Low grade chondrosarcoma is currently followed up with the same schedule as all other chondrosarcoma patients. When treated adequately, low grade chondrosarcoma appears to have a very benign follow up course. Patients and Methods:. A retrospective study of follow ups was done on fifty six patients treated for chondrosarcoma at the Pretoria musculoskeletal tumour unit from 1987–2009, evaluating the outcome and presence of local recurrence. The lesions were divided into four histological grades; atypical enchondroma (grade 0), low grade chondrosarcoma (grade 1), intermediate grade chondrosarcoma (grade 2), and high grade chondrosarcoma (grade3). Results:. Biopsy revealed forty-six patients with low grade chondrosarcoma (82%), nine cases of grade 2 chondrosarcoma (16%), and one patient with a metastatic mesenchymal chondrosarcoma. The femur and humerus were the commonest sites involved, while the axial skeleton was only affected in three cases, all of whom had more aggressive lesions. Forty-nine patients had no recurrence after a mean period of 57.5 months follow up. Forty- two of those had low grade lesions, and seven patients had grade 2 lesions. Two patients with low grade lesions were lost to follow up. Three patients involved in the study presented with local recurrence, but no metastases were detected radiographically. Two patients died, one with metastatic mesenchymal chondrosarcoma involving the scapula, and the other with a grade 2 lesion of the pelvis. Conclusion:. We believe that adequately treated low grade chondrosarcomas have a very low local recurrence rate. When faced with recurrence, it rarely presents with distant metastases. A revised schedule of visits at 3 and 6 months followed by yearly visits for five years appears to be adequate for timely detection and treatment of any local recurrence

Chondrosarcoma responds poorly to adjuvant therapy and therefore, new targeted therapy is required. Animal models have been utilised to test therapeutic candidates, however clinically relevant, orthotopic models are lacking. The aim of this study was to develop such a model. In vitro: two human chondrosarcoma cell lines, JJ012 and FS090, were compared with respect to proliferation, colony formation, invasion, MMP-2 and MMP-9 secretion, osteoclastogenesis, endothelial tube stimulation, and expression of the angiogenic factor VEGF, and the anti-angiogenic factor RECK on western blotting. In vivo: 20,000 cells (JJ012 or FS090) were injected either into the intramedullary canal of the mouse tibia (n=5 for each cell line), or into the tibial periosteum (n=5 for each cell line). Animals were measured, and x-rayed weekly. Once euthanised, tibias and lungs were preserved, embedded and sectioned to determine the presence of tumour and lung metastases. In vitro: compared with FS090, JJ012 demonstrated significantly higher proliferative capacity at both day two and day four (p=0.017, and p=0.01). JJ012 had a significantly greater ability to invade Matrigel with an average number of 812.5 invading cells, versus 140.8 FS090 cells (p=0.0005). JJ012 readily formed colonies in collagen I, while FS090 formed none. JJ012 conditioned medium stimulated endothelial tube formation and osteoclastogenesis with a greater potency than FS090 conditioned medium. In vivo: tumours formed in the intratibial and periosteal groups injected with JJ012, whilst no mice injected with FS090 cells developed discernable tumours on physical inspection, caliper measurement or histological section. Periosteal tumours grew to three times the non-injected limb size by seven weeks, whereas intratibial injected limbs required 10 weeks to achieve the same extent of tumour growth. All JJ012 periosteal tumours resulted in lung micrometastases, while only 2/4 JJ012 intratibial tumours demonstrated metastases. Lung metastases stained positive with Von Kossa and alizarin red stains, indicating a tendency for calcification, which is similar to metastases in the human disease. Sectioned tumour tissue demonstrated features of grade II-III chondrosarcoma. Similarities with the human disease were also noted on the X-ray, including endosteal scalloping, and cortical thickening. Both intratibial and periosteal JJ012 models replicate the site, morphology, and many behavioural characteristics of human chondrosarcoma. Local tumour invasion of bone and spontaneous lung metastasis offer valuable assessment tools to test the potential of novel agents for future chondrosarcoma therapy

