We have analysed the pattern of symptoms in patients presenting with synovial sarcoma to identify factors which led to long delays in diagnosis. In 35 children, the early symptoms and the results of clinical and radiological investigation were reviewed, along with the presumed diagnoses. The duration of symptoms was separated into patient delay and doctor delay. Only half of the patients had one or more of the four clinical findings suggestive of sarcoma according to the guidance of the National Institute for Clinical Excellence at the onset of symptoms. Of the 33 children for whom data were available, 16 (48.5%) presented with a painless mass and in ten (30.3%) no mass was identified. Seven (21.2%) had an unexplained joint contracture. Many had been extensively investigated unsuccessfully. The mean duration of symptoms was 98 weeks (2 to 364), the mean patient delay was 43 weeks (0 to 156) and the mean doctor delay was 50 weeks (0 to 362). The mean number of doctors seen before referral was three (1 to 6) and for 15 patients the diagnosis was obtained after unplanned excision. Tumours around the knee and elbow were associated with a longer duration of symptoms and longer doctor delay compared with those at other sites. Delays did not improve significantly over the period of our study of 21 years, and we were unable to show that delay in diagnosis led to a worse prognosis. Our findings highlight the variety of symptoms associated with synovial sarcoma and encourage greater awareness of this tumour as a potential diagnosis in childhood

Aims

Exosomes derived from bone marrow mesenchymal stem cells (BMSCs) have been reported to be a promising cellular therapeutic approach for various human diseases. The current study aimed to investigate the mechanism of BMSC-derived exosomes carrying microRNA (miR)-136-5p in fracture healing.

We report the results of limb salvage for non-metastatic osteosarcoma of the distal tibia using resection arthrodesis, autogenous fibular graft and fixation by an Ilizarov external fixator. In six patients with primary osteosarcoma of the distal tibia who refused amputation, treatment with wide en bloc resection and tibiotalar arthrodesis was undertaken. The defect was reconstructed using non-vascularised free autogenous fibular strut graft in three patients and a vascularised pedicular fibular graft in three, all supplemented with iliac cancellous graft at the graft-host junction. An Ilizarov external fixator was used for stabilisation of the reconstruction. In five patients sound fusion occurred at a mean of 13.2 months (8 to 20) with no evidence of local recurrence or deep infection at final follow-up. The mean post-operative functional score was 70% (63% to 73%) according to the Musculoskeletal Tumour Society scoring system. All five patients showed graft hypertrophy. Union of the graft was faster in cases reconstructed by vascularised fibular grafts. One patient who had a poor response to pre-operative chemotherapy developed local tumour recurrence at one year post-operatively and required subsequent amputation

1. An analytical study of eighty histologically proven cases of Paget's sarcoma confirms and elaborates existing knowledge of the etiology of this tumour. The frequency with which this tumour occurs in certain parts of England may be seen by comparison with the numbers of osteosarcoma and fibrosarcoma cases registered at Bristol and Leeds. An overall incidence figure for Paget's sarcoma of 0·l6 and 0·18 per 100,000 population per annum has been calculated for the Bristol and Leeds areas respectively. The similarity of the age incidence curves of Paget's disease and Paget's sarcoma are shown and discussed in the light of demographic information. 2. Histologically, most Paget's sarcomata resemble recognised tumour types as seen in otherwise normal bones, the most frequent forms being osteosarcoma and fibrosarcoma. The type of tumour arising in an osteitic bone is uninfluenced by age, sex or site of origin. Thirty-eight per cent of the cases were complicated by fracture, which occurred with slightly greater frequency in fibrosarcoma and with advancing age. A causal connection between sarcoma and fracture is supported but not vice versa. 3. Survival statisticsfor this series are compared with Bristol records for osteosarcoma and fibrosarcoma (without Paget's disease), the mean survival of seventy-four patients being 11·1 months from the date of the initial symptoms of malignancy. Four long-term survivors are reported, the five-year and ten-year survival rates being respectively 5 per cent and 3·1 per cent. The effects of age, site, sex, fracture and treatment upon survival are tabulated and discussed

