The aim of the our study was to analyze prognostic factors characterizing biological behaviour of a tumour and specific features of the patient and to develop rational strategy of the combined treatment of chondrosarcoma (CHS) in children.

Between 1982 and 2008 seventy seven patients with CHS were observed and treated in our center. 38 (49,4%) were male and 39 (50,6%) were female. In all cases the diagnosis was confirmed by histological examination. In cases of high grade/mesenchymal or metastatic (into lungs) CHS we use polychemotherapy consist of alternating courses of CDDP, adriamicin, ifosfamide and etoposide and high-dose methotrexate (8–12 g/m²). Intensive polychemotherapy allow us to expand indications for limb salvage treatment. Using growing (conventional and non-invasive types) endoprostesis improved the quality of life. 5-years RFS was 75,4±7,8 % (Kaplan-Meier curves, p=0.02). The most significant prognostic factors were grade of histological response, morphological type of tumour and type of polychemotherapy (conventional or intensive)

We analyzed the results of the intensive multimodality therapy of children with localized Ewing’s family tumors of the ribs (EFTR). 22 patients with localized EFTR were treated in our institute between 1996 and 2006. The age is ranged from 4 to 15 years. 10 patients were male, 12 – female. Eight patients had a classic Ewing sarcoma(ES), 14 – PNET. The high risk criterion was tumor volume over 100 ml. The treatment plan included intensive induction chemotherapy (adapted to risk group) consist of 5 courses (1, 3, 5 courses – vincristine, adriamicin, cyclophosphamide, 2, 4 courses – ifosfamide and etoposide) and local control (surgery and radiotherapy), and consolidation with or 5 courses standard therapy or high-dose chemotherapy with stem-cell rescue. 4 patients underwent high-dose chemotherapy, 18 – consolidation with standard arm.

Two patients died from complications of chemotherapy, 20 patients completed the treatment. 5-year overall survival (OAL) of patients was 70±10,4%, 5-year event-free survival (EFS) was 58,9±11,4%. We conclude that Ewing’s family tumors are the most common tumors of ribs in childhood. Improved EFS requires more aggressive systemic chemotherapy and surgery (removing of entire affected ribs). Long-term survival is possible, even for high risk patients.

Synovial sarcoma is the most common NRSTS, that typically affects the extremities of adolescents. To improve the results of the treatment of synovial sarcoma for children and adolescents is the target of this study. 19 children and adolescents at the mean age of 10,84±3,28 years (9 males, 10 females) with synovial sarcoma were treated between 1999 and 2008 years at the Research Institution of Pediatric Oncology in the Russian Cancer Center. Histologically, 5 patients had the biphasic,12 had the monophasic, and 2 of them had the poorly differentiated pattern. The most often affected area was the area of the lower extremity – 10 cases, the area of the upper extremity was affected in 3 cases, and the trunk – 6 cases. According to the staging systems adopted, the size > 5cm (TB) was reported in 12 cases. Five patients (non-staging) had relapse of disease. Four patients had nodal involvement, and 4 had distant metastases (mostly at lungs).

The general scheme of the treatment included: 8 courses of chemotherapy (used ifosfamide or cyclophosphamide, ethoposide, carboplatine); the harvesting and preservation of the stem cells after the stimulation of the haemophoesis by G-CSF, the stage of the local control of the tumor consisting of the surgical ablation of the primary lesion (in 1 case it was not available) and the radiotherapy of the initial tumor and metastasis left after the induction.

The partial effect was registered by most of the patients – 80%. We observed 1 case of progression of the disease during inductive CT. The toxicity of intensive chemotherapy was reduced by support of sub transplantation doses of peripheral blood stem cells – 0,9-1,5±0,1·106 per kg. In our research we have analyzed the 5-year overall and disease free survival. Thus, 5-year disease-free survival was 66,1±11,3 %, overall 5-year survival −75,6±10,6%.

The purpose of the our study was to analyze prognostic factors characterizing biological behaviour of a tumour and specific features of the patient and to develop rational strategy of the combined treatment of malignant fibrous histiocytoma (MFH) in children.

Between 1982 and 2008 fifty patients with MFH were observed and treated in our center. 24 (48%) were male and 26 (52%) were female. In all cases the diagnosis was confirmed by histological examination. We use polychemotherapy consist of alternating courses of CDDP, adriamicin, ifosfamide and etoposide and high-dose methotrexate (8–12 g/m²). Intensive polychemo-therapy allow us to expand indications for limb salvage treatment. Using growing (conventional and non-invasive types) endoprostesis improved the quality of life. 2-years RFS was 80,9±8,5% and 5-years RFS was 70,4±10,1% (Kaplan-Meier curves, p=0.03). The most significant prognostic factors were grade of histological response and type of polychemotherapy (conventional or intensive).

The aim of our study was to increase of survival of children with osteosarcoma by intensification of chemotherapy by inclusion of high dose methotrexate. 53 patients were treated in our centre between 2003 and 2007. Age are ranged from 5 to 16 years. 23 (43,4%) patients had metastetic disease. Polychemotherapy consist of alternating courses of CDDP, adriamicin, ifosfamide and etoposide and high-dose methotrexate (8–12 g/m²). In 25 (51%) cases have been received objective response (CR+PR). 38 (71,7%) patients alive at present time. 2 patients died from complications of treatment. 7 patients had PD, 1 — local relapse, 4 — metastatic relapse, 1 — combined relapse. 2-year OAS was 75,2±6,8%, 2-year RFS was 65±7,8%.