Chondrosarcoma is a malignant tumour and accounts for approximately 20% of bone sarcomas. The pelvis is one of the commonest sites. Chondrosarcoma of the pelvis lends itself to surgical excision and is relatively resistant to irradiation and chemotherapy. A long term survival analysis of this challenging condition is rarely reported in literature. We review and evaluate the oncological and functional results of all the patients operated at our centre and we analyse the survival analysis of these patients with special focus on the prognostic factors. Fifty-four consecutive patients with chondrosarcoma of the pelvis who were treated at the Royal National Orthopaedic Hospital, Stanmore, UK between 1987 and 2001 were included in the study. Demographic data, case notes, histopathological results and follow-up data were obtained and statistically analysed. There were 38 males and 16 females with a mean age of 48.4 years [18-77]. The chondrosarcomas were primary [n=38], secondary [n-7] or recurrences [n=9]. The anatomical sites in the pelvis were in the epicentre I [n=24], II [n=20] and III [n=10]. The surgical procedures performed were local resection [n=28], local resection and hip arthroplasty [n=6], hemipelvectomy (+endoprothesis) [n=16], hemipelvectomy [+fibular strut graf] [n=2] and hinquarter amputation [n=2]. The histological grade was Gr [n=27], Gr 2[n=20] and Gr 3 [n=7]. The complication rate was 24%:wound revision [9%], dislocation [8%] and infection [7%]. There was a 5, 10 and 15 year cumulative survival rate of 74%, 65% and 40%. The overall recurrence rate was 24%. The factors associated with a worse prognosis were high histologic tumour grade, increasing patient age, anatomical location in site I and III, primary surgery outside of tumour centre, inadequate surgical margins, and those treated by local extension. Aggressive surgical approach significantly improves the prognosis of the patients with chondrosarcoma of the pelvis

Dedifferentiated chondrosarcoma is a rare but highly malignant manifestation that can occasionally arise in patients with cartilage tumours. There remains uncertainty as to the best treatment for this condition and in particular whether chemotherapy may have a role in improving prognosis. Members of EMSOS were invited to contribute data on patients, tumours, treatment and outcomes of patients with dedifferentiated chondrosarcoma. Eight centres contributed data on 317 patients from 7 countries. The mean age was 59 (range 15 to 89) and the most common site was the femur (46%) followed by the pelvis (28%). 25% of patients presented with a pathological fracture and the most common high grade component identified was MFH. 23% had metastases at diagnosis and these patients had a median survival of 5 months. 30% of patients received chemotherapy, with 47% under 60 having chemotherapy compared with 10% over 60. One third of this group had neoadjuvant chemotherapy and the rest had adjuvant reatment. 88% had surgery with limb salvage in 80% of this group. The overall survival was 38% at 2 years and 24% at 5 years but in patients without metastases at diagnosis these figures were 44% and 28% respectively. Poor prognostic factors for survival were: metastases at diagnosis, amputation or no operation, local recurrence, age over 60 and pathological fracture at presentation. We were unable to identify any group in whom chemotherapy appeared to have a survival benefit. Dedifferentiated chondrosarcoma carries a dismal prognosis. Although 30% of patients received chemotherapy in this study we were not able to prove that it improved survival. Early diagnosis and complete surgical excision still offer the best prognosis for this condition

