Ewing Sarcoma is the second most common primary bone sarcoma in young patients, however, there remains geographical variation in the treatment of these tumours. All patients receive neoadjuvant chemotherapy and, in most cases, the soft tissue mass diminishes significantly in volume. Controversy surrounds whether to then treat the pre- or post-chemotherapy tumour volume. Many centres advocate either (1) resection of the pre-chemotherapy volume or (2) treatment of the pre-chemotherapy volume with radiation followed by resection of the post-chemotherapy volume. These approaches increase both the short and long-term morbidity for this young patient population. In this study, we retrospectively reviewed our experience resecting only the post-chemotherapy volume without the use of (neo)adjuvant radiotherapy. A retrospective analysis of all patients with Ewing Sarcoma treated at a tertiary orthopaedic oncology centre was conducted. All patients were treated as per the consensus opinion of the multidisciplinary tumour board. Demographic and oncological variables were collected from our institutional database. Presentation and re-staging MRI scans were reviewed to evaluate pre- and post-chemotherapy tumour volumes. Operative and pathology reports were utilized to determine the extent of the surgical resection. Outcome variables included local recurrence free-, metastasis free- and overall survival. Sixty-five patients were identified in our institutional database of which 56 did not receive (neo)adjuvant radiotherapy. Median age at diagnosis was 24 years (range 13–64), 60% of patients were male and 67.6% of tumours were located in the appendicular skeleton. All 56 patients not treated with radiotherapy had resection of the post-chemotherapy tumour volume. There were 3 local recurrences in this group with a mean follow-up of 70.8 months (range 2 to 328). The median overall survival was 47 months and the mean of 70.8months. The rate of local recurrence is comparable to reports in the literature in which patients had their entire pre-chemotherapy tumour volume treated by radiation and/or surgery. Similarly, two-year overall survival for our patient cohort is not significantly different from previous studies in which more aggressive local control measures were employed. Resecting the post-chemotherapy tumour volume in Ewing Sarcoma without the use of (neo)adjuvant radiotherapy does not appear to increase the risk of local recurrence or negatively impact overall survival. This approach should be studied further as it reduces the risk of short and long-term complications for this patient population.”

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

Aims. Present the outcomes of those patients diagnosed with Ewing's Sarcoma of the foot within the past 10 years and treated at the Royal National Orthopaedic Hospital's Bone Tumour Unit, Stanmore. Methods. Retrospective study of the cases identified from the pathology database. Notes reviewed for presentation, treatment and follow up. TESS (Toronto Extremity Salvage Score) and MSTS (Musculoskeletal tumour score) were calculated. Results. 6 patients identified with positive diagnosis of Ewing's Sarcoma of the Foot. Male:Female ratio of 5:1. Age range 15–31 (Mode 25). 4 cases skeletal, 2 extra skeletal. All cases reviewed by supra-regional MDT and received adjuvant and neo-adjuvant chemotherapy. All except one patient underwent limb-salvage surgery. The MDT decision for the remaining patient was that amputation was the only viable surgical option but the patient and his parents requested radiotherapy without surgical treatment. Mean survival 40 months (15–107 months). All patients survive at time of submission. Mean MSTS/TESS scores 93% (80–100%) and 94.6% (85–100) respectively. Discussion. All patients reported a delay between first presentation and referral to the sarcoma unit. This experience is common across the literature for this rare pathology. Lowest scores were submitted by the two patients who had amputation of their great toe. All patients are happy with their outcome and decision to salvage their limb. All patients scored themselves as “not at all disabled” and two stated this would not have been their response if they had lost their foot. Conclusion. Amputation is psychologically difficult to accept and patients are more receptive to limb salvage surgery. Our patients demonstrate good functional outcome. Our experience at Stanmore suggests that limb salvage surgery with adequate MDT surveillance for Ewing's Sarcoma of the Foot can be a viable alternative to amputation

Purpose

Ewings Sarcoma (ES) and Osteosarcoma (OS) behave and respond differently to chemotherapy and any interpretation of diagnostics tests to predict a patients response to treatment must consider this. We reviewed 18F-FDG PET imaging characteristics of consecutive series of ES and OS patients to determine if any differences in PET imaging existed between them.

Purpose

Ewings sarcoma (EWS) is the second most common primary malignancy of bone. Five-year survival ranges between 50 and 70% for patients who present with localized disease. EWS occurs in both axial and appendicular locations. The prognostic importance of anatomic location (axial vs. appendicular) is unclear. The purpose of this study is to report a single institution experience in the management of patients with EWS, and to investigate anatomic location as a prognostic factor.

