Background. Although soft tissue sarcoma (STS) is a rare malignancy, myxofibrosarcoma is a common form diagnosed. Myxofibrosarcoma is complicated by a high local recurrence rate (18–54%) and significant morbidity following treatment, hence management can be challenging. Patients and Methods. Patients treated between 2003–2012 were identified via a database within the histopathology department and case notes were retrospectively assessed. All histology samples were reviewed by a senior histopathologist to ensure a correct diagnosis. Results. 29 patients (12 male, 17 female) with an average age of 61 years (range 19–89 years) underwent surgery at a single centre, with 24 patients receiving adjuvant and two receiving neo-adjuvant radiotherapy. 22 patients had lower limb and 7 had upper limb tumours. 3 were treated for secondary recurrence after having primary surgery elsewhere. 21 patients had Trojani Grade 2 or 3 tumours. All underwent limb-sparing surgery initially but six patients (20.7%) suffered local recurrence after an average follow-up of 28 months and all ultimately required above knee amputation. Four patients developed wound infection, with one requiring VAC therapy. One patient required a flap repair of the forearm. 5-year survival rate was 87.5%. Conclusions. Our results compare favourably against results published so far in the literature with a low local recurrence rate and mortality. Limb-sparing surgery aims to reduce morbidity and disability following treatment but more research is required in adjuvant treatments to further reduce the risk of local recurrence of tumour

Ewing sarcoma (ES) and Osteosarcoma (OS) are the 2 most common malignant primary bone tumors. A patient's response to neoadjuvant chemotherapy has important implications in subsequent patient management and prognosis, as a favourable response to chemotherapy allows orthopedic oncologists to be more aggressive in pursuing limb-sparing surgery. An accurate and non-invasive pre-operative marker of response would be ideal for planning surgical margins and as a prognostic tool. ES and OS have differing biological characterisitcs and respond differently to chemotherapy. We reviewed 18F-FDG PET imaging characteristics of ES and OS patients at baseline and following treatment to determine whether this biological variation is reflected in their imaging phenotype. A retrospective review of ES and OS patients treated with neoadjuvant chemotherapy and surgery was done, correlating PET results with histologic response to chemotherapy. Change in the maximum standardized Uptake Value (SUVmax) between baseline and post-treatment scanning was not significantly associated with histologic response for either ES or OS. Metabolic tumor volume (MTV) and the percentage of injected 18F-FDG dose (%ID) in the primary tumor were found to be different for ES and OS response subgroups. A 50% reduction in MTV (MTV2:1 < 0.5) was found to be significantly associated with histologic response in OS. Using the same criteria for ES incorrectly predicted good responders. Increasing the cut-offs for ES to a 90% reduction in MTV (MTV 2:1 < 0.1) resulted in association with histologic response. Response to neoadjuvant chemotherapy as reflected by changes in PET characteristics should be interpreted differently for ES and OS

Background. Decisions about local treatment are important in osteosarcoma treatment. The purpose of this study was to review decisions about local treatment in one centre. Methods. This was a retrospective review of the records of all patients with high-grade extremity osteosarcoma presenting to our centre between 1997 and 2008. Particular attention was paid to local control decisions. Results. 54 patients were included, 37 were male. Median age was 18 (4.1 to 71.3 years). The anatomical location was distal femur in 33, tibia in 8, humerus in 7, ankle/foot in 3, fibula in 2 and clavicle in 1. 8 (14.8%) patients had metastases at presentation. 13 (24.1%) patients underwent primary amputation, predominantly in the early years of the series. The remaining 41 patients had limb-sparing surgery, 5 of whom had microscopically positive margins. 21 of 54 (38.8%) had >90% necrosis in the resected tumour. 3 patients had poor necrosis and positive margins. These were a 70 yo intolerant of chemotherapy, who refused amputation, developed LR and metastatic disease; a 15 yo with metastatic disease, who had a secondary amputation and metastatectomy and survived and a 43 yo who developed metastases and LR on chemotherapy. 4 further patients had local recurrence after LSS. All had poor necrosis after chemotherapy but adequate margins. All developed metastatic disease and 3 have died. Overall survival was 60%. 5-year survival without metastatic disease at presentation was 65%. Conclusion. Our series is similar to other centres. Challenges include older patients, poor response to chemotherapy and metastases

Prior to the 1970s, almost all bone sarcomas were treated by amputation. The first distal femoral resection and reconstruction was performed in 1973 by Dr Kenneth C Francis at the Memorial Sloan-Kettering Cancer Centre in New York. Since that time, limb-sparing surgery for primary sarcoma has become the mainstay of sarcoma surgery throughout the world. Initially, the use of mega-prostheses of increasing complexity, involving all the major long bones and both pelvic and shoulder girdles, was popularised. In the early 1980s, wide use of massive allograft reconstructions became widespread in both Europe and in multiple centres in the USA and UK. Since that time, increasing complexity in the design of prostheses has allowed for increasing functional reconstructions to occur, but the use of allograft has become less popular due to the development of late graft failures of patients survive past ten years. Fracture rates approaching 50% at 10 years are reported, and thus, other forms of reconstruction are being sought. Techniques of leg lengthening, and bone docking procedures to replace segmental bone loss to tumour are now employed, but the use of biological vascularised reconstructions are becoming more common as patient survivorship increases with children surviving their disease. The use of vascularised fibular graft, composite grafts and re-implantation of extra-corporeally irradiated bone segments are becoming more popular. The improvement in survivorship brought about the use of chemotherapy is producing a population of patients with at least a 65% ten year survivorship, and as many of these patients are children, limb salvage procedures have to survive for many decades. The use of growing prostheses for children have been available for some 25 years, first commencing in Stanmore, UK, with mechanical lengthening prostheses. Non-invasive electro-magnetic induction coil mechanisms are now available to produce leg lengthening, with out the need for open surgery. Whilst many of these techniques have great success, the area of soft tissue attachment to metallic prostheses has not been solved, and reattachment of muscles is of great importance, of course, for return of function. There are great problems in the shoulder joints where sacrifice of rotator cuff muscles is necessary in obtaining adequate disease clearance at the time of primary resection, and a stable shoulder construct, with good movement, has yet to emerge. Similar areas of great difficultly remain the peri-acetabular and sacro-iliac resections in the pelvis. Perhaps the real future of the art of limb salvage will be in the reconstruction of failed major joint replacements where there is great loss of bone stock, and already massive tumour prostheses are providing a salvage pathway for failed standard joint replacement. The final future for limb salvage, however, may not rest with increasing surgical complexity and innovation, but with the development of molecular biology and specific targeted treatments, according to the cytogenetics of a particular tumour. We are on the threshold of yet another quantum change in the approach to cancer management; just as chemotherapy brought a tremendous change in the 1970s, molecular biology is the frontier to make much of the current limb salvage surgery that is performed redundant