Cartilage lesions vary in the spectrum from benign enchondromas to highly malignant dedifferentiated chondrosarcomas. From the treatment perspective, enchondromas are observed, Grade 1 chondrosarcomas are curetted like aggressive benign tumors, and rest are resected like other sarcomas. Although biopsy for tissue diagnosis is the gold standard for diagnosis and grade determination in chondrosarcoma, tumor heterogeneity limits the grading in patients following a biopsy. In the absence of definite pre-treatment grading, a surgeon is therefore often in a dilemma when deciding the best treatment option. Radiology has identified aggressive features and aggressiveness scores have been used to try and grade these tumors based on the imaging characteristics but there have been very few published reports with a uniform group and large number of cases to derive a consistent scoring and correlation. The authors asked these study questions :(1) Does Radiology Aggressiveness and its Score correlate with the grade of chondrosarcoma? (2) Can a cut off Radiology Agressiveness Score value be used to guide the clinician and add value to needle biopsy information in offering histological grade dependent management?. A retrospective analysis of patients with long bone extremity intraosseous primary chondrosarcomas were correlated with the final histology grade for the operated patients and Radiological parameters with 9 parameters identified a priori and from published literature (radiology aggressiveness scores - RAS) were evaluated and tabulated. 137 patients were identified and 2 patients were eliminated for prior surgical intervention. All patients had tissue diagnosis available and pre-treatment local radiology investigations (radiographs and/or CT scans and MRI scans) to define the RAS parameters. Spearman correlation has indicated that there was a significant positive association between RAS and final histology grading of long bone primary intraosseous chondrosarcomas. We expect higher RAS values will provide grading information in patients with inconclusive pre-surgery biopsy to tumor grades and aid in correct grade dependant surgical management of the lesion. Prediction of dedifferentiated chondrosarcoma from higher RAS will be attempted and a correlation to obtain a RAS cut off, although this may be challenging to achieve due to the overlap of features across the intermediate grade, high grade and dedifferentiated grades. Radiology Aggressiveness correlates with the histologic grade in long bone extremity primary chondrosarcomas and the correlation of radiology and biopsy can aid in treatment planning by guiding us towards a low-grade neoplasm which may be dealt with intralesional extended curettage or high-grade lesion which need to be resected. Standalone RAS may not solve the grading dilemma of primary long bone intraosseous chondrosarcomas as the need for tissue diagnosis for confirming atypical cartilaginous neoplasm cannot be eliminated, however in the event of a needle biopsy grade or inconclusive open biopsy it may guide us towards a correlational diagnosis along with radiology and pathology for grade based management of the chondrosarcoma

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)

Sarcomas generally metastasize to the lung, while extra-pulmonary metastases are rare. However, they may occur more frequently in certain histological sub-types. Bone metastases from bone and soft tissue sarcomas account for a significant number of extra-pulmonary disease. Resection of lung metastases is widely accepted as therapeutic option to improve the survival of oligometastatic patients but there is currently no literature supporting curative surgical management of sarcoma bone metastases. Most are treated on a case-by-case basis, following multidisciplinary tumour boards recommendations. One study reported some success in controlling bone metastases using radiofrequency ablation. Our goal was to assess the impact of curative resection of bone metastases from soft tissue and bone sarcomas on oncologic outcomes. Extensive review of literature was done to evaluate epidemiological and outcomes of bone metastases in sarcoma. We examined our prospective database for all cases of bone metastases from sarcoma treated with surgical resection between 1990 and 2016. Epidemiology, pathology, metastatic status upon diagnosis, type of secondary relapses and their treatments were recorded. Overall survival and disease-free survival were calculated and compared to literature. Thirty-five patients were included (18 men, 17 women) with a mean age of 46 years. Fifteen were soft tissue (STS) and 20 were bone (BS) sarcomas. Most STS were fibrosarcomas, leiomyosarcomas or UPS while chondrosarcomas and osteosarcomas were the most frequent BS. Nine (60%) STS were grade 3, 4 (27%) grade 2 and one grade 1 (3%). Eight (23%) were metastatic upon diagnosis (6 lungs, 3 bone). Treatment of the primary tumour included wide excision with reconstruction and (neo)-adjuvant therapies as required. Margins were negative in 32 cases and micro-positive in 3 cases. Amputation occurred in 6 (17%) cases. Primary lung metastases were treated by thoracotomy and primary bone metastases by wide excision. First relapse occurred in bone in 19 cases (54%), lungs and bone in 7 cases, 5 in lungs and 4 in soft-tissues. Lung metastases were treated by thoracotomy and chemotherapy in 3 cases, chemotherapy alone in the remaining cases. Bone metastases were treated by wide resection-reconstruction in 24 cases, extensive curettage in 4. Soft tissue relapses were re-excised in 4 patients. Two amputations were required. All margins were negative except for the 4 treated by curettage. Fourteen second relapses occurred in bone, 7 were radically-excised and 2 curetted. At last follow-up, 6 patients were alive (overall survival of 17%), with a mean survival of 57 months, a median overall survival of 42.5 months and a median disease-free survival (DFS) of 17 months. Overall survival was 17%, compared to an 11% 10-year survival previously reported in metastatic sarcomas. Median disease-free survival was better in this study, compared to 10 months in literature, so as median OS (42.5 months vs 15). Three patients were alive with no evidence of disease. DFS, OS and median survival seemed to be improved by bone metastases wide excision and even if several recurrences occur, curative surgery with adjuvant therapies should be considered

