Relevance. The success of treatment of children with hepatoblastoma (HB) is associated primarily with the optimization of diagnostic and therapeutic strategies. The introduction of a system of therapeutic definition of a risk group developed by the International Society for the Study of Liver Tumors in Children’s Hepatic tumors International Collaboration (CHIC) in patients with HB allows for effective risk stratification. Patients of the very low-risk group have a favorable prognosis and, unlike patients of other groups, they can perform surgery at the 1st stage, and children from the very low-risk group with a “pure” fetal version of the morphological structure of the tumor can be cured without chemotherapy – only by surgical method. This article presents an analysis of a series of observations of children with HB very low-risk.

Methods and patients. A retrospective analysis of the data of 138 patients who received treatment at the Research Institute of Pediatric Oncology and Hematology of N.N. Blokhin National Medical Research Centre of Oncology of the Ministry of Health of Russia from January 2006 to February 2018, all patients underwent examination in the following volume: computed tomography (CT) of the chest, ultrasound of the abdominal cavity and retroperitoneal space, CT and/or magnetic resonance imaging of the abdominal cavity with contrast enhancement. Serum levels of human chorionic gonadotropin and alpha-fetoprotein (AFP) were measured. The prevalence of tumor lesions was retrospectively evaluated using the PRETEXT system (Pre-Treatment Extent of Disease, assessment of the prevalence of the tumor process before treatment, 2017 version). Based on the data obtained, 18 (13 %) patients with hypertension were identified in the very low-risk group using the CHIC stratification system. Of 18 (100 %) patients of the very low-risk group, 8 (44.4 %) received therapy in the interval from January 2006 to October 2010 according to the treatment protocols of the SIOPEL group. The treatment included a combination of neoadjuvant chemotherapy with delayed surgery and adjuvant chemotherapy. Since November 2010, the Research Institute of Pediatric Oncology and Hematology adopted a protocol for the treatment of children with hypertension, suggesting the possibility of performing surgical treatment at the 1 st stage in patients with a localized stage of the disease with the spread of PRETEXT I/II. From December 2010 to November 2018, 10 (55.6 %) patients from the very low-risk group were operated on at the 1 st stage. A tumor biopsy was not performed before the intervention. After a histological examination, 4 (22.2 %) patients had an epithelial variant of the structure of HB that did not meet the criteria of a “pure” fetal variant. He was given adjuvant chemotherapy according to the SIOPEL protocol. And in 6 (33.3 %) patients, according to the results of a morphological study, the HB structure variant was interpreted as a “pure” fetal variant. Further therapy in this group of patients was not performed. The analysis of the results of treatment of these 6 patients without the use of chemotherapy is presented in our article.

Results. The average age of patients is 3.7 (1–6) years, the median is 3.7 ± 2.0. Boys/girls – 4:2. In all cases, there was an asymptomatic course of the disease, a neoplasm was detected by chance during an ultrasound scan. All children had an elevated AFP level in the range of 95.2–695 (286.0 ± 116.8) IU/ml. Distribution according to PRETEXT I/II – 2 (33.3 %) and 4 (66.7 %) patients, respectively. Patients did not have additional PRETEXT criteria and metastases. Surgical treatment was performed by the laparoscopic method in 2 of 6 patients. Scope of operation: right-sided hemihepatectomy – 3, left-sided – 1, bisegmentectomy – 1, atypical resection – 1. Lymphatic dissection in the area of the hepatoduodenal ligament was not performed. There were no complications of treatment and repeated surgical interventions. The radicalness of the intervention in the volume of R0 was confirmed by histological examination in all patients. Morphological examination in all cases revealed a “pure” fetal version of the structure of the tumor. All patients are alive without signs of relapse at follow-up periods of 14.7 to 59.2 (32.0 ± 18.7) months.

Conclusion. Using the system of therapeutic stratification into risk groups CHIC allows you to effectively distinguish patients with very low-risk groups, and for some children with a “pure” fetal version of the histological structure of the tumor, only the surgical approach can be used for treatment. At the same time, the short observation period for the patients and their small number do not allow us to draw final conclusions and dictate the need for a multicenter study. It should be noted that this cohort of patients requires careful observance of the post-therapeutic observation algorithm.

Introduction. Treatment of children with mediastinal and lung tumors is an urgent problem in both surgery and anesthesiology. Main goal is to grant optimal conditions for the surgery – collapse of the lung on the side of the operation, while maintaining adequate oxygenation and efficient transport of oxygen to the tissues. The choice of devices for carrying out one-lung ventilation (OLV) is the most important factor determining the safety of the patient.

Aim of the study. To increase the efficiency and safety of anesthesia for thoracic surgeries in children due to the use of bronchial blockers (BB) for one-lung ventilation (OLV). Materials and methods. 92 surgeries were performed (49 – thoracoscopic, 43 – thoracotomies) in 11–17 years old 92 patients. Patients were randomly divided into two groups: BB group (n = 56) – OLV was performed using bronchial blocker; double-lumen tubes (DLT) group (n = 36) – OLV was performed using a DLT. Ventilation parameters, hemodynamics, the incidence of complications in the perioperative and postoperative periods were recorded.

Results. A much less prolonged lungs separation time in BB group was found compared to the DLT group (65.32 ± 17.32 sec versus 99.0 ± 9.03 sec respectively, p < 0.0001) and a lower frequency of repositioning (0.2 ± 0.4 and 0.57 ± 0.51 respectively, p = 0.04). In patients with BB, less complications were recorded, such as postoperative aphonia and sore throat.

