Bone sarcomas account for 7% of malignant tumors in children, and osteosarcomas make up 35–50% of them. Many patients are diagnosed at spread stages of the disease, which dictates the need to search for new approaches to treatment. At the same time, the optimal therapeutic strategy is unknown, the results of treatment of such children remain unsatisfactory. Part I of this article describes currently used treatment regimens. Part II presents achievements in the field of personalized therapy. Thus, changes in treatment approaches illustrate the vector of development of the treatment principles of such complex diseases, being fatal for most patients a few years ago.

Solid tumors in children occupy the second place in the structure of morbidity, yielding to hemoblastosis. Among solid tumors, approximately 5% are bone sarcomas: osteosarcoma (3%) and Ewing’s sarcoma (2%). Atypicality of the course of these diseases makes it difficult to diagnose them early. The article describes a series of clinical observations of patients with bone sarcomas, which illustrate the complexity of diagnosing diseases of this group.

Actually targeted drugs became a therapeutic component for adult patients with malignancies and merge into clinical therapeutic recommendations. Targeted drugs in pediatric oncology had been using for 10-15 years and this experience under systematization and analysis. In the current issue 12-year experience of targeted therapy of Pediatric Oncology and Haematology Research Institute of N.N.Blokhin National Cancer Research Center is presented. Authors estimated efficacy and toxicity of rituximab, bortezomib, sorafenib and everolimus in 108 patients with advanced stages of mature B-cell non-Hodgkins lymphomas (B-NHL), relapsed/refractory acute lymphoblastic leukemia (ALL) and refractory osteosarcoma. Rituximab plus standard chemotherapy of advanced stages of B-NHL increased 10-years event-free survival up to 93.8±3%. Bortezomib plus anti-relapsed chemotherapy protocol (COG AALL07P1) reached minimal residual disease - negative status in 64.3% patients, but relapse-free survival was 24.5±19.4% (median 28±3.3 months). Sorafenib and everolimus were used for refractory osteosarcoma treatment, response rate was 100%, progression-free survival more than 6 months was 46% (median follow up 7±1.2 months). Toxicity profile was acceptable.Our data demonstrate a fact of significant improvement of advanced stages B-NHL treatment results by rituximab use, but relapsed/refractory ALL and osteosarcoma need treatment improvement.

Fundamental studies in molecular biology, immunology, cytogenetics and epigenetics of tumour cell revealed main molecular ways of neoplastic transformation and helped to perform their modification using targeted drugs (TD). With the development of knowledge in molecular and biologic basis of oncogenesis the spectrum of targets have been increased: suppression of angiogenesis, pathways, oncogene proteins, and enzymes. Nowadays TD became an essential part of malignancies therapy standards for adult patients. Place and role of TD in pediatric oncology are studying. Adverse events are revealing, treatment results of traditional chemotherapy protocols and programs with TD are analyzing. This review summarizes current international experience in targeted therapy, highlights indications and regimens of administration. Future perspectives in individualized chemotherapy based on tumor «molecular portrait» are demonstrated.

ВВЕДЕНИЕ Остеосаркома — первично злокачественная опухоль костей, которая развивается из примитивных мезенхимальных стволовых клеток, способных дифференцироваться в костную, хрящевую или фиброзную ткани; составляет 3% всех злокачественных опухолей у детей и подростков. Частота встречаемости — 4 случая на 1 млн детского населения в год, что составляет 35–50% всех злокачественных опухолей костей в детском возрасте [1, 2]. DOI: 10.15690/onco.v2.i4.1467 А. Штернхайм , Д. Левин , М. Министерский , А. Ниркин , Дж. Бикелс , С. Дадиа , Е. Коллендер , Е. Гортзак , М.Ю. Рыков, Э.Р. Сенжапова, А.З. Дзампаев, В.Г. Поляков 4 1 Клинический центр педиатрии Дана-Дуэк, Тель-Авив, Израиль 2 Медицинский центр им. Э. Сураски, Тель-Авив, Израиль 3 Российский онкологический научный центр им. Н.Н. Блохина Минздрава России, Москва, Российская Федерация 4 Российская медицинская академия последипломного образования Минздрава России, Москва, Российская Федерация