The incidence of metastatic osteosarcoma is increasing because of improved results following multi-agent chemotherapy and resection of the primary tumour. Metastases occur most commonly in the lungs, whereas bowel metastases are rare. We describe a 25-year-old female who presented with melaena six years after successful resection of an osteosarcoma of her right femur, and one year after resection of a solitary pulmonary metastasis. Imaging revealed a lesion arising within both the duodenum and the pancreas for which a Whipple’s pancreatoduodenectomy was carried out, achieving complete resection. Histological examination confirmed the diagnosis of metastatic osteosarcoma. We believe this is only the second such case reported. At 11 months post-operatively she had no detectable disease. Although rare, osteosarcoma can metastasise to the intestine. The surgeon must be aware of this complication, and that bowel metastases are potentially resectable

We have investigated whether improvements in design have altered the outcome for patients undergoing endoprosthetic replacement of the proximal tibia following resection of a tumour. Survival of the implant and ‘servicing’ procedures have been documented using a prospective database. A total of 194 patients underwent a proximal tibial replacement, with 95 having a fixed-hinge design and 99 a rotating-hinge with a hydroxyapatite collar; their median age was 21.5 years (10 to 74). At a mean follow-up of 14.7 years (5 to 29), 115 patients remain alive. The risk of revision for any reason in the fixed-hinge group was 32% at five years, 61% at ten years and 75% at 15 and 20 years, and in the rotating-hinge group 12% at five years, 25% at ten years and 30% at 15 years. Aseptic loosening was the most common reason for revision in the fixed-hinge knees, fracture of the implant in the early design of rotating hinges and infection in the current version. The risk of revision for aseptic loosening in the fixed-hinge knees was 46% at ten years. This was reduced to 3% in the rotating-hinge knee with a hydroxyapatite collar. The cemented, rotating hinge design currently offers the best chance of long-term survival of the prosthesis

Aims

One-stage revision hip arthroplasty for periprosthetic joint infection (PJI) has several advantages; however, resection of the proximal femur might be necessary to achieve higher success rates. We investigated the risk factors for resection and re-revisions, and assessed complications and subsequent re-revisions.

Aims

Poly (ADP-ribose) polymerase (PARP) inhibitor has been reported to attenuate inflammatory response in rat models of inflammation. This study was designed to investigate the effect of PARP signalling in osteoarthritis (OA) cartilage inflammatory response in an OA rat model.

Between 1966 and 2001, 1254 patients underwent excision of a bone tumour with endoprosthetic replacement. All patients who had radiotherapy were identified. Their clinical details were retrieved from their records. A total of 63 patients (5%) had received adjunctive radiotherapy, 29 pre-operatively and 34 post-operatively. The mean post-operative Musculoskeletal Tumor Society scores of irradiated patients were significantly lower (log-rank test, p = 0.009). The infection rate in the group who had not been irradiated was 9.8% (117 of 1191), compared with 20.7% (6 of 29) in those who had pre-operative radiotherapy and 35.3% (12 of 34) in those who radiotherapy post-operatively. The infection-free survival rate at ten years was 85.5% for patients without radiotherapy, 74.1% for those who had pre-operative radiotherapy and 44.8% for those who had post-operative radiotherapy (log-rank test, p < 0.001). The ten-year limb salvage rate was 89% for those who did not have radiotherapy and 76% for those who did (log-rank test, p = 0.02). Radiotherapy increased the risk of revision (log-rank test, p = 0.015). A total of ten amputations were necessary to control infection, of which nine were successful. Radiotherapy may be necessary for the treatment of a bone sarcoma but increases the risk of deep infection for which amputation may be the only solution

We evaluated the long-term fixation of 64 press-fit cemented stems of constrained total knee prostheses in 32 young patients with primary malignant bone tumours. Initial stable fixation, especially in rotation, was achieved by precise fit of the stem into the reamed endosteum, before cementation. Complementary fixation, especially in migration and rotation, was obtained by pressurised antibiotic-loaded cement. The mean age at operation was 33 years (13 to 61). No patient was lost to follow-up; 13 patients died and the 19 survivors were examined at a mean follow-up of 12.5 years (4 to 21). Standard revision press-fit cemented stems were used on the side of the joint which was not involved with tumour (26 tibial and six femoral), on this side there was no loosening or osteolysis and stem survival was 100%. On the reconstruction side, custom-made press-fit stems were used and the survival rate, with any cause for revision as an end point, was 88%, but 97% for loosening or osteolysis. This longevity is similar to that achieved at 20 years with the Charnley-Kerboull primary total hip replacement with press-fit cemented femoral components. We recommend this type of fixation when extensive reconstruction of the knee is required. It may also be suitable for older patients requiring revision of a total knee replacement or in difficult situations such as severe deformity and complex articular fractures