The management of primary malignant bone tumors with metastatic disease at presentation remains a challenge. While surgical resection has been shown to improve overall survival among patients with non-metastatic malignant bone tumors, current evidence regarding the utility of surgery in improving overall survival in metastatic patients remains limited. The 2004–2016 National Cancer Database (NCDB) was queried using International Classification of Diseases 3rd Edition (ICD-O-3) topographical codes to identify patients with primary malignant bone tumors of the extremities (C40.0-C40.3, C40.8 and C40.9) and/or pelvis (C41.4). Patients with malignant bone tumors of the axial skeleton (head/skull, trunk and spinal column) were excluded, as these cases are not routinely encountered and/or managed by orthopaedic oncologists. Histological codes were used to categorize the tumors into the following groups - osteosarcomas, chondrosarcomas, and Ewing sarcomas. Patients who were classified as stage I, II or III, based on American Joint Commission of Cancer (AJCC) guidelines, were excluded. Only patients with metastatic disease at presentation were included in the final study sample. The study sample was divided into two distinct groups – those who underwent surgical resection of the primary tumors vs. those who did not receive any surgery of the primary tumor. Kaplan-Meier survival analysis was used to report unadjusted 5-year overall survival rates between patients who underwent surgical resection of the primary tumor, compared to those who did not. Multi-variate Cox regression analyses were used to assess whether undergoing surgical resection of the primary tumor was associated with improved overall survival, after controlling for differences in baseline demographics, tumor characteristics (grade, location, histological type and tumor size), and treatment patterns (underwent metastatectomy of distal and/or regional sites, positive vs. negative surgical margins, use of radiation therapy and/or chemotherapy). Additional sensitivity analyses, stratified by histologic type for osteosarcomas, chondrosarcomas and Ewing sarcomas, were used to assess prognostic factors for overall survival. A total of 2,288 primary malignant bone tumors (1,121 osteosarcomas, 345 chondrosarcomas, and 822 Ewing sarcomas) with metastatic disease at presentation were included – out of which 1,066 (46.0%) underwent a surgical resection of the primary site. Overall 5-year survival rates, on unadjusted Kaplan-Meier log-rank analysis, were significantly better for individuals who underwent surgical resection vs. those who did not receive any surgery (31.7% vs. 17.3%; p<0.001). After controlling for differences in baseline demographics, tumor characteristics and treatment patterns, undergoing surgical resection of primary site was associated with a reduced overall mortality (HR 0.42 [95% CI 0.36–0.49]; p<0.001). Undergoing metastectomy (HR 0.92 [95% CI 0.81–1.05]; p=0.235) was not associated with a significant improvement in overall survival. On stratified analysis, radiation therapy was associated with improved overall survival for Ewing Sarcoma (HR 0.71 [95% CI 0.57–0.88]; p=0.002), but not for osteosarcoma (HR 1.14 [95% CI 0.91–1.43]; p=0.643) or chondrosarcoma (HR 1.08 [95 % CI 0.78–1.50]; p=0.643). Chemotherapy was associated with improved overall survival for osteosarcoma (HR 0.50 [95% CI 0.39–0.64]; p<0.001) and chondrosarcoma (HR 0.62 [95% CI 0.45–0.85]; p=0.003), but not Ewing sarcoma (HR 0.79 [95% CI 0.46–1.35]; p=0.385). Surgical resection of the primary site significantly improves overall survival for primary malignant bone tumors with metastatic disease at presentation. Physicians should strongly consider surgical resection of the primary tumor, with adjunct systemic and/or radiation therapy (dependent on tumor histology), in patients presenting with metastatic disease at presentation

Primary malignant bone tumours are a scarce entity with limited population-based data from developing countries. The aim of the study is to investigate the frequency and anatomical distribution of primary malignant bone tumours in a local South African population. This will be an epidemiological retrospective study. Data will be used of patients that were diagnosed with primary malignant bone tumours over a period of nine years spanning from 1 January 2014 to 31 December 2022. This data will be received from private and government laboratories. Data to be considered are type of primary malignant bone tumours diagnosed, incidence of primary malignant bone tumours over a period of nine years and the most common anatomical sites of primary malignant bone tumours. The rationale behind our study is to assess the frequency of different primary malignant bone tumours in another geographic area of South Africa and to compare these findings to local and international literature. With a projected increase in diagnosis of primary malignant bone tumours in developing countries it is important to have more available data about primary malignant bone tumours from these areas to have a better understanding of these conditions and to understand the impact of the burden they impose on healthcare systems so that management of these conditions can also be improved. Preliminary results show that 23.83% of primary malignant bone tumours occurred in the age group 0–24 years of age, 49.22% in the 25–59 age group and 26.95% in the 60+ age group. The most common tumour that occurred was chondrosarcoma (49.21%) followed by osteosarcoma (41.80%) then Ewing's sarcoma (4,69%) and lastly chordoma (4.30%). From the 256 samples that met the inclusion criteria the five most common anatomical sites were distal femur (63), proximal tibia (41), proximal humerus (38), pelvis (34) and proximal femur (20)