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

Extracorporeal irradiation of resected bone segments has been used for pelvic reconstruction for 12 years in our unit. The technique allows for a perfect fit, preservation of muscle attachments, and thus, facilitating reconstruction, more rapid osteotomy of autograft bone, low cost, and a low infection rate. Of particular interest is its use in children; revascularisation appears more complete with irradiated autograft, as compared to allograft. There is sparing of adjacent growth plates if the irradiation is given outside the body, whereas external beam radiation would destroy the proximal femoral growth plate—leading to significant leg length discrepancy. Avoiding external beam irradiation also removes any risk of late radiation induced sarcoma and removes the late effects of soft tissue fibrosis due to external beam irradiation. Twenty-seven pelvic reconstructions have been done over a 12-year period, with three failures due to infection and seven due to further disease. Four of these have been for de-differentiated chondrosarcoma of the pelvis, where local recurrence led to failure. In the presence of de-differentiated pelvic chondrosarcoma, we do not recommend this form of reconstruction. Long term functional outcome scores for the first 50 cases completed between August 1996 and June 2003 gave a TESS score of 82% and MSTS score of 78%. This is more than comparable to published series using other forms of reconstruction

Background. Advances in diagnosis and treatment should mean that hindquarter amputation is now rarely needed. Unfortunately this is not the case. We have performed 166 of these amputations in the past 36 years. We have investigated the reasons why this procedure is still required and the outcomes following it. Method. A retrospective review of data stored on a prospective database. Results. Hindquarter amputation was used as treatment for 15% of all primary bone tumours affecting the pelvis. 146 were performed with curative intent but 20 were performed purely for palliation, usually to relieve pain. 96 of the procedures were needed as part of primary treatment, with the other 70 being needed following failure of local control after other surgical procedures. The indication for amputation in primary disease was almost always due to a significant delay in diagnosis, allowing tumours (particularly chondrosarcomas) to become massive by the time of diagnosis. The peri-operative mortality was 3% and 45% had major wound healing problems or infection. The median survival times after curative and palliative procedures were 36 months and 8 months respectively. The survival after hindquarter amputation for curative intent at 1, 3 and 5 years was 74%, 60% and 48%. Overall survival was better with chondrosarcoma – 52% of the patients surviving more than 10 years had chondrosarcoma. Phantom pain was a significant problem; fewer than 10% use their prosthesis regularly. Despite this functional scores averaged 61% – not significantly worse than patients who had undergone pelvic replacements!. Conclusion. Hindquarter amputation is still regularly required both for primary and salvage treatment in musculoskeletal oncology. Earlier diagnosis of pelvic tumours may avoid its use. Survival is not surprisingly worse than for tumours at other sites

