Aim

To compare two consecutive treatment programs in metastatic osteosarcoma at presentation.

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)

The aim of this study was to analyze the frequency and reveal the most common reasons of the endoprosthetic instability in patients with malignant bone tumors. From 1992 – 2008, 625/515 patients, endoprosthetic replacement of major joints were performed. The median age of the patients was 30.3 years (13 to 72 years).

Aseptic instability was observed after 3/71(4.2%) humeral joint replacement out of total operations at this location, after 4/80 (5%) hip prosthesis, after 19/133 (14%) proximal tibial prostheses, after 44/299 (14.7%) distal femoral prostheses and after 2/37 (5.4%) total femur replacements.

The retrospective analyses has shown that the reasons of instability were the following: aseptic loosening of the stems of endoprosthesis in 26 cases (24.4%), stem break in 31 (36.1%), endoprosthetic unit destruction in 10 (11.6%), untwistment of fixational screws in 10 (11,6%), migration of hip endoprosthesis components in 2 (2.3%) and endoprosthesis dislocation in 12 (14%). The timing of endoprosthetic instability ranged from 7 days to 12.2 years (average 26.2 months). Statistic analyses was performed in a group of patients with aseptic endoprosthesis instability developed after proximal tibia and distal femur resection.

We conclude that the most frequent reason of aseptic instability was endoprosthetic stem break. The instability rate was actually lower among the patients who had underwent 5–10cm distal tibia resection comparing with the group of 10–15cm bone mass resection (p=0.05). Femoral resection enhanced the instability frequency comparing with proximal tibia resection in the group of 5–10cm bone mass resection (p=0.05).

The purpose of study was to evaluate retrospectively the efficacy of Ifosfamide-Carboplatin containing chemotherapy in recurrent/refractory osteosarcoma and MFH of the extremities.

Twenty seven osteosarcoma and 2 MFH pts who had achieved complete surgical remission after multimodal treatment and then progressed soon after en-bloc bone resection or developed recurrent disease were included in two chemotherapy protocols. There were 20M/9F with ages ranging from 15 to 36 yrs (mean 20). Chemotherapy consisted of ifosfamide (median dose per cycle 7.5 g/m2) + carboplatin (median dose 350 mg/m2) + etoposide (median dose 450 mg/m2) – (regimen ICE) or doxorubicin 60 mg/m2 (regimen ICA). Response was evaluated according to RECIST. Survival was calculated from the time of R1 to death and analyzed as February 11, 2009.

In total 93 (from 1 to 5. mean 3) cycles were administrated between October 2003 and December 2008. Of 17 ICE pts 3 had PR (17.6%), 10 had SD (58.8%) and 4 (23.5%) – PD. Among 12 ICA pts 3 (25%) had PR, 6 (50%) had SD and 3 (25%) had PD. Sixteen pts (55%) without progression during chemotherapy achieved second surgical remission. At last follow-up 12 pts died of disease, 8 are AWD and 9 are NED. Actuarial 5-year survival was 35±16%, median 38 mos. Outcome was related to relapse-free interval. Five-year survival was 23±18% among patients who relapsed < 12 mos after CR1 and 64±18% among pts who relapsed later, p=0.3. 5-year survival was significantly better in pts in whom chemotherapy was followed by surgery for distant metastases − 37.8±27% (median 38 mos), versus 23.3±19% (median 11 mos.) in patients treated without surgery, p< 0.05.

We conclude that retrieval chemotherapy stopped disease progression in the majority of cases. Followed by surgery it was associated with better survival. These regimens and treatment strategy need further investigation in prospective trials.

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.

From 1992 on 2008, 615/515 patients underwent primary or revisional endoprosthetic replacement of major joints. In 51 patients (31 men & 20 women) modular system MUTARS (Implantcast, Germany) has been used. The median age was 23.3 years (15 to 52 years).

MUTARS modular endoprosthesis has been used in 10 patients with deep infection of endoprosthetic bed as a revisional endoprosthetic replacement: 1 Total endoprosthetic replacement of femur, 5 Total knee joint replacement (2 for distal femoral defect and 3 for proximal tibial defect). In 3(27%) patients, we used newly patented silver ion coated MUTARS either after two stage treatment for infection of endoprosthetic bed or as a prophylaxis of endoprosthetic infection.

In 1 patient (23 yrs), with 12cm limb length shortening, we used extensible MUTARS as a revisional endoprosthetic replacement.

The following complications we observed: Instability of endoprosthesis – 3/51 (5.9%), deep endoprosthetic bed infection – 4/51 (7.8%).

In comparison group, when using custom-made endoprosthesis, the frequency of infectious complications have made 60/574 (10.5 %), and instability of implants was observed in 79/574 (13.8 %) cases.

Transition of using modular systems for primary and revisional endoprosthesis allows to reduce the level of instability from 13.8 % to 5.9 %. The quantity of infectious complications is also not great as in comparison with control group. For revisional endoprosthetic replacement, we think, the given modular system is optimal, for correcting limb length deficiency and restoration of basic function at patients. Use of silver ion coated modular implants is a promising method for treating deep endoprosthetic bed infection.

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%.

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).

Analysis of correlation of proliferation index Ki67 with grade and recurrence type of soft tissue sarcomas.

We reviewed 34 patients treated in RCRC RAMS. 53% patients were female, 47% – male. Adult patients – 97%, children – 3%. Soft tissue tumors localized on lower extremities in 47% cases (hip, shank), on upper extremities in 20% cases (shoulder, forearm, hand), on trunk in 24% cases, on head and neck in 9% patients. Histological subtypes were monophase synovial sarcoma – 32%, malignant fibrous histiocytoma – 23%, liposarcoma – 18%, malignant shwannoma – 6%, and other types in isolated instances. Synovial sarcoma more often observed in young and middle age women, malignant fibrous histiocytoma – in old men, liposarcoma – equally often in middle and old men and women. We observed soft tissue sarcoma grade 2 (FNCLCC) more frequently. Local recurrence development in 35% cases, number of recurrences was from 1 to 6. Distant metastases were in 8 patients (in lungs, lymph nodes, bones). We used monoclonal antibody Ki67 (clone MIB-1). Proliferation index Ki67 evaluated in the following way: low level – < 25% of tumor cells, middle level – 25–50%, and high level – > 50% of tumor cells.

Proliferation activity Ki67 increase in cases with high grade soft tissue sarcoma (in grade 1 tumors – low and middle proliferation activity, in grade 2 tumors – middle and high proliferation activity, in grade 3 tumors – only high proliferation activity).