Background. Dislocation is a common complication after proximal and total femur prosthesis reconstruction for primary bone sarcoma patients. Expandable prosthesis in children puts an additional challenge due to the lengthening process. Hip stability is impaired due to multiple factors: Resection of the hip stabilizers as part of the sarcoma resection: forces acts on the hip during the lengthening; and mismatch of native growing acetabulum to the metal femoral head. Surgical solutions described in literature are various with reported low rates of success. Objective. Assess a novel 3D surgical planning technology by use of 3D models (computerized and physical), 3D planning, and Patient Specific Instruments (PSI) in supporting correction of young children suffering from hip instability after expandable prosthesis reconstruction following proximal femur resection. This innovative technology creates a new dimension of visualization and customization, and could improve understanding of this complex problem and facilitate the surgical decision making and procedure. Method. Two children, both patients with Ewing Sarcoma of the left proximal femur stage-IIB, ages 3/5 years at diagnosis, were treated with conventional chemotherapy followed by proximal femur resection. Both were reconstructed with expandable prosthesis (one at resection and other 4 years after resection). Hip migration developed gradually during lengthening process in the 24m follow up period. 3D software (Mimics, Materialise, Belgium) were used to make computerized 3D models of patients' pelvises. These were used to 3D print 1:1 physical models. Custom 3D planning software (MSk Lab, Imperial College London) allowed surgeons visualizing the anatomical status and assess of problem severity. Thereafter, osteotomies planes and the desired position of acetabular roof after reduction of hip joint were planned by the surgeons. These plans were used to generate 3D printed PSIs to guide the osteotomies during shelf and triple osteotomy surgeries. Accuracy of planning and PSIs were verified with fluoroscopy and post-op X-rays, by comparing cutting planes and post-op position of the acetabulum. Results. Surgeons reported excellent experience with the 3D models (computerized and physical). It helped them in the decision process with an improved understanding of the relationship between prosthesis head and acetabulum, a clear view of the osteophytes and bone formation surrounding the pseudoacetabulum, and osteophytes inside the native acetabulum. These osteophytes were not immediately visible on 2D CT imaging slices. Surgeons reported a good fit and PSIs' simplicity of use. The hip stability was satisfactory during surgery and in the immediate post-op period. X-ray showed a good and centered position of the hip and good levels of the osteotomies. Conclusions. 3D surgical planning and 3D printing was found to be very effective in assisting surgeons facing complex problems. In these particular cases neither CT nor MRI were able to visualize all bony formation and entrapment of prosthesis in the pseudoacetabulum. 3D visualisation can be very helpful for surgical treatment decisions, and by planning and executing surgery with the guidance of PSIs, surgeons can improve their surgical results. We believe that 3D technology and its advantages, can improve success rates of hip stability in this unique cohort of patients

There is very limited literature available on the use of prosthetic replacement in the treatment of primary and secondary tumours of the radius. In the past these were treated with vascularised and non-vascularised autografts which had associated donor site morbidity, problems of non union, graft or junctional fractures and delayed return to function. Our study is a mid to long term follow-up of implant survivorship and the functional outcome of metal prosthetic replacement used for primary and metastatic lesions of radius. We had 15 patients (8 males:7 females) with a mean age of 53 years. 8 patients underwent proximal radial replacement, 2 with mid-shaft radial replacement and 6 patients had distal radial replacements with wrist arthrodesis. The indications for replacement included metastatic lesions from renal cell carcinoma, primary giant cell tumours, ewings' sarcoma, chondroblastoma, radio-ulnar synostosis and benign fibrous histiocytoma. The average follow up was 5 years and 6 months (range 3 months - 18 years). Four patients died as a result of dissemination of renal cell carcinoma and two patients were lost to follow-up. There were no complications with the prosthesis or infection. Clinically and radiographically there was no loosening demonstrated at 18 years with secure fixation of implants. Two patients developed interossoeus nerve palsies which partially recovered. Functional outcomes of the elbow were assessed using the Mayo performance score with patients achieving a mean score of 85 postoperatively (range 65-95). All but one patient had full range of motion of the elbow. The patient with radio-ulnar synostosis had a 25 degree fixed flexion pot-operatively. Although the distal radial replacements had decreased range of movements of the wrist due to arthrodesis, they had excellent functional outcomes. Only one patient required revision surgery due to post-traumatic loosening of the implant. Our results of the use of endoprosthetic replacement of radius in the treatment of tumours are encouraging with regards to survivorship of the implant and functional outcome. This type of treatment results in an early return to daily routine activties, good functional outcome and patient satisfaction