Introduction. Bony tumours of the foot account for approximately 3% of all osseous tumours. However, literature regarding os calcis and talar tumours comprises individual case reports, short case series or literature reviews with no recent large series. Methods. We retrospectively reviewed the medical notes and imaging for all patients with calcaneal or talar tumours recorded in the Scottish Bone Tumour Registry since the 1940's. Demographics, presentation, investigation, histology, management and outcome were reviewed. Results. 34 calcaneal tumours and 23 talar tumours were identified. Calcaneal tumours. 2:1 male prevalence, mean age at presentation 30, average length of symptoms 9 months. 4 cases presented with pathological fracture. 21 benign tumours including 6 unicameral bone cysts, 3 chondroblastoma, and a wide variety of individual lesions. 13 malignant tumours comprising 6 osteosarcoma, 5 chondrosarcoma and 2 Ewings sarcoma. Talar Tumours. male to female ratio 3:1, mean age at presentation 28, average length of symptoms 5 months. 20 benign cases including 7 osteoid osteoma, 4 chondroblastoma, and several individual lesions. 3 malignant lesions comprising 2 chondrosarcoma, 1 osteosarcoma. Discussion. Tumours of the hindfoot frequently are delayed in diagnosis due to their rarity and lack of clinician familiarity. They are more common in men, especially talar tumours, which are most commonly benign osteoid osteoma or chondroblastoma. Calcaneal tumours have 1 in 3 risk of malignancy and cover a wider variety of lesions. Osteosarcoma of the foot tends to present later than other anatomical regions. Outcome is dependant on early diagnosis, timely surgery and most importantly neo-adjuvant chemotherapy. Diagnosis is often made on plain radiograph but MRI is the gold standard. Despite their rarity clinicians should maintain a high index of suspicion as accurate and timely diagnosis is important to management and outcome

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

Biological reconstruction techniques after diaphyseal tumour resection have increased in popularity in recent years. High complication and failure rates have been reported with intercalary allografts, with recent studies questioning their role in limb-salvage surgery. We developed a technique in which large segment allografts are augmented with intramedullary cement and fixed using compression plating. The goal of this study was to evaluate the survivorship, complications and functional outcomes of these intercalary reconstructions. Forty-two patients who had reconstruction with an intercalary allograft following tumour resection between 1989 and 2010 were identified from our prospectively collected database. Allograft survival, local recurrence-free, disease-free and overall survival were assessed using the Kaplan-Meier method. Patient function was assessed using the Musculoskeletal Tumour Society (MSTS) scoring system and the Toronto Extremity Salvage Score (TESS). The 23 women and 19 men had a mean age of 33 years (14–77). The most common diagnoses were osteosarcoma (n=16) and chondrosarcoma (n=9). There were 9 humerus, 18 femur and 15 tibia reconstructions. At a mean follow-up of 95 months (5–288), 31 patients were alive without disease, 10 were dead of disease and 1 was deceased of other causes. There were 4 local recurrences and 11 patients developed metastatic disease. 5-year local recurrence free survival was 92%, 5-year disease-free survival was 70% and overall survival was 75%. Fourteen of 42 patients (33%) experienced complications: 5 wound healing complications, 4 infections, 2 non-unions, 2 fractures and 1 nerve palsy. Four allografts (9.5%) were revised for complications and 2 (5%) for local recurrence. Mean allograft survival was 85 months (4–288). Mean time to union was 8.2 (3–36) months for the proximal osteotomy site and 8.1 (3–23) months for the distal osteotomy site. The mean score for MSTS 87 was 29.4 (+/− 4.4), MSTS 93 was 83.7 (+/−14.8) and TESS was 81.6 (+/−16.9). An intercalary allograft augmented with intramedullary cement and compression plate fixation provides a reliable and durable method of reconstruction after tumour resection. Complication rates are comparable to the literature and are associated with high levels of patient function and 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