We studied 55 patients with stage-IIB osteosarcoma around the knee with respect to the expression of matrix metalloproteinase (MMP)-9 in the surviving tumour cells in surgical resection specimens. They were followed up for a minimum of 2.5 years. Factors significantly associated with poor overall survival were a high serum level of alkaline phosphatase at diagnosis and tumour cells expressing MMP-9 in the resection specimens. The only factor strongly associated with disease-free survival was the immunohistochemical status of tumour cells for MMP-9 in the resection specimens. The percentage of necrosis after chemotherapy failed marginally to reach statistical significance. On Cox regression analysis only MMP-9 remained significant for overall and disease-free survival

The occurrence of an osteosarcoma at the site of a cobalt-chrome total hip replacement is described, and the possibility of the tumour arising as a result of the liberation of cobalt particles is discussed. The experimental and clinical evidence relating tumour formation to the presence of particulate metals, and to the presence of solid and particulate polyethylene, is presented. It is considered that the risk of tumour formation at the site of any total hip replacement is very small

Bone allografts can be used in any kind of surgery involving bone from minor defects to major bone loss after tumour resection. This review describes the various types of bone grafts and the current knowledge on bone allografts, from procurement and preparation to implantation. The surgical conditions for optimising the incorporation of bone are outlined, and surgeon expectations from a bone allograft discussed

1. Clinical and histological features in ten patients with smooth muscle tumours of the limbs are described. 2. Severe pain and tenderness and the radiographic demonstration of calcification are useful pointers to the diagnosis. 3. Local recurrence of the tumour occurred in four patients despite apparently adequate excision. In three of these the tumours were shown histologically to be benign

Teratomas in the spinal cord are rare. We report a case in a 54-year-old man. CT scans revealed tumours of different densities within the spinal canal; this heterogeneity may help to differentiate teratoma from other spinal cord tumours. After resection of the tumours under microscopy, the result was excellent. Histological examination showed a variety of tissues, including elements of all three germinal layers

Aims

Acquired heterotopic ossification (HO) is a debilitating disease characterized by abnormal extraskeletal bone formation within soft-tissues after injury. The exact pathogenesis of HO remains unknown. It was reported that BRD4 may contribute to osteoblastic differentiation. The current study aims to determine the role of BRD4 in the pathogenesis of HO and whether it could be a potential target for HO therapy.

Aims

To evaluate the effect of ultrasound-targeted simvastatin-loaded microbubble destruction (UTMD_SV) for alleviation of the progression of osteoarthritis (OA) in rabbits through modulation of the peroxisome proliferator-activated receptor (PPARγ).

We report the results of vertebral column resection (VCR) for paediatric patients with spinal deformity. A total of 49 VCRs in paediatric patients from four university hospitals between 2005 and 2009 with a minimum two-year follow-up were retrospectively identified. After excluding single hemivertebral resections (n = 25) and VCRs performed for patients with myelomeningocele (n = 6), as well as spondylectomies performed for tumour (n = 4), there were 14 patients who had undergone full VCR at a mean age of 12.3 years (6.5 to 17.9). The aetiology was congenital scoliosis in five, neuromuscular scoliosis in three, congenital kyphosis in two, global kyphosis in two, adolescent idiopathic scoliosis in one and secondary scoliosis in one. A total of seven anteroposterior and seven posterolateral approaches were used. The mean major curve deformity was 86° (67° to 120°) pre-operatively and 37° (17° to 80°) at the two-year follow-up; correction was a mean of 54% (18% to 86%) in the anteroposterior and 60% (41% to 70%) in the posterolateral group at the two-year follow-up (p = 0.53). The mean Scoliosis Research Society-24 total scores were 100 (92 to 108) for the anteroposterior and 102 (95 to 105) for the posterolateral group. There was one paraparesis in the anteroposterior group necessitating urgent re-decompression, with a full recovery. Patients undergoing VCR are highly satisfied after a successful procedure