INTRODUCTION. Bone tumour resection and subsequent reconstruction remains challenging for the surgeon. Obtaining adequate margins is mandatory to decrease the risk of local recurrence. Improving surgical margins quality without excessive resection, reducing surgical time and increasing the quality of the reconstruction are the main goals of today's research in bone tumour surgical management. With the outstanding improvements in imaging and computerised planning, it is now a standard. However, surgical accuracy is essential in orthopaedic oncologic surgery (Grimmer 2005). Patient specific instruments (PSI) may greatly improve the surgeon's ability to achieve the targeted resection. Thanks to its physical support, PSI can physically guide the blade yielding to a better control over the cutting process (Wong, 2014). Surgical time might significantly be reduced as well when compared to conventional method or navigated procedure. Finally, reconstruction may gain in rapidity and quality especially when allograft is the preferred solution as PSI can be designed as well for allograft cutting (Bellanova, 2013). Since 2011, PSI have systematically been used in our institution for bone tumour resection and when applicable allograft reconstruction. This paper reports the mid- to long-term medical outcomes on a large series. MATERIALS AND METHODS. Between 2011 and 2016, we systematically used PSI to remove bone tumours in 30 patients. The pre-operative planning involved the tumour delineation drawn on MRI by the surgeon. The MRI and obtained tumour volume were transferred to the CT-scan by image fusion (co- registration). Cutting planes were positioned around the tumour including a safe margin. The PSI were designed to ensure a sufficient stability but kept thin enough to limit the bone exposure. The PSI was manufactured by 3D-printing in a biocompatible and sterilisable material. PSI has been intraoperatively to cut the bone with predetermined margins. Medical files were reviewed for large data collection: type, size and site of the tumour, pre-and post-operative metastatic status, bone and soft tissues resection margins, local recurrence, use of an allograft and a PSI for graft adjustment or not for the reconstruction, the fusion of the allograft when applicable, the follow-up time and early/late complications. RESULTS. Over a period of 5 years, 30 patients were operated on with PSI (10 osteosarcomas, 4 chondrosarcomas, 10 Ewing sarcomas and 6 other types of bone tumours). Mean follow-up was 27±20 months. 18 cases out of 30 have more than 2 years follow-up and 13 out of 30 have more than 3 years of follow-up. Mean operating time was 6h02±3h44. Mean size of the tumours was 8,4±4,7cm and location was the upper limb in 5 cases, inferior limb in 15 cases and the pelvis in 10 occurrences. Metastatic disease developed postoperatively in 5 patients. Surgical margins in the bone were R0 in all cases but one case where a R1 surgery was planned to preserve a nerve root. We did not observe any local recurrence in the bone. Within soft tissues, margins were classified as R0 in 28 patients and R1 in 2 patients. In 26 cases, an allograft was used to reconstruct the bone defect. In 23 of those patients, the allograft was selected by CT scan and cut using a PSI. In the 3 allografts cut free-handily, only one demonstrated a fusion. Of the 23 cut with a guide, 12 fused completely, 2 demonstrated a partial fusion and 9 were not fused at the last follow-up. At the last follow-up, 2 patients were dead of disease, 5 were alive with metastatic disease and 23 were alive without disease. DISCUSSION. Oncology is probably the field where PSI can bring the largest advantage when compared to the conventional procedure. Several papers have reported the use of PSI for bone tumour resection. All of them have shown very promising results on in-vitro experiments (Cartiaux 2014), cadaver experiment (Wong 2012) or small clinical series (Bellanova 2013, Gouin, 2014). None of these papers report a large patient series associated with a clinically relevant follow-up. This series is the first mid- to long-term follow-up series involving PSI tumour surgery. These results are showing strong evidences of clinical improvements. It comes into contradiction with PSI for total knee arthroplasty where controversial results on the patient's outcome has been reported (Thienpont 2014). R0 margin has been systematically obtained for all bone cuttings, and local recurrence has been strongly decreased (3%) when compared to the usual recurrence rates published in the literature (from 15% to 35% according to the location). Allograft fusion seems improved as well thanks to the shape-matching of the selected allograft and a close contact between host and allograft at bony junctions. With a longer follow-up, these evidences should be stronger to definitely make PSI the best option for bone tumour resection