The purpose of this study is to provide a systematic review of the literature and assess outcome of our experience of Ilizarov Bone Transport in reconstruction for primary malignant tumours of bone (PMTB). A systematic review of the literature for reported cases of primary reconstruction of PMTB using distraction osteogenesis was performed. All cases of distraction osteogenesis for primary reconstruction of PMTB in our institution were reviewed. Outcome was determined from retrospective review of case notes and radiology. Patients were contacted to define final status. There are few cases of primary reconstruction of PMTB using Ilizarov method in the literature. Most reports relate to benign tumours or reconstruction of secondary deformities or non-union after tumour resection. At our institution we have treated 7 patients with bone defects resulting from excision of a PMTB. Mean age was 42.1 years (23–48). Tumours occurred in the tibia in 4 cases and the femur in 3 cases. Histologic diagnosis was chondrosarcoma in 3, malignant fibrous histiocytoma in 2, adamantinoma in 1 and malignant intraosseous nerve sheath tumour in 1. All patients were assessed through the hospital sarcoma board and shown to have isolated bone lesions without metastases. Mean bone defect after resection was 13.1 cm (10–17). Mean frame time was 13.6 months (5–23). Mean follow-up was 46 months (15–137). Complications included pin infection, docking site non-union, premature fusion of corticotomy, soft tissue infection and minor varus deformity. There was one local recurrence of tumour at five months after resection, resulting in a through hip disarticulation. The other cases remain tumour-free with united, well-aligned bones and acceptable long-term function. PMTB is rare and poses a major reconstructive dilemma. Distraction osteogenesis provides an effective method of biologic reconstruction in selected cases, and good outcomes can be achieved

Resecting bone tumours within the pelvis is highly challenging and requires good cutting accuracy to achieve sufficient margins. Computer-assisted technologies such as intraoperative navigation have been developed for pelvic bone tumour resection. Patient-specific instruments have been transposed to tumour surgery. The present study reports a series of 11 clinical cases of PSI-assisted bone tumour surgery within the pelvis, and assesses how accurately a preoperative resection strategy can be replicated intraoperatively with the PSI. The patient series consisted in 11 patients eligible for curative surgical resection of primary bone tumor of the pelvis. Eight patients had a bone sarcoma of iliac bone involving the acetabulum, two patients had a sacral tumor, and one patient had a chondrosarcoma of proximal femur with intra-articular hip extension. Resection planning was preoperatively defined including a safe margin defined by the surgeon from 3 up to 15 mm. PSI were designed using a computer-aided design software according to the desired resection strategy and produced by additive manufacturing technology. Intraoperatively, PSI were positioned freehand by the surgeon and fixed on the bone surface using K-wires. The standard surgical approach has been used for each patient. Dissection was in accordance with the routine technique. There was no additional bone exposure to position the PSI. Histopathological analysis of the resected tumor specimens was performed to evaluate the achieved resection margins. Postoperative CT were acquired and matched to the preoperative CT to assess the local control of the tumor. Two parameters were measured: achieved resection margin (minimum distance to the tumor) and location accuracy (maximum distance between achieved and planned cuttings; ISO1101 standard). PSI were quick and easy to use with a positioning onto the bone surface in less than 5 minutes for all cases. The positioning of the PSI was considered unambiguous for all patients. Histopathological analysis classified all achieved resection margins as R0 (tumor-free), except for two patients : R2 because of a morcelised tumour and R1 in soft tissues. The errors in safe margin averaged −0.8 mm (95% CI: −1.8 mm to 0.1 mm). The location accuracy of the achieved cut planes with respect to the desired cut planes averaged 2.5 mm (95% CI: 1.8 to 3.2 mm). Results in terms of safe margin or the location accuracy demonstrated how PSI enabled the surgeon to intraoperatively replicate the resection strategies with a very good cutting accuracy. These findings are consistent with the levels of bone-cutting accuracy published in the literature. PSI technology described in this study achieved clear bone margins for all patients. Longer follow-up period is required but it appears that PSI has the potential to provide clinically acceptable margins

