cancer.

Introduction . Improvement of the quality of medical care (QMC) is the backbone of any health care system. It is necessary to conduct an external audit of medical institutions to identify the currently existing quality defects. Materials and methods . We evaluate the available equipment and existing competence of the staff of the Department of Oncology at one of the Regional Children’s Clinical Hospital in accordance with the requirements of the Annexes 5-6 to the Order of Ministry of Health of the Russian Federation № 560n dated October 31, 2012 (as amended September 02, 2013 № 608n) “On approval of procedures for the provision of healthcare services in the profile ‘Pediatric oncology’”. The analysis of the case histories of patients with solid tumors (except brain tumors) treated in the period of 2011-2015 was also performed. Results . Two hematologists work at the department at the moment. The pediatric oncologist is a hospital consultant and is not included in the staff. 40 positions of the equipment are required for the department functioning. Only 25 (62.5%) items are available, 6 (15%) positions do not exist, and 9 (22.5%) are presented in insufficient quantities. Out of 22 patients with solid tumors (11 girls and 11 boys, mean age — 4.8 years (3 months — 14 years)) who received specialized and symptomatic treatment in 2011-2015, six (27.3%) had sarcomas of bones and soft tissue, nine (40.9%) had neuroblastomas, five (22.7%) had nephroblastomas, and two (9.1%) had rhabdomyosarcomas. Eleven (50%) patients underwent chemotherapy, 3 (13,6%) received symptomatic treatment, 4 (18?2%) were provided with palliative care, and four (18.2%) patients were hospitalized for a control examination. Conclusion . Elaboration of recommendations is required. These recommendations should provide rationalization for feasibility of certain treatment stages (chemotherapy, surgery, radiotherapy) in patients from different regions of the Russian Federation with regard to their remoteness from the federal centers, density of pediatric population, and the annual number of primary patients.

Despite the fact that the importance of venous access is becoming more evident in Russia, few clinics and specialists have the necessary skills to create long-term access to the venous system. CVC even with a subclavian catheter is often performed by physicians in violation of technology that increases the number of complications. Implantation of venous port systems, unfortunately, is still the exception to the rule in the treatment of cancer in children, while abroad has long been a standard. Article covers the highlights of the art installation subclavian venous catheters and port systems.

Russian and foreign literature presents insufficient data about the bone marrow immune system of children under normal and pathological conditions (acute leukemia (AL) and solid tumors). The aim of the research was to study the quantitative characteristics of bone marrow lymphocytes subpopulations in children with developing acute leukemia and small round cell sarcomas (rhabdomyosarcoma (RMS) and Ewing's sarcoma family tumors (ESFT). Materials and methods. The study included 111 children aged 9 months to 17 years (mean age – 7.6±0.4 years). Rhabdomyosarcoma was diagnosed in 16 children, Ewing's sarcoma (CIO) ― in 16 children, acute lymphoblastic leukemia (ALL) ― in 38 children, and acute myeloid leukemia (AML) ― in 26 children. Malignancy was excluded basing on complex assessment in 15 cases (experimental group). All patients underwent cytological and immunological examination of bone marrow. Results. Typical change in the bone marrow subpopulations of patients with both types of sarcomas was the increase in the percentage of CD3+HLADR+ and CD8+HLADR+ T-cells and the decrease in the percentage of Leu8+CD4+ T cells versus the control group data (p 0.05). Comparison of bone marrow lymphocytes subpopulations in ALL and AML patients demonstrated the significant differences only in the percentage of CD19+ and CD19+CD5+ B cells, the value was significantly lower in AML patients (13.4%±1.8% vs 19.5±2.1, p=0.033 and 2.2%±0.5% vs 4.7±0.8, p=0.016, respectively). The percentage of CTLs was proportionately lower under AL than sarcomas, the maximum relative content of CD8 + T cells was revealed in the bone of RMS patients. The T-helper cells content in all four treatment groups did not significantly differ in relative and absolute values (p>0.05). Conclusions . Comprehensive morphological and immunological examination results demonstrated that these cancers are characterized by specific changes in subpopulation content of T and B bone marrow lymphocytes that revealed their clinical and prognostic value, the mechanisms of interaction between the tumor and the immune system, and may be useful in the development of immunotherapy programs.

