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Purpose: The aim of the study was to report overall survival, local recurrence and development of metastasis in a group of patients with femur chondrosarcoma that presented with or without a pathological fracture., Methods: A retrospective review was performed, and 182 patients (39 pathological fractures) that had been treated by oncologic surgery for femoral chondrosarcoma were included. The mean age of the series was 50 years (range, eight to 90) and 114 patients were male (63%). Mean follow-up was 113 months (range, three to 216). Cancer-specific overall survival, development of metastasis and local recurrence were analysed, grouping patients by grade (grade I / grade 2-3 / dedifferentiated)., Results: Disease-specific survival in the entire group of chondrosarcoma of the femur was 69% (CI95% 63-76) at 5 years. Five-year disease-specific survival in the fracture group was 49% lower than in the control group 75% (p = 0.0001). Survival of patients with grade 1 chondrosarcoma with fracture was significantly less than those without fracture (p = 0.02) but there was no difference in those with grade 2-3 (p = 0.49) and dedifferentiated tumours (p = 0.09). The local recurrence rate of the entire series was 27%. Only dedifferentiated chondrosarcomas with an associated pathological fracture had a significantly higher rate of local recurrence. There was no relationship between development of metastases and fracture., Conclusion: A pathological fracture of the femur has a negative prognostic influence in grade 1 chondrosarcoma and increases the risk of local recurrence in dedifferentiated femur chondrosarcomas.

Purpose: We report on a group of patients with tumours in the Hoffa's fat pad (HFP), their clinical presentation, histological type and treatment, including two synovial sarcomas with their clinical follow-up, which have not been described previously in the literature., Methods: We performed a retrospective review of our prospectively collected database of 25 cases of HFP tumours with at least six months follow-up., Results: The gender, age at presentation (over and under 16 years of age), clinical features, history of trauma, treatment chosen, and complications were recorded. The mean age of the patients was 32 years (three to 47). Six patients were under 16 years old. Pain was the most common symptom, present in 92% (n = 23/25). The final diagnoses included 23 (92%) benign tumours and two (8%) malignant tumours. The most common benign tumour was pigmented villonodular synovitis (PVNS) (48% n = 12). The two malignant tumours were synovial sarcomas and both presented in patients under 16 years old., Conclusions: Hoffa's fat pad tumours are an uncommon and rarely diagnosed group of lesions that can be misinterpreted as any knee pathology. Although the majority of HFP tumours are benign, malignant tumours should be considered in the differential diagnosis for the paediatric population.

Purpose: We report the outcome of intercalary resection of the femoral diaphysis and extracorporeal irradiated autologous bone graft reconstruction, without the use of vascularized fibular graft., Methods: Six patients with Ewing sarcoma of the mid-shaft femur who were treated by limb sparing tumour resection and reconstruction with extracorporeal irradiated autologous bone graft with intramedullary cement between 2002 and 2010 were studied., Results: Mean age at the time of surgery was ten years (range, four-23). The length of resected femoral bone averaged 23 cm (15-32 cm). The ratio of bone resection length to total femoral length averaged 60 % (56-66 %). The patients had been followed up for between 16 and 79 months (mean, 41 months) at the time of the study. There was no infection nor fracture in this series. Primary union of the distal and proximal osteotomy sites was achieved in three patients. Delayed union of the proximal osteotomy site occurred in one patient that was successfully treated with iliac crest bone grafting. One patient developed non-union at the distal osteotomy site which failed to heal with bone grafting and was therefore converted to endoprosthetic replacement, and another patient was converted to rotationplasty at five months post-surgery because of contaminated margins. Function was excellent in all patients with surviving re-implanted bone. Local recurrence arose in one patient., Conclusion: Our experience suggests that cement augmentation of extracorporeal irradiated and re-implanted bone autografts offer a useful method of reconstructing large femoral diaphyseal bone defects after excision of primary malignant bone tumours.