Objective. was to review the experience of the Rizzoli with megaprosthetic reconstruction of the extremities in musculoskeletal oncology. Material and methods. Between April 1983 and December 2007, 1036 modular uncemented megaprostheses of the lower limbs were implanted in 605 males and 431 females: 160 KMFTR(r), 633 HMRS(r) prostheses, 68 HMRS(r) Rotating Hinge and 175 GMRS(r). Sites: distal femur 659, proximal tibia 198, proximal femur 145, total femur 25, distal femur and proximal tibia 9. Histology showed 612 osteosarcomas, 113 chondrosarcomas, 72 Ewing's sarcoma, 31 metastatic carcinomas, 89 GCT, 36 MFH,68 other diagnoses. Between 1975 and 2006 at Rizzoli 344 reconstructions of the humerus using prosthetic devices (alone or in association with allografts) were performed: 289 MRS(r), 37 HMRS(r), 2 Osteobridge(r), 4 composite prostheses, 8 Coonrad-Morrey(r), 4 custom made prostheses. Sites of reconstruction were: proximal humerus 311, distal humerus 19, diaphysis 5, total humerus 9. Histology showed 146 osteosarcomas, 56 chondrosarcomas, 23 Ewing's sarcoma, 67 metastatic carcinomas, 14 GCT, 10 MFH, 28 other diagnoses. Patients were followed periodically in the clinic. Information were obtained from clinical charts and imaging studies with special attention to major complications requiring revision surgery. Major prostheses-related complications were analysed and functional results evaluated according to the MSTS system. Univariate analysis by Kaplan-Meier actuarial curves was used for studying implant survival to major complications. Results. Major complications causing implants failure in lower limbs were 80 infections (7.7%), 64 aseptic loosening (6.2%) and 33 breakages (3.2%). In lower limbs infection occurred in 18 KMFTR(r), 47 HMRS(r), 5 HMRS(r) Rotating Hinge, 10 GMRS(r). Breakage of the prosthetic reconstruction occurred in 16 KMFTR(r), 16 HMRS(r), 1 HMRS(r) Rotating Hinge. Aseptic loosening occurred in 15 KMFTR(r), 28 HMRS(r), 18 HMRS(r) Rotating Hinge, 3 GMRS(r). Major complications causing implants failure in upper limbs were 15 infections (4.3%), 8 aseptic loosening (2.3%) and 4 breakages (1.2%). In upper limbs infection occurred in 14 MRS(r) and 1 Coonrad-Morrey(r). Aseptic loosening in 8 cases MRS(r). Breakage in 4 cases MRS(r) prostheses. Most patients in both lower and upper extremities series showed satisfactory function (good or excellent) according to the MSTS evaluation system. Implant survival to all major complications of lower limb megaprostheses evaluated with Kaplan-Meier curve was 80% at 10 years and 60% at 20 years. Implant survival for the newer designs (GMRS(r)) available only at middle term follow up showed an implant survival to major complications at about 90% at 5 years. Implant survival to all major complications was over 80% at 10 years and 78% at 20 years. Conclusions. Megaprostheses are the most frequently used type of reconstruction after resection of the extremities, since they provide good function and a relatively low incidence of major complications. Both function and implant survival improved in the last decades with the introduction of newer designs and materials

The rate of fracture and subsequent nonunion after radiation therapy for soft-tissue sarcomas and bone tumors has been demonstrated to quite high. There is a paucity of data describing the optimal treatment for these nonunions. Free vascularized fibular grafts (FVFG) have been used successfully in the treatment of large segmental bone defects in the axial and appendicular skeleton, however, their efficacy with respect to treatment of radiated nonunions remains unclear. The purpose of the study was to assess the 1) union rate, 2) clinical outcomes, and 3) complications following FVFG for radiation-induced femoral fracture nonunions. We identified 24 patients who underwent FVFG for the treatment of radiation-induced femoral fracture nonunion between 1991 and 2015. Medical records were reviewed in order to determine oncologic diagnosis, total preoperative radiation dose, type of surgical treatment for the nonunion, clinical outcomes, and postoperative complications. There were 11 males and 13 females, with a mean age of 59 years (range, 29 – 78) and a mean follow-up duration of 61 months (range, 10 – 183 months). Three patients had a history of diabetes mellitus and three were current tobacco users at the time of FVFG. No patient was receiving chemotherapy during recovery from FVFG. Oncologic diagnoses included unspecified soft tissue sarcomas (n = 5), undifferentiated pleomorphic sarcoma (UPS) (n = 3), myxofibrosarcoma (n = 3), liposarcoma (n = 2), Ewing's sarcoma (n = 2), lymphoma (n = 2), hemangiopericytoma, leiomyosarcoma, multiple myeloma, myxoid chondrosarcoma, myxoid liposarcoma, neurofibrosarcoma, and renal cell carcinoma. Mean total radiation dose was 56.3 Gy (range, 39 – 72.5), given at a mean of 10.2 years prior to FVFG. The average FVFG length was 16.4 cm. In addition to FVFG, 13 patients underwent simultaneous autogenous iliac crest bone grafting, nine had other cancellous autografting, one received cancellous allograft, and three were treated with synthetic graft products. The FVFG was fixed as an onlay graft using lag screws in all cases, additional fixation was obtained with an intramedullary nail (n = 19), dynamic compression plate (n = 2), blade plate (n = 2), or lateral locking plate (n = 1). Nineteen (79%) fractures went on to union at a mean of 13.1 months (range, 4.8 – 28.1 months). Musculoskeletal Tumor Society scores improved from eight preoperatively to 22 at latest follow-up (p < 0.0001). Among the five fractures that failed to unite, two were converted to proximal femoral replacements (PFR), two remained stable pseudarthroses, and one was converted to a total hip arthroplasty. A 6th case did unite initially, however, subsequent failure lead to PFR. Seven patients (29%) required a second operative grafting. There were five additional complications including three infections, one wound dehiscence, and one screw fracture. No patient required amputation. Free vascularized fibular grafts are a reliable treatment option for radiation-induced pathologic femoral fracture nonunions, providing a union rate of 79%. Surgeons should remain cognizant, however, of the elevated rate of infectious complications and need for additional operative grafting procedures