Since 1970, 2200 primary endoprosthetic replacements have been carried out at our Unit for bone tumours. 3% were carried out in the 1970s, 21% in the 1980s, 33% in the 1990s and 43% since 2000. The most common diagnosis was osteosarcoma (839)(38%) followed by metastatic disease (18%) and chondrosarcoma (13%). The most common site was the distal femur (35%) followed by the proximal femur and proximal tibia. The least common sites were the scapula and distal radius. The mean age at operation was 35 but varied widely, being 19 in patients with osteosarcoma and 58 for those with metastases. 239 extendable prostheses were inserted in children. 50% of the patients were alive at 10yrs and 42% at 20yrs. 7% required an amputation either for local recurrence or infection. The failure rate of the prostheses varied by age and site, with pelvic and proximal tibial replacements having the highest risk of infection and proximal humeral replacements the lowest risk of any complication. The failure rates of the most common prostheses (distal femur and proximal tibia) have improved over time and are now ~ 1.5% per year. Hydroxyapatite collars have largely resolved the problem of aseptic loosening. Silver coated prostheses have been used in 48 complex cases with encouraging results in controlling infection. Functional scores averaged 80% and were the same for revision as for primary implants. Conclusion. Significant improvements of design have improved endoprosthetic replacements for tumours. Results are now fairly predictable but still not as good as primary joint replacemenrts. Infection remains the most serious challenge

Introduction. Three-dimensional preoperative planning and bone tumour resection by navigation have been used in the past ten years. According to literature this workflow increases the surgical “accuracy”. However, there are a few and not completely clear reports describing accuracy in preoperative planning and navigation. The objective of this preliminary study was to determine the accuracy of osteotomies planned and guided by navigation in pelvis tumour resection. We assume that the surgical specimen scanned and 3D reconstructed is an acceptable method to determine the accuracy qualitatively and quantitatively of a virtual planning and navigation surgical workflow. Materials and Methods. A total of four patients were evaluated between May 2010 and February 2011, age range: 6–38, 17.4 mean; 2 males and 2 females. There were 4 malignant tumours: Malignant Schwannoma (1), Ewing's tumor (1) and Chondrosarcoma (2). All anatomic regions compromised by the tumour were preoperatively CT scanned (Mutislice 64, Aquilion, Toshiba Medical Systems, Otawara, Japan). Magnetic resonance images (MRI) of the corresponding region were acquired using a 1.5-T unit (Magnetom Avanto, Siemens Medical Solutions, Erlangen, Germany). Image fusion was applied to CT and MRI studies in order to determine the bone cortex and the intra-extraosseous soft tissues tumour extension. Once the fusion was obtained osteotomies were planned, taking into account the tumour extension in a three-dimensional virtual scenario. All patients were planned for two uniplanar osteotomies (intercalary resection). The minimal margin was determined in each plane by measuring the closest distance between malignant tumour and osteotomy plane. These studies allowed the visualisation of the tumour and the application of a virtual osteotomy. The simulation was carried out by using a computer-aided design (CAD) software, Mimics (Materialise, Leuven, Belgium). Three-dimensional preoperative planning was obtained in CAD format. Next, 3D models were exported to CT data sets in Digital Imaging and Communications in Medicine (DICOM) format and uploaded to the navigation system (3D OrthoMap navigation software v1.0, Stryker Navigator, Freiburg, Germany). Using the standard navigation tools (navigated pointer, camera and infrared tracker devices applied to the patient) the surgeon was able to establish a correspondence in a computer monitor between 3D images and real bone. Once osteotomies were performed, the tumour surgical specimen obtained was CT scanned and 3D reconstructed similarly to what was done previously to surgery to CT images acquired with the preoperative protocol in patients. Results. The correlation between the osteotomies preoperatively planned and the osteotomies achieved by navigation was in a global mean of 0.73 millimeters (SD: 3.14) in a total of 6 planes evaluated. Conclusion. According to clinical relevance, this work shows an acceptable accuracy in preoperative planning and navigation. Furthermore, we demonstrate the usefulness of three-dimensional models of surgical specimens when surgeons need to determine quantitative and qualitative accuracy of 3D planning and navigation procedures

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