Chondroblastoma of the femoral head presents particular problems in treatment because the tumour is surrounded by articular cartilage on one side and epiphyseal plate on the other. Ten patients underwent treatment for a chondroblastoma involving the proximal femoral capital epiphysis. The patients were aged between eight and 19 years and in four the epiphysis was not yet fused. Five had curettage via a drill hole created up the femoral neck, of whom two developed local recurrence. Five had a direct approach to the chondroblastoma through the femoral neck, and there were no local recurrences in this group. Both patients with local recurrence were under 14 years of age-one was cured by a direct approach through the neck of the femur and the other by lifting a trap door of articular cartilage. Apart from one patient with a temporary leg length discrepancy, there were no other complications. We conclude that a direct approach is likely to lead to the best outcome for this rare condition.

Giant cell tumours are rare bone tumours that are characteristically benign but locally aggressive, most frequently occurring in the distal femur with pathological fractures being common. This paper investigates relationships between tumour size and cortical breach on initial X-rays and subsequent treatment. The X-rays of 54 patients with distal femoral giant cell tumours were reviewed. The volumes of the tumour, distal femur and a ratio between the two parameters were estimated. The presence of a cortical breach, discrete fracture and Campanacci grade was recorded. X-rays revealed intact cortical rim in 20 patients (37%), cortical breach in 22 patients (41%) and discrete fracture in 12 patients (22%). There was a significant difference in the ratio of tumour volume to distal femoral volume between the discrete fracture group and the cortical breach group. No significant differences in rates of local recurrence were demonstrated. Extended curettage was effective for intact and cortical breach groups; however, patients in the fracture group often required radical treatment.

We retrospectively studied 35 patients who underwent endoprosthetic reconstruction of diaphyseal bone defects after excision of primary sarcomas. The patients were treated between February 1979 and May 1999 and had more than 5 years follow-up. There were 22 males and 13 females and the median age at diagnosis was 29 (8-75) years. The bone defect measured a mean of 19 (10-27.6) cm. There were 29 femoral reconstructions, three tibial and three humeral. Cumulative overall survival for all patients was 65% at 10 years. Cumulative overall survival for prosthetic reconstruction, using revision surgery as an end point, was 63% at 10 years. Cumulative risk of failure of reconstruction, including infection, fracture, aseptic loosening, local recurrence and amputation, was 60% at 10 years. Tibial and humeral reconstructions fared less well than femoral. Endoprosthetic replacement is a useful method of reconstructing long intercalary defects, especially if situated in the femur.

Endoprosthetic replacements are commonly used for limb salvage following surgical excision of bone tumours. Advantages include initial reliability, rapid restoration of function and their ready availability. Potential long-term problems include loosening, infection and mechanical failure. Increasing problems may lead to the necessity for amputation; this paper assesses that risk. A total of 1,261 patients have undergone endoprosthetic replacements in our centre in the past 34 years, with a total of 6,507 patient years of follow up. A total of 112 patients have had subsequent amputation. The reasons for amputation were local recurrence in 71, infection in 38, mechanical failure in two and chronic pain in one. The proximal tibia had the greatest risk of amputation (n=38/245). The time to amputation varied from 2 days to 16 years, with a mean of 31 months. The risk of amputation decreased with time, although 10% took place after more than 5 years.

Below knee amputation remains the treatment of choice for most patients with aggressive tumours of the distal tibia. We report the clinical and functional outcome of limb preserving surgery and endoprosthetic reconstruction of the distal tibia and ankle joint in five patients who declined amputation. The mean age was 32 years. Two had osteosarcoma, one Ewing's sarcoma, leiomyosarcoma and Giant cell tumour. Three patients developed significant complications including local recurrence, wound dehiscence and infection, and fibula impingement. Despite these complications the patients declined amputation even in the presence of significant discomfort. Early function was excellent in all patients but deteriorated with time. The patients still maintained an Enneking Score of more than 50%. Some patients are unwilling to undergo amputation for aggressive tumours of the distal tibia. For these, excision and reconstruction with endoprosthesis allow early functional recovery but there is significant medium term morbidity and functional deterioration.