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The article presents the features of the clinical manifestation of multiple primary sporadic and heritable malignant tumors in 104 children who observed at Pediatric Oncology and Hematology Research Institute of «NN Blokhin's National Medical Research Center of Oncology» from 1998 to 2012. The age of patients at the time of occurrence of the second malignant tumor ranged from 2.8 to 28 years and averaged 15.6 years. The second neoplasia occurred significantly more frequently in the group of primary hemoblastosis, compared with the group of solid tumors - 8.7% versus 3.4%, respectively (p <0.0001). In children with multiple primaries, were found tumors that occur in common the most frequency. Retinoblastoma patients have an increased risk of developing sarcoma. Patients with Hodgkin's lymphoma have an increased risk of developing leukemia. In surviving children after treatment of the first neoplasia the thyroid, bone tissue and breast have a specific risk for the development of metachronous cancer and are target organs for control. Children with new hematopoietic tumors may be candidates for metachronous development of leukemia and bone tissue tumors. The using methods Next Generation Sequencing (NGS) and Multiplex Ligation-dependent Probe Amplification (MLPA) revealed germinal mutations in 12 children with of multiple primary tumors. The mutations in the TP53, RB1, CHEK2, FANCN/PALB2, MLH1, PMS2 genes identified in patients were associated with hereditary syndromes and an increased risk of developing second tumors, among which: sarcomas, brain tumors, hematopoietic tumors were the most frequent. It was shown that the second tumors can appear at the any age. Children who survived the treatment of the first tumor in later life should be monitored annually. Clinical management of children with multiple primary tumors requires a multidisciplinary approach.

Около 20-25 % всех рабдомиосарком у детей располагается в урогенитальной зоне. Благодаря внедрению новых протоколов лекарственного лечения, применению лучевой терапии бо льшая часть пациентов вылечиваются консервативными методами. Хирургическому лечению подвергаются только около 20 % пациентов, значительному числу из них выполняются органосохраняющие операции. В тех случаях, когда опухоль не поддается консервативному лечению, имеется остаточная опухоль или развиваются рецидивы заболевания, выполняются радикальные органоуносящие операции. Среди методов деривации мочи после цистэктомии преимущество отдается вариантам, позволяющим пациентам осуществлять произвольное выведение мочи: формированию соединенных с уретрой ортотопических или открывающихся на кожу гетеротопических резервуаров, конструируемых из различных отделов кишечника. Представлен клинический случай позднего послеоперационного осложнения -образование конкрементов в резервуаре через 7 лет после цистпростатэктомии с формированием сухой стомы у пациента, прооперированного по поводу урогенитальной рабдомио саркомы.

Композитная феохромоцитома (КФ) -редкое злокачественное новообразование, встречающееся в различном возрастном диапазоне. Основным методом лечения феохромоцитомы является полное хирургическое удаление опухоли, тогда как в случае с нейробластомой используется терапия в зависимости от группы риска и могут применяться полихимиотерапия, аутологичная трансплантация костного мозга, лучевая терапия, иммунотерапия и терапия 131 I-метайодбензилгуанидином. В связи с редкостью КФ стандартных подходов к системной терапии нет. Выбор тактики и объем лечения определяются по наиболее неблагоприятному компоненту опухоли. В данной статье представлен редкий случай КФ с низкодифференцированной нейробластомой левого надпочечника у ребенка 4 лет. Родители пациента дали согласие на использование информации, в том числе фотографий ребенка, в научных исследованиях и публикациях.

Relevance. Autologous hematopoietic stem cell transplantation (auto-HSCT) is an integral part of the treatment of patients with high-risk malignancies. However, carrying out the mobilization and collection of hematopoietic stem cells (HSC) for patients weighing up to 15 kg is a complex task that requires a special approach and multidisciplinary interaction.The purpose of the study is to present the experience of collecting HSC in young children weighing up to 15 kg at the Research Institute of Pediatric Oncology and Hematology of the Federal State Budgetary Institution N.N. Blokhin National Medical Research Centre of Oncology, Ministry of Health of Russia.Materials and methods. The study included 30 patients weighing up to 15 kg who received treatment from January 2020 to May 2021 at the Research Institute of Pediatric Oncology and Hematology of the Federal State Budgetary Institution N.N. Blokhin National Medical Research Centre of Oncology, Ministry of Health of Russia. Median age was 30.6 (12–48) months, median body weight was 12.2 (7.8–15) kg. Apheresis in children weighing up to 15 kg was performed in the resuscitation and intensive care unit with the condition of pre-filling the cell separator circuit with a donor irradiated erythrocyte suspension to prevent hypovolemic complications. Thirty two apheresis was performed on a cell separator of the “Spectra Optia” type. To mobilize HSC, preparations of granulocyte colony-stimulating factor with the main active ingredient filgrastim were used.Results. Twenty eight HSC apheresis procedures were successful on the first attempt, 2 patients underwent repeated apheresis (a total of 32 procedures were performed). The median number of CD34+ cells obtained was 13.9 × 106/kg (0.04–92.0 × 106/kg), and the median apheresis duration was 251 (160–415) min. In 2 children during the procedure, an immediate organization of repeated venous access was required.Conclusions. Performing HSC apheresis in children weighing up to 15 kg and young children is a safe and effective technique with the participation of a multidisciplinary team. The apheresis algorithm proposed by us makes it possible to carry out a high-quality collection of CD34+ cells, sufficient for auto-HSCT.