Introduction. A shortage of qualified staff and equipment in some clinics of the Russian Federation limits the possibility for wide application of long-term venous access system. Thereof it is acceptable to use the peripherally inserted central catheters (PICC) in these patients though the duration of treatment exceeds one year in the majority of cases. Materials and methods. 10 patients aged 8 to 17 years (mean age 11.2 years) with a variety of cancers were treated at the Institute of Pediatric Oncology and Hematology N.N. Blokhin from March to June 2016 and underwent the installation of Bard Groshong 3 F (4 F) Single-Lumen PICC (USA). Results. All the inserted catheters continue functioning. Only one case of the catheter thrombosis was detected and successfully eliminated by introducing a solution of Urokinase 500 ME/ml (3 ml) with the 15 min exposure. Catheter-related bloodstream infections were not observed through the analyzed period. Conclusion. The obtained findings, the experience of foreign clinics, simplicity of insertion that does not require intraoperative fluoroscopy or general anesthesia, and positive feedback from patients contribute to the further implementation of PICC lines in clinical practice.

The incidence of neuroblastoma is 7–8% in the structure of oncological morbidity and is mostly found in young children. Among the extracranial neuroblastoma arising in paravertebral sympathetic ganglia, intraparavertebral neuroblastoma is detected up to 15% of cases and causes difficulty in the differential diagnosis of tumors of different morphological patterns. Radiological methods are leading in the detection of tumors, but the similarity of their clinical and radiological features impede early diagnostics. The widespread introduction into clinical practice of x-ray computed tomography and MRI have significantly improved the quality and the quantity of diagnostic information. However, the effectiveness of radiology for diagnostics of tumors of different intraparavertebral localization remains insufficient. Our research had the objective to improve the efficacy of different clinical and radiological diagnostic techniques of intraparavertebral neuroblastoma in children. This article contains the algorithm of children examination for intraparavertebral neuroblastoma detection depending on age in accordance with the objectives of radiodiagnosis. ( For citation: Kirillova O.A., Volkova L.D., Mikhailova E.V., Zakharova E.V., Polyakov V.G., Kazantsev A.P. Clinical Features and Radiology of Intraparavertebral Neuroblastoma in Children. Onkopediatria. 2015; 2 (2): 121–130. Doi: 10.15690/onco.v2i2.1343)

Development of pediatric oncology, formation and spread of specialized cancer care for children across the country is impossible without educational training of specialists ― pediatric oncologists. Academic Department of Pediatric Oncology realize postgraduate specialized training in the Russian Federation. The paper presents the History of the of Pediatric Oncology Academic Department, its capabilities, goals, objectives and future prospects.

The article describes a unique clinical case of multifocal synchronous osteosarcoma patient 10 years old with the defeat of the right femur and humerus identified in a clinic in Israel in 2014. The patient was conducted a combined treatment including arthroplasty. At the time of this writing, the patient is alive without evidence of disease manifestations and can independently move without crutches.

Public health management is an important sector of clinical medicine providing both a legislative framework and further course of development within the limits of available resources. This is particularly topical in regard to pediatric oncology – one of the most complex and costly branches of medicine. Achieving the best results in the treatment of children with cancer requires cooperation of various medical specialists, treatment at different levels and conduction of multicenter studies. This article reviews the available statistical data and analyses the key regulations governing medical care for children with cancer.

Introduction . The article presents the five-year experience of implantation and maintenance of venous access systems in children with cancer, and provides the clinical and economic analysis of this type of manipulation. Basing on the sales statistics and the results of the training of medical personnel the analysis of the availability of implantable venous port systems in the clinics of the country was conducted. Materials and methods. The analysis included data on 2,262 patients aged from 2 months up to 17 years, who had been taking a course of chemotherapy in 2010-2014 and were implanted different venous access systems: 2099 children (92.8%) — 3930 external central venous catheters and 163 (7.2%) — 163 implantable venous port systems. Results . Complications in patients with external central venous catheter occurred in 11.8% of cases, technical difficulties — in 72.9%, 55.8% of which were corrected intraoperatively. Maintenance complications were noted in 61.7% of cases, 44% of which required removal of the outer central venous catheters. Complications in patients with implanted port systems occurred in 4.3% of cases, technical difficulties — in 29.4% but all the complications were corrected intraoperatively. Maintenance complications were noted in 15.9% of patients. They were caused by improper operation of the port systems. It resulted in the removal of 2.4% port systems. Conclusion . Maintenance of implantable venous port systems carries the lowest risk of complications, and is more economical. One of the few deterrents for their mass adoption is the lack of a sufficient number of qualified medical personnel able to deal with the implantation and maintenance of long-term venous access. Another reason — the lack of required radiological and ultrasound equipment in clinics.