Introduction. The reconstruction of the knee in growing children considers many options and the chosen solution is often patient (or surgeon) based. Megaprostheses represent a reliable solution but quite expensive in the non-invasive growing version and not free from complications. In an Italian reference center for Bone and Soft tissue sarcomas, following the experience of Rizzoli Institute in Bologna, we performed the reconstruction with a resurfaced allograft for the distal femur or the proximal tibia in selected patients. The aim of the study is to confirm the reliability of this technique and to identify its potential advantages and indications. Methods. Among 60 children below 16 years old with bone sarcomas (39 osteosarcomas, 21 Ewing's sarcomas, age range 4–16) treated since 2007, 35 cases were around the hip and the knee. 7 pediatric knees (age range 5–12 ys) with the tumor involving the epiphysis were reconstructed using a resurfaced allograft for distal femur (2) or proximal tibia (6) leaving intact the other half of the joint. Functional outcome (MSTS score), complication rate, and oncologic follow up were evaluated. Results. Oncologic follow up has been regularly conducted (range 2–9 years). No patient died of disease or developed a local recurrence. Two patients are alive with stable lung disease. Mean MSTS score was 32. No complications such as delayed union at the junction allograft-host bone, segmental deformities, fractures of the allograft, or infection have been observed. No prosthesis-related complications occurred. One limb length discrepancy with secondary scoliosis and 2 requiring a contralateral epiphysiodesis were also observed. Conclusions. In children older than 12 years old and with an expected lower limb discrepancy within 5 cm an adult megaprostheses eventually oversized is the gold standard; in children younger than 6 years old with an expected limb discrepancy longer than 10 cm the big choice is between an amputation (conventional or rotantionplasty) or a temporary reconstruction for the future implant of a growing megaprostheses. In the range 6–12 years old with an expected limb length discrepancy of 5–10 cm one of the options is the resurfaced allograft. It has been found a reliable solution in our case series with an excellent functional result probably derived from the capsule and ligaments reconstruction. Applying this protocol we observed a low mechanical-implant related complication rate. Comparing our results to Literature data of other techniques (induced membrane technique, distraction epiphysiolisis, custom-made or growing prostheses) we observed a lower reintervention rate. It is not possible to evaluate the infection rate among the different techniques used because of the low number of cases. A revision with a conventional first implant or revision total knee arthroplasty is always feasible reducing mechanical complications from megaprostheses. Further studies with longer follow up are mandatory to obtain an international consensus on reconstructive techniques in children with bone sarcomas around the knee

Non-invasive expandable prostheses for limb salvage tumour surgery were first used in 2002. These implants allow ongoing lengthening of the operated limb to maintain limb-length equality and function while avoiding unnecessary repeat surgeries and the phenomenon of anniversary operations. A large series of skeletally immature patients have been treated with these implants at the two leading orthopaedic oncology centres in England (Royal National Orthopaedic Hospital, Stanmore, and Royal Orthopaedic Hospital, Birmingham). An up to date review of these patients has been made, documenting the relevant diagnoses, sites of tumour and types of implant used. 87 patients were assessed, with an age range of 5 to 17 years and follow up range of up to 88 months. Primary diagnosis was osteosarcoma, followed by Ewing's sarcoma. We implanted distal femoral, proximal femoral, total femoral and proximal tibial prostheses. All implants involving the knee joint used a rotating hinge knee. 6 implants reached maximum length and were revised. 8 implants had issues with lengthening but only 4 of these were identified as being due to failure of the lengthening mechanism and were revised successfully. Deep infection was limited to 5% of patients. Overall satisfaction was high with the patients avoiding operative lengthening and tolerating the non-invasive lengthenings well. Combined with satisfactory survivorship and functional outcome, we commend its use in the immature population of long bone tumour cases

Developments in adjuvant therapies and surgical techniques have allowed more confident excision of the neoplastic scapula without radical margins. Total scapular excision has been proven to be an effective limb salvage procedure for tumours involving the whole scapula, with or without gleno-humeral extension. The two most common types of excision are the Tikhoff-Linberg procedure or total scapulectomy. We identified 13 patients who had undergone total scapular excision between 1995 and 2008. Eight patients underwent total scapulectomy and five underwent a Tikhoff-Linberg procedure. All reconstructions were in the form of humeral suspension. There were four females and nine males with a mean age at operation of 47.7 years (range 16-81). Most tumours excised were either Ewing's sarcoma or chondrosarcoma and mean follow-up was 44 months (7-167). Functional outcomes were assessed using the Musculoskeletal Tumor Society Score (MSTS) and the Disabilities of the Arm, Shoulder and Hand Score (DASH). Active flexion and abduction ranges were also assessed. Of the original 13 patients, five died at a mean of 21 months post-operatively. One patient developed a recurrence after five months, which was successfully excised. The mean forward flexion and abduction following all procedures was 22.5 degrees (0-30) and 22.9 degrees (0-40) respectively. There was no statistical difference between ranges of motion of total scapulectomy and Tikhoff-Linberg procedures. The mean MSTS score for the entire group was 65.8% and there was no statistical difference between total scapulectomy and Tikhoff-Linberg (p = 0.69). The mean DASH score for all patients was 39.7 with no statistically significant difference between the two procedures (p = 0.46). Both procedures allow successful excision of scapular tumours with an acceptable level of post-operative function. Total scapulectomy and Tikhoff-Linberg procedures followed by humeral suspension compare favourably with forequarter amputation, endoprosthetic reconstruction and allografting