Conclusion. BB demonstrate efficacy and safety in OLV management for thoracic surgeries in pediatric oncology. They provide faster lungs separation time than DLT, irrespective of the side of the surgery, allow complete collabation of the lung with a less frequent need for repositioning. The introduction of a new method of OLV with the use of BB in children is associated with reduction of the length of stay of patients in the intensive care unit during the postoperative period due to the reduction in complications such as trauma of the main bronchi, aphonia and sore throat.

Relevance. Hodgkin’s lymphoma (HL) is a rare clonal neoplasm of lymphatic tissue. In the structure of malignant neoplasms in children HL accounts for 4–6 % and for 16 % in adolescents. Now children and adolescents with confirmed histology of HL in the Department of Oncology and Hematology, Morozovskaya Children’s Clinical Hospital, treated with the GPOH-HD-2003 protocol. The authors of the article presented the experience of treatment children and adolescents with HL according to recommendations of GPOH-HD-2003 in the Department of Oncology and Hematology, Morozovskaya Children’s Clinical Hospital.

Patients and methods. From January 1, 2003 until December 31, 2018, 62 patients (35 boys, 27 girls) received therapy according to recommendations of GPOH-HD-2003 study. Age of patients ranges from 4.7 to 17.6 years, mean age was 13.3 years. All patients are allocated to three treatment groups (TGs) based on staging, in TG-1 were 2 patients, in TG-2 – 30 patients, in TG-3 – 30 patients. TG-1 patients received two courses of for induction: OEPA for boys and OPPA for girls. TG-2 and TG-3 patients received further two or four cycles COPP (girls) or COPDAC (boys), respectively. After chemotherapy all patients received initially involved-field irradiation with 19.8 Gy.

Results. CTCEA grade 1 or 2 hematotoxicity was the most common recorded adverse reaction in the OEPA regimen for boys, grade 3 or 4 hematotoxicity – in the OPPA regimen for girls. Also hematotoxicity was more pronounced with COPP than COPDAC. Overall (OS), event-free (EFS) and relapse-free (RFS) survival rates at 5 years were 100 % for the period of the observation time (the median observation time was 48.12 months). No treatment-related deaths, relapses or second malignancies were recorded during this period.

Conclusion. OS, EFS and DFS rates indicates high efficiency and low toxicity of GPOH-HD-2003 study regardless of the initial stage.

Atypical teratoid rhabdoid tumors (ATRT) are the most common malignant embryonal tumors of central nervous system in young children. Metastatic stage reveals in 20–30 % cases at the diagnosis. ATRT is a primarily monogenic disease characterized by the biallelic mutation of the SMARCB1, more rarely SMARCA4 genes. The survival rate of ATRT’s patients is poor. Multimodal treatment approaches including surgery, conventional dose chemotherapy with intrathecal therapy, high-dose chemotherapy with autologous stem cell resсue and radiotherapy have shown significant potential for improving outcomes. Recent epigenetic and transcriptional studies conducted by two independent research groups have identified three subgroups of ATRT. It has different molecular characteristics with appropriate therapeutic sensitivity. Further study of molecular types, inclusion of biologically targeted agents may be a promising strategy for optimizing treatment in future studies.

Choroid plexus carcinoma (CPC) is a rare malignant tumor arising from the epithelium of the choroid plexus of the brain. More than 80 % of CPCs occur in children. Mutations in the TP53 gene is played the main role in the pathogenesis of these tumors. Choroid plexus carcinomas in 40 % of cases are associated with Li–Fraumeni syndrome. Survival rates in patients with CPC and Li–Fraumeni syndrome are extremely low. The standards of the therapy for patients with CPC are not defined. The extent of surgical resection and treatment modality correlate with prognosis. The role of adjuvant therapy in CPC remains unclear: doses and volumes of radiation therapy (RT), combinations of chemotherapeutic drugs, timing, and a combination of RT and chemotherapy (CT) have not been identified. Also, there is neither a standard CT regimen nor a prospective international study assessing the efficacy and toxicity of various combinations of cytostatics in patients with CPC. The article presents an overview of the existing molecular genetic changes, existing methods for the diagnosis and treatment of choroid plexus carcinoma.

Liver resection remains one of the most important option in combined treatment for liver tumors; however, it harbors the risk of developing posthepatectomy liver failure. The principal risk is associated with the quality and quantity of the future remnant liver. Therefore, preoperative assessment of the future remnant liver is essential in patients scheduled for major hepatectomy. Technetium-^99m-mebrofenin hepatobiliary scintigraphy is increasingly applied for the quantitative assessment of liver function before major liver resection.

One of rare location of teratoma in children is head and neck region. Treatment options, including surgery, are determined by tumour location, its grade of differentiation, and patients age. This article presents case report of immature paratracheal neck teratoma in a 3-month old child, subjected for second-look surgery for residual tumour removal due to the risk of life-threatening complications.

Infantile fibrosarcoma (IF) is a rare soft tissue sarcoma that occurs with a frequency of 1% of all cases of malignant neoplasms in children in the first year of life. IF most often affects the soft tissues of the upper and lower extremities. Rare localizations include the trunk, the head and neck, and the gastrointestinal tract. The article describes a rare clinical case of congenital IF of soft tissues of the chest wall in a newborn, as well as a literature review.

This article is devoted to the analysis of problems associated with the state of the pulmonary system in patients who survived a malignant neoplasm in childhood. The main diseases and pathological conditions that can develop in this population are considered. The risks of pathological changes in the pulmonary system, treatment and prevention are described. The problems that may occur after treatment with bleomycin, including pneumonitis, fibrosis, acute respiratory failure syndrome, are analyzed separately.

The authors of the translation: G.M. Muftakhova, E.A. Tikhomirova, V.A. Grigorenko, O.A. Kapkova.