Aim. To determine current rates of childhood cancer incidence and mortality at a national level for Russia and to evaluate recent trends. Materials and Methods. Using the Annual reports of Ministry of health and Federal State Statistics Service we calculated childhood cancer incidence and mortality rates for the 5-year period 2008–2012 and trends between 1989 and 2012 by sex, age and site. Rates were directly age-standardised to the 2000 World Standard Population, and linear regression was used to determine the magnitude and significance of trends. Results. The age-adjusted incidence rate in children aged 0–17 years was 125 per 1,000,000 individuals per year for 2008–2012. The highest age-specific incidence (159 per 1 000 000 children/year) was observed in early childhood (0–4 years). Between 1989 and 2012, a significant increase in the cancer incidence was observed in children aged 0–14 years: average annual percent change was 1,6% [95%CI 1,5; 1,7]. The greatest increase for this period was observed for soft tissue sarcomas (3,7% [2,6; 4,9]), hepatic tumors (3,6% [2,6; 4,6]), thyroid carcinomas (3,7% [3,2; 4,2]), CNS neoplasms (2,9% [2,6; 3,1]), renal tumors (2,1% [1,7; 2,5]) и leukaemias (1,9% [1,7; 2,1]). The decrease of incidence was observed for Hodgkin (- 1,6% [-2,1; -1,2]) and non-Hodgkin (- 1,4% [-1,8; -1,0]) lymphomas. Childhood cancer mortality for 2008–2012 was 40 cases per 1 000 000 children/year. The highest age-specific mortality rate (52,3 per 1 000 000) was observed in infants. The significant decrease of mortality were found from 1989 (70 per million) to 2012 (37 per million). The greatest average annual decrease for this period was observed for leukaemias (-3,8% [-4,0; -3,5]) and lymphomas (-6,8% [-7,3; -6,4]). The significant decrease of mortality in 1999–2012 was found for malignant bone tumors(-5,9% [-7,4; -4,3]), renal tumors (-2,9% [-4,7; -1,1]) and CNS neoplasms (-1,1% [-1,9; -0,3]) with the only exeption for soft tissue sarcomas (average annual increase was 3,2% [1,8; 4,7]). Conclusions. significant temporal trends of childhood cancer incidence and mortality in Russia were found during the study period. Although rates of cancer mortality are generally decreasing in Russia there are still very high levels for common childhood cancer types.

Introduction. The treatment of any oncologic disease is impossible without a venous access. What kind of properties should it possess? It has to be safe, easy to use, implanted only once during the treatment course and have minimal risks associated with implantation and use. Purpose. Prevention of complications of intravenous chemotherapeutic agent administration. Materials and Methods: From 2010 to 2013 we were monitoring the treatment of 228 children (aged 3 months to 17 years) with oncologic diseases. 110 patients underwent 605 subclavian vein catheterization, 118 patients — 118 venous port implantation. Results. Complications and technical difficulties during catheter insertion were observed in 98,3% of cases, during venous port implantation — in 23% of cases. Complications of subclavian catheter and venous port use were observed in 97,3% and in only 11% of cases, respectively. Subclavian catheters compromised cancer treatment in 45,9% of patients, implantable venous ports — in 1,7% of patients. Each patient with a subclavian catheter underwent central venous catheterization 4 to 19 times (mean 6 times) during treatment. Catheter dwell time exceeded the recommended limit in all patients except for cases of catheter removal by patients. On multiple occasions all patients were discharged with a subclavian catheter in place. Conclusion. Venous ports obviously match the criteria mentioned in the introduction. Subclavian catheter use resulted in cancer treatment protocol deviation in almost 50% of cases, thus leading to a poorer prognosis and significantly increasing the number of invasive procedures and instances where general anesthesia was needed.