Introduction and aims. After internal hemipelvectomy for malignant pelvic tumors, pelvic reconstruction is necessary for eventual weight bearing and ambulation. Non-vascularised, fibular grafts (NVFG) offer fast, and stable reconstruction, post- modified Enneking's type I and I/IV resection. This study aimed to evaluate the success of graft union and patient function after NVFG reconstruction. Methods. From 1996 to 2009, 10 NVFG pelvic reconstructions were performed after internal hemipelvectomy in four cases of chondrosarcoma, three of Ewing's sarcoma, and single cases of osteosarcoma, malignant peripheral nerve sheath tumour, and malignant fibrous histiocytoma. A key indication for internal hemipelvectomy was sciatic notch preservation confirmed by preoperative MRI. Operation time and complications were recorded. The mean follow-up was 31.1 months (range: 5 to 56), and lower limb function was assessed using the Musculoskeletal Tumour Society scoring system. Plain radiographs and/or computer tomography were used to determine the presence or absence of NVFG union. Results. The mean operation time was four hours and 56 mins, with no major intraoperative complications and partial wound break-down in only one patient. The NVFG united successfully in all patients, with a mean union time of 7.3 months. Post-operative deep infection did not occur in any case. Seven patients were eventually able to ambulate without a cane, and overall, the average function rating percentage was 75.4%. Recurrent tumour occurred in two cases following primary resection. Death from tumour-related disease occurred in one of the ten cases. Conclusions. NVFG is a stable and reliable pelvic reconstruction method after type I and type I/IV hemipelvectomy. Reliability may be linked to preservation of graft periosteum, and careful patient selection, limited to those with an intact sciatic notch. Furthermore, the relatively short operative time required should help minimise deep infection

Background. Endoprosthetic reconstruction is an established method of treatment for primary bone tumours in children. Traditionally these were implanted with cemented intramedullary fixation. Hydroxyapatite collars at the shoulder of the implant are now standard on all extremity endoprostheses, but older cases were implanted without collars. Uncemented intramedullary fixation with hydroxyapatite collars has also been used in an attempt to reduce the incidence of problems such as aseptic loosening. Currently there are various indications that dictate which method is used. Aims. To establish long term survivorship of cemented versus uncemented endoprosthesis in paediatric patients with primary bone tumours. Methods. This was a retrospective study of 441 endoprostheses implanted in 367 consecutive patients aged 18 years or less, between 1973 and 2005. This included the use of case notes, hospital databases and a radiological review. Information obtained included patient demographics, indications for surgery, anatomical distribution and type of implants, complications and survivorship. Results. Mean age was 13.9 (range 3 - 38). 210 patients were male, 157 were female. There were 364 primaries and 77 revision implants. 161 extendable and 280 definitive prostheses. 282 patients had osteosarcoma, 54 had Ewing's sarcoma and 28 had other diagnoses. Commonest sites included 197 distal femoral replacements, 85 proximal tibial implants and 57 were in the upper limb. Kaplan-Meier survival analysis was used to compare anatomical sites and method of fixation. Upper limb implants had the best long term survival. Failure rates for distal femoral replacements were compared for cemented fixation (21.7% due to aseptic loosening) with cement plus hydroxyapatite collars (3.1%) and uncemented implants with hydroxyapatite collars (6.2%). Conclusions. In the distal femur cemented fixation with hydroxyapatite collars gave the best survivorship in definitive primary prostheses. Uncemented fixation with hydroxyapatite collars gave the best survivorship in extendable prostheses. Cemented fixation without hydroxyapatite gave the worst survivorship

Objective. To evaluate functional and oncological outcomes following sacral resection. Methods. A retrospective review was conducted of 97 sacral tumours referred to tertiary referral spinal or oncology unit between 2004 and 2009. Results. The study included Chordoma 26; Metastases 17; Chondrosarcoma 9; Osteosarcoma 8; Lymphoma 7; Ewing's Sarcoma 6; Giant Cell Tumours 5; Other Sarcomas 5; Aneurysmal Bone Cyst 4; Myeloma 4; Others 7. There were 61 males, 37 females with an average age of 47 (range 3-82). The average duration of pre-diagnosis symptoms was 13 months. In 17 cases the diagnosis was metastatic disease and these were excluded from further discussion. Of the remainder 36/81(44%) underwent surgery: 21 excision, 9 excision and instrumented stabilisation, and 6 curettage. Thirteen (16%) patients were inoperable: 8 advanced disease, 3 unable to establish local control and 2 cases of recurrence. Colostomy was performed in 11/21 (52%) patients who underwent excision. Deep wound infections in 6/21 (29%). No difference in infection rates between definitive surgery with or without colostomy – 3/11 (27%) vs 3/10 (30%). In the instrumented group, no colostomies were performed due to concerns about deep infection and none resulted (0/9). Radiological failure of stabilisation was noted in 7/9(78%). However, functionally, 3/9 (33%) were mobilising independently, 3/9 (33%) with crutches, 2/9 (22%) able to transfer and 1/9 (11%) undocumented. Mean follow-up was 25 months (range 0-70). Local recurrence in 9/36 (25%) of operated patients. Metastasis occurred in 4/36 (11%) and mortality 8/36 (22%) although follow-up period was noted to be short. Conclusions. Results are comparable with current literature. Mechanical stabilisation for extensive sacral lesions is challenging. Despite radiological failure in 7/9 instrumented stabilisations, patients were relatively asymptomatic and only 1/9 required revision stabilisation surgery. By design none had colostomies and there were no deep infections

