Radical tumor removal remains the key task in hepatoblastoma surgery. Patients who need extended resection and have a risk of posthepatectomy liver failure are particularly difficult. In cases with insufficient future liver volume, the method of choice is a two-staged hepatectomy or liver transplantation. Despite the wide spread of laparoscopic liver surgery in adults, the minimally invasive approach has not been studied in pediatrics, and the literature on this issue is scarce. In current publication a case of successful partial associating liver partition with portal vein ligation for staged hepatectomy (ALPPS) in a child, firstly accomplished pure laparoscopically. Described experience suggests the feasibility of laparoscopic liver resections in children and effectiveness of partial in situ liver splitting to reach appropriate future liver remnant volume and to avoid liver transplantation and its disadvantages.

Gaucher disease (GD), code E75.2, is the most common form of hereditary enzymopathies united in the group of lysosomal diseases with autosomal recessive type of inheritance. The occurrence of GD is caused by a mutation of the gene responsible for the synthesis of the enzyme в-glucocerebrosidase (acid в-glucosidase), which is involved in the hydrolytic cleavage of glucosylceramide (a component of cell membranes) into glucose and ceramide. Insufficient activity (or insufficient amount) of в-glucocerebrosidase leads to the accumulation of glucosylceramide inside the cells of the reticuloendothelial system, which are thus converted into Gaucher cells. Clinically, this is expressed by a polysystemic disease with progressive hepatosplenomegaly, hematological disorders and lesions of the bones of the skeleton, which leads to severe pain syndrome, increased bone fragility and as a consequence — to severe disability. The “gold standard” of GD treatment is currently enzyme replacement therapy. To increase the availability of drug therapy and optimize approaches to the treatment of children with GD the search and development of new drugs is carried out.

Congenital mesoblastic nephroma (CMN) is a rare primary kidney tumor in young children, characterized by intermediate biological behavior. The main treatment method is the radical removal of the tumor. The purpose of this study is to analyze our own experience in the treatment of patients with a diagnosis of CMN. Parents of patients agreed to the use of information about them, including photographs, in scientific research and publications.

Introduction. Despite great achievements in treatment of neuroblastoma (NB) for the last decades, the outcome for high-risk patients remains adverse: 5-year overall survival does not exceed 30 %.

The purpose of the study. Demonstration of anti-GD2 monoclonal antibodies immunotherapy and assessment of its toxicity in patients treated at the N.N. Petrov National Medical Research Center of Oncology.

Material and methods. Anti-GD2 antibodies have been used as post consolidation in 8 high-risk NB patients aged from 1.8 to 10 years old since 2018 to present. After completion of induction chemotherapy according to NB-2004protocol, all patients underwent local tumor control with surgery and additional local radiotherapy in 3 cases. Four patients were received a single high-dose chemotherapy with autologous bone marrow transplant; one patient was received high-dose chemotherapy with haploidentical stem cell transplantation. Tree patients were received the tandem high-dose chemotherapy with autologous stem-cell transplantation. Two (25 %) patients had a primary resistant NB, and remission was achieved only after three lines of chemotherapy. One (12.5 %) child was diagnosed with a recurrence of NB of a solitary lesion in the right frontal lobe of the brain 8 months after the completion of the treatment according to the NB-2004protocol. Thus, 3 people from the study group received anti-GD2 antibodies after applying several lines of chemotherapy. All children at the start ofanti-GD2 antibody therapy were no progressive disease.

Results. A total of 21 courses of immunotherapy were conducted. No progressive disease was the main condition of anti-GD2 antibodies immunotherapy, such patients were excluded from the research. The antibodies were administered as a continuous infusion during 10 days (240 hours) in a daily dose of 10 mg/m2. When using the antibodies, the following adverse events were noted: hematotoxicity in 90.4 % cases, fever in 71.4 %, diarrhea in 23.8 %, capillary leak syndrome in 19 % patients, neuropathy and cytokine release syndrome were detected in 14.9 % cases, pain — in 9.5 % patients. All complications were tolerable and cured, dose reduction was not required. Now 7patients are in remission, in one patient after the first cycle of immunotherapy progressive disease was revealed.

Conclusions. The immunotherapy monoclonal anti-GD2 antibodies are well tolerable and safe procedure with adequate maintenance therapy. According to international experience the efficiency does not raise her doubts.

Introduction. Neuroblastoma (NB) — is a malignant tumor of sympathetic nervous system in children. In our country there are about 50 % of patients in high risk group, 50 % have a relapse or a progression of the disease which is the main reason for their death.

The purpose of the study. Our aim was to characterize a relapse/progression of NB in children in the Republic of Belarus, the analysis of results of treatment of patients from 1997 till 2017.

Materials and methods. From October, 1997 to December, 2017 in Republican Scientific and Practical Center for Pediatric Oncology, Hematology and Immunology 293 protocol and observed patients received 1st line therapy. Out of them 73 patients had a progression or a relapse of the disease. This group of patients was taken for the analysis.

