Alveolar Rhabdomyosarcoma (RMA) are characterised by chromosomal translocations fusing the PAX3 or PAX7 gene with FKHR in ~85%. Previous studies have suggested that PAX3/7-FKHR fusion types are related to prognosis. In order to prove these findings we performed a retrospective analysis of the PAX-FKHR fusion status and its relation to outcome in patients treated in the CWS trials. Between 1986 and 2004, out of 446 RMA patients treated in four consecutive CWS trials (CWS-86, -91, -96 or -2002-P), tumor samples from 121 patients with adequate quality for analysis of PAX-FKHR fusion status by RT-nested PCR were available. Survival analysis depending on clinical risk factors and fusion status was performed using the Kaplan-Meier Method, the log rank test and the Cox regression model. There were no major differences in distribution of known risk factors in the analysed cohort of 121 patients compared to all patients enrolled in the CWS trials. PAX-FKHR fusions were detected in 83%: 72 PAX3-, 29 PAX7-FKHR fusions. Patients with PAX3-FKHR positive tumors more often showed a pattern of adverse clinical risk factors (age > 10 years, primary metastases, lymph node involvement) than the PAX7-FKHR positive group. The 5-year event free survival rate of patients with initially metastatic tumors positive for either of the two fusion transcripts was significantly lower compared with the fusion transcript negative cohort and the non-analysed RMA patients. There was no significant outcome difference between patients with PAX3-FKHR and PAX7-FKHR positive tumors in uni- and multivariate analysis. In the present analysis, which is to our knowledge the largest reported so far, PAX-FKHR fusion type was no significant predictor for prognosis, thus not supporting results of previous studies

Introduction: Ewing’s sarcoma is the second commonest primary tumor in childhood and its 5-year survival is currently just over 70%. The aim of the current study was to identify the prognostic significance of p53 and hsp70 overexpression into the nuclei of tumor cells. Method: 30 patients treated for Ewing’s in a 15-year period, in a Children’s hospital, were included in the study. Treatment protocols included always Neo-adjuvant Chemo and did not considerably change in time. The male to female ratio was 1.8: 1. The average age was 10.5 years (range 2–18y). Central axis and extremities were equally affected, with pelvis being the commonest site. Expression of P53 and HSP70 in > 20% and > 15% of the tumor nuclei respectively, was considered compatible with mutation. The mean follow up was 7.2 years. Results: Seventeen patients eventually died. HSP70 trace was negative, meaning that at the time of biopsy the tumor nuclei were not expressing the protein. Mutated p53 was traced in 13% of the tumor nuclei and had significant negative prognostic value in 5-year survival (p=0.039) and in Event Free Survival (p=0.006). Discussion: The search of factors that could independently affect the prognosis in Ewing’s sarcoma continues. Identification of these factors would lead to application of more intense therapeutic means only to those patients with a poorer prognosis without getting everyone in the risk of adverse effects

Background: The incidence of osteosarcoma varies considerably with age and preschool children are extremely rarely affected. This study was conducted to investigate presentation, treatment, and outcome in very young children with osteosarcoma. Patients and methods: The authors retrospectively analyzed the data of 2706 consecutive COSS patients with newly diagnosed high-grade osteosacroma of bone and identified 28 patients (1.0%) aged less than five years at diagnosis. Demographic, diagnostic, tumor, and treatment related variables; response and survival data of these 28 were analyzed. Results: Of the 28 (male, N=16; female, N=12) toddlers, 27 presented with high-grade central osteosarcoma of an extremity (femur, N= 12; humerus, N=10; tibia, N=5) and one with a secondary osteosarcoma of the orbit. The size of primary extremity tumors was large (≥ 1/3 of the involved bone) in 20/27 evaluable patients. Primary metastases were detected in 4 children. All patients received multiagent chemotherapy, and 13/20 analyzed tumors responded well (> 90% necrosis) to neoadjuvant chemotherapy. Limb sparing surgery was performed in 11, ablative procedures were performed in 14, and no local surgery was performed in two patients with extremity tumors. With a median follow-up of 3.8 years (6.2 years for survivors), 13 patients were alive (CR1, N=12; CR3, N=1). Four patients never achieved a complete remission and 12 developed recurrences (local, N=3; metastatic, N=8; site unknown, N=1); and 15 of these 16 patients died. Five-year overall and event free survival probabilities were 50% (SE 10%) and 46% (SE 10%). Better survival was correlated with good response to chemotherapy. Conclusions: Osteosarcoma is extremely rare in pre-school children. These young patients often have large tumors which may require mutilating resections. Prognosis may be poorer than in older patients

Osteosarcoma is the most common bone tumour of the paediatric age. Long time survival can be reached in 70% of patients when metastatic disease is absent at presentation. But in spite of aggressive chemotherapy regimens, about 30% of patients die of the disease. This retrospective study was carried on 120 patients with primary non-metastatic osteosarcoma of the extremities, attending at Cairo University Hospitals (Faculty of Medicine and National Cancer Institute) between January 1993 and June 2006. The patients’ functional outcome was evaluated according to the Musculoskeletal Tumor Society Functional Rating System. All patients have undergone surgical resection of the tumour and limb salvage. They have received different chemotherapy regimens depending on the time of entry to the study. Four patients were treated according the Osteosarcoma Group Study I (OSGI): six courses of adjuvant cisplatin and doxorubicin. Twenty patients received OSGII: 2 neoadjuvant and 4 adjuvant courses of cisplatin and doxorubicin. Twenty-nine patients received OSGIII: high-dose methotrexate, ifosfamide, doxorubicin, and cisplatin. Sixty-seven patients received OSGIV: high dose cisplatin, ifosfamide, doxorubicin and a cardioprotective agent. Patients with limb salvage surgery were divided into 3 groups: mobile joints (33 patients), fused joints (75 patients) and rotation plasty (12 patients). The 5-year event free survival and overall survival for the 120 patients were 70.9 % and 71.3% respectively at median follow-up of 54.5 months and a range of 5–153 months. Functional outcome for available patients according to MST rating system was < 70% in 34 patients and > 70 % in 86 patients. There was not a statistically significant difference between survival and different prognostic factors (age, sex, tumour site, tumour size, tumour necrosis, pathology and time of chemotherapy). Only serum LDH and alkaline phosphatase were statistically significant when correlated with survival. The results of this study seem to be in accordance with other studies in the literature

The December 2012 Trauma Roundup³⁶⁰ looks at: more is not always better, especially when its chemotherapy; new hope for skeletal metastasis; biopsy tracts; intra-operative imaging of sarcomas; curettage with adjuvant therapy; amputation and distal tibial osteosarcoma; and diaphyseal tibial tumours.

The October 2013 Oncology Roundup³⁶⁰ looks at: En bloc resection, irradiation and re-implantation; Metastasis and osteosarcoma; Mobile spine and osteosarcoma; Denosumab miraculous for GCT; Fevers, megaprostheses and sarcomas; PET and prognosis; Canine sarcomas not so different?; Bone cement and giant cell tumours.