Objectives. To assess the affects of a delay in diagnosis on the survival rates of Bone Sarcoma (BS) using size and symptom duration as measures of delay. Methods. All patients diagnosed with a Primary BS from 1970 to 2005 were included. Demographic data concerning age, sex, diagnosis and tumour site were recorded. The data were collected retrospectively from a prospective database, with 2573 patients included. Symptom duration is defined as the length of time the patient was experiencing symptoms for until the date of diagnosis. Survival analysis was performed using Kaplan-Meier curves and Cox Regression was carried out to identify variables affecting outcome. Results. 2489 patients with Primary BS that matched out inclusion and exclusion criteria were identified. Osteosarcoma (40.4%), Chondrosarcoma (20.6%) and Ewing's Sarcoma (17.6%) were the most common diagnoses, with a median size of 10cm at presentation. Overall 5 year survival was 61.2%, extremity sited tumours carried a statistically significant better prognosis compared to axial tumours (5yr survival 63.2% vs. 53.3%) and patients with axial tumours had significantly longer symptom duration (Mann-Whitney U test p = 0.000 31 and 12 weeks). Overall analysis of all BS diagnoses showed lengthier duration of symptoms was associated with a better outcome (Hazard Ratio = 0.998, p = 0.000), which suggests that 1 extra week of symptoms confers an increased survival of 0.2%. Larger tumour size was associated with a poorer survival rate (p=0.000 to 3 sig. fig, Hazard Ratio 1.059 respectively) which suggests that for every 1cm increase in size there is a decreased survival of 5.9%. Conclusions. Although many factors have been associated with survival, the limited literature concerning symptom interval and outcome is varied. This large study has shown that regardless of diagnosis, overall, shorter symptom duration and a large tumour size are associated with a poorer prognosis

Objective. of this study was to analyse results of two stage revisions in infected megaprostheses in lower limb. Material and methods. Between April 1983 and December 2007, 1036 modular uncemented megaprostheses were implanted in 605 males and 431 females with mean age 33.5 yrs: 160 KMFTR. (r). , 633 HMRS. (r). prostheses, 68 HMRS. (r). Rotating Hinge and 175 GMRS. (r). Sites: distal femur 659, proximal tibia 198, proximal femur 145, total femur 25, distal femur and proximal tibia 9. Histology showed 612 osteosarcomas, 113 chondrosarcomas, 72 Ewing's sarcoma, 31 metastatic carcinomas, 89 GCT, 36 MFH,68 other diagnoses. Infection occurred in 80 cases (7.7%) at mean time of 4 yrs (min 1 month, max 19 yrs) in 18 KMFTR. (r). , 47 HMRS. (r). , 5 HMRS. (r). Rotating Hinge, 10 GMRS. (r). Sites: 51 distal femurs, 21 proximal tibias, 6 proximal femurs, 1 total femur and 1 extrarticular knee resection. Most frequent bacteria causing infection were: Staphilococcus Epidermidis (39 cases), Staphilococcus Aureus (17) and Pseudomonas Aeruginosa (5). Infection occurred postoperatively within 4 weeks in 9 cases, early (within 6 months) in 12 cases, late (after 6 months) in 59 cases. Usual surgical treatment was “two stage” (removal of implant, one or more cement spacers with antibiotics, new implant), with antibiotics according with coltures. One stage treatment was used for immediate postoperative infections, only since 1998. Functional results after treatment of infection were assessed using the MSTS system. Results. A two stage revision was attempted in 73 pts (91.2%): in 58 cases a new prostheses was implanted (with negative laboratory tests for infection) at mean time of 5 months (min 2, max 16 months), but in 3 pts infection recurred and they were amputated; 4 pts died before implanting a new prosthesis; 11 pts were amputated after several spacers since infection did not heal. One stage revision was performed in 4 of the 9 immediate postoperative infections, with successful results. In 3 cases an amputation was primarily performed, to proceed with chemotherapy. Revisions for infection were successful in 63 pts (79%), while 17 pts were amputated (21%). Functional results evaluated in 53 revised cases were good or excellent in 43 (81.1%). Conclusions. Two stage treatment of infected megaprostheses is successful in most cases. One stage has selected indications, mainly in postoperative immediate infections