Results. 14-year overal survival of patients with a relapse/progression of the disease was 21 + 6 % (n = 73), event free survival 18 + 5 % (n = 53). Patients older than 12 months at the time of diagnosis were statistically significantly more likely to recur, with 4th stage disease and having N-MYC amplification, diploid karyotype in the tumor. Adverse relapse options for localization are about 65 % (generalized — 41 %, osteomedullary — 12.3 %, relapses with CNS damage — 12.3 %). The most curable variants of relapses are found in 35 % (n = 25) of cases. In patients from the group of favorable and intermediate prognosis, relapses in the zone of primary tumor localization were significantly more frequent (p < 0.0001), bone loss in the relapse of the disease was significantly less common (p < 0.0001). The best results with the use of standard therapies are relapse with the primary tumor zone and soft tissue relapse (by 5 year relapse-free survival — 25% and 36 % respectively). Treatment of osteomedullary and generalized relapses using the methods available to us is not effective (EFS is 0).

Conclusion. Further detailed study of the biology of the tumor is necessary, in order to create for the therapy of the disease targeted drugs and other biologically based approaches, such various types of immunotherapy.

Neuroblastoma (NB) is the most common extracranial solid tumor of childhood, accounting for 6—10 % of all malignant neoplasms in children. One of the fundamentally important problems for the clinician is the stratification of the risk group and the definition of treatment tactics for patients with NB. In this work, we studied the dependence of the expression of CRABP1 protein in the primary focus and tumor metastases on the type of genetic disorders (amplification of the MYCN gene, deletion of the 11q23 locus) and the effect of the therapy in patients with NB.

There were two pediatric patients under our supervision at Saint-Petersburg State Pediatric Medical University clinic from 2018 to 2019 with type 3 disease of von Willebrand, who needed the surgical treatment. The surgery was made with Haemate® P which is used as for long-term prophylaxis, as for short-term prophylaxis with surgical treatment for disease of von Willebrand. Application of Haemate® P was 100 % successful in both cases.

The receptor tyrosine kinase genes (NTRK1, 2, 3) are proto-oncogenes that are found in various solid tumors in children and adults. Currently, next-generation sequencing (NGS) is the preferred platform for detecting NTRK gene translocations. The scientific literature presents data on the effectiveness of entrectinib in patients with translocation of the NTRK, ROS1 and ALK genes, regardless of the partner gene. In the future, entreсtinib therapy can become an alternative to chemotherapy or can be used in combination or complex treatment of both primary malignant neoplasms and in the case of refractory, unresectable and recurrent forms of the disease.

Venous thromboembolism (VTE) is a frequent complication in children being treated for cancer. Pathogenesis of VTE in children with cancer is multifactorial, including changes in the hemostatic system caused by the primary disease itself as well as methods of treatment.

According to biopsychosocial approach in modern standards of medical care for oncological patients one of the integral component is attention to the psycho-social functioning of both the patient and his family members. The issues of confirmation of adaptation/maladaptation criteria is particularly important for developing effective models of psycho-social support of treatment and rehabilitation, methods of identification and monitoring of families in need for various types of psychological support. International organizations recommend in their manuals the use of regular screening of emotional distress as an integrative assessment of the patient psychological conditions. They define distress as a “sixth life indicator” in addition to body temperature, breathing, blood pressure, pulse and pain. The article summarizes the basic information about the concept of “emotional distress”, analyzes the possibilities and limitations of its use as a criterion for assessing maladaptation in the treatment process. The feasibility of implementing this concept and the corresponding measurement method in the Russian practice of psychological support of pediatric patients and theirfamilies at different stages of treatment is shown. The article substantiates the possibility of using this criterion for rapid and reliable identification of the need for additional (psychological/psychiatric) care in relation to the treatment of the underlying disease of the child; to monitor the dynamics of the emotional state of patients and their parents at each stage of treatment.

Introduction. Retinoblastoma (Rb) is a rare intraocular tumor, which predominantly occurs in young children. The number of scientific studies and research, dedicated to extraocular Rb with extension on the optic nerve is limited. There is no uniform opinion on how to choose place and method on the surgical phase of treatment for patients with tumor’s extension on the optic nerve.

Description of the case report. The article presents the clinical case of extraocular Rb with extension on the optic nerve in a patient at the age of 5years 1 month. After 3 courses of polychemotherapy a one-stage sequential operation was performed, namely, prechiasmal resection of the optic nerve with eye enucleation. Radiation therapy and adjuvant therapy was carried out in the postoperative period. It was decided to abstain from performing high-dosed chemotherapy. The disease-free observation period from the end of treatment is 6.5 years.

Conclusion. In view of the lack of sufficient experience in treating patients with extraocular Rb, due to the small number of studies in this section of oncology, we were interested to present this clinical case with an emphasis on the surgical stage which has positively influenced the outcome of the disease.

Leiomyoma is a benign tumor of smooth muscle cells. Localization of this formation in the adrenal glands is extremely rare, even less common is the defeat of both adrenal glands. In the literature, we found less than 10 described cases of the occurrence of a similar pathology in children. The article presents a case of a 17-year-old girl with complaints of tachycardia and an increase in pressure, which subsequently revealed bilateral adrenal masses. Histological verification diagnosed leiomyoma.