We evaluated the risk of late relapse and further outcome in patients with soft-tissue sarcomas who were alive and event-free more than five years after initial treatment. From our database we identified 1912 patients with these pathologies treated between 1980 and 2006. Of these 1912 patients, 603 were alive and event-free more than five years after initial treatment and we retrospectively reviewed them. The mean age of this group was 48 years (4 to 94) and 340 were men. The mean follow-up was 106 months (60 to 336). Of the original cohort, 582 (97%) were alive at final follow-up. The disease-specific survival was 96.4% (95% confidence interval (CI) 94.4 to 98.3) at ten years and 92.9% (95% CI 89 to 96.8) at 15 years. The rate of late relapse was 6.3% (38 of 603). The ten- and 15-year event-free rates were 93.2% (95% CI 90.8 to 95.7) and 86.1% (95% CI 80.2 to 92.1), respectively. Multivariate analysis showed that tumour size and tumour grade remained independent predictors of events. In spite of further treatment, 19 of the 38 patients died of sarcoma. The three- and five-year survival rates after the late relapse were 56.2% (95% CI 39.5 to 73.3) and 43.2% (95% CI 24.7 to 61.7), respectively, with a median survival time of 46 months. Patients with soft-tissue sarcoma, especially if large, require long-term follow-up, especially as they have moderate potential to have their disease controlled.

Cite this article: Bone Joint J 2013;95-B:1139–43.

The aim of this study was to determine whether the level of circulating C-reactive protein (CRP) before treatment predicted overall disease-specific survival and local tumour control in patients with a sarcoma of bone.

We retrospectively reviewed 318 patients who presented with a primary sarcoma of bone between 2003 and 2010. Those who presented with metastases and/or local recurrence were excluded.

Elevated CRP levels were seen in 84 patients before treatment; these patients had a poorer disease-specific survival (57% at five years) than patients with a normal CRP (79% at five years) (p < 0.0001). They were also less likely to be free of recurrence (71% at five years) than patients with a normal CRP (79% at five years) (p = 0.04). Multivariate analysis showed the pre-operative CRP level to be an independent predictor of survival and local control. Patients with a Ewing’s sarcoma or chondrosarcoma who had an elevated CRP before their treatment started had a significantly poorer disease-specific survival than patients with a normal CRP (p = 0.02 and p < 0.0001, respectively). Patients with a conventional osteosarcoma and a raised CRP were at an increased risk of poorer local control.

We recommend that CRP levels are measured routinely in patients with a suspected sarcoma of bone as a further prognostic indicator of survival.

Cite this article: Bone Joint J 2013;95-B:411–18.

A total of 157 hindquarter amputations were carried out in our institution during the last 30 years. We have investigated the reasons why this procedure is still required and the outcome. This operation was used as treatment for 13% of all pelvic bone sarcomas. It was curative in 140 and palliative in 17, usually to relieve pain. There were 90 primary procedures (57%) with the remaining 67 following the failure of previous operations to control the disease locally. The indication for amputation in primary disease was for large tumours for which limb-salvage surgery was no longer feasible. The peri-operative mortality was 1.3% (n = 2) and major complications of wound healing or infection arose in 71 (45%) patients. The survival at five years after hindquarter amputation with the intent to cure was 45%, and at ten years 38%. Local recurrence occurred in 23 patients (15%). Phantom pain was a significant problem, and only 20% used their prosthesis regularly. Functional scores were a mean of 57%. With careful patient selection the oncological results and functional outcomes of hindquarter amputation justify its continued use.

Cite this article: Bone Joint J 2013;95-B:127–31.

The purpose of this study was to assess the outcome of 15 patients (mean age 13.6 years (7 to 25)) with a primary sarcoma of the tibial diaphysis who had undergone excision of the affected segment that was then irradiated (90 Gy) and reimplanted with an ipsilateral vascularised fibular graft within it.

The mean follow-up was 57 months (22 to 99). The mean time to full weight-bearing was 23 weeks (9 to 57) and to complete radiological union 42.1 weeks (33 to 55). Of the 15 patients, seven required a further operation, four to obtain skin cover. The mean Musculoskeletal Society Tumor Society functional score at final follow-up was 27 out of 30 once union was complete. The functional results were comparable with those of allograft reconstruction and had a similar rate of complication.

We believe this to be a satisfactory method of biological reconstruction of the tibial diaphysis in selected patients.

Amputation was once widely practised for primary bone tumours of the limbs. Yet this situation has changed with limb salvage surgery becoming increasingly popular in the last 30 years. Many different techniques are now available. These include allografts, autografts, endoprostheses and allograft-prosthesis composites. This article reviews these methods, concentrating on the functional outcomes and complications that have been reported.

Since1986 we have monitored the actual diagnosis of all cases referred to our Unit with the diagnosis of ‘possible primary malignant bone tumour’. We have excluded all patients referred with a known diagnosis of either a benign condition or known to have bone metastases. In most cases the suspected diagnosis was based on X-rays alone, sometimes supported by further imaging.

We reviewed our initial seven-year experience with a non-invasive extendible prosthesis in 34 children with primary bone tumours. The distal femur was replaced in 25 cases, total femur in five, proximal femur in one and proximal tibia in three. The mean follow-up was 44 months (15 to 86) and 27 patients (79%) remain alive. The prostheses were lengthened by an electromagnetic induction mechanism in an outpatient setting and a mean extension of 32 mm (4 to 80) was achieved without anaesthesia. There were lengthening complications in two children: failed lengthening in one and the formation of scar tissue in the other. Deep infection developed in six patients (18%) and local recurrence in three. A total of 11 patients required further surgery to the leg. Amputation was necessary in five patients (20%) and a two-stage revision in another. There were no cases of loosening, but two patients had implant breakage and required revision. The mean Musculoskeletal Tumor Society functional score was 85% (60% to 100%) at last known follow-up. These early results demonstrate that the non-invasive extendible prosthesis allows successful lengthening without surgical intervention, but the high incidence of infection is a cause for concern.

We retrospectively compared the outcome after the treatment of giant cell tumours of bone either with curettage alone or with adjuvant cementation. Between 1975 and 2008, 330 patients with a giant cell tumour were treated primarily by intralesional curettage, with 84 (25%) receiving adjuvant bone cement in the cavity. The local recurrence rate for curettage alone was 29.7% (73 of 246) compared with 14.3% (12 of 84) for curettage and cementation (p = 0.001). On multivariate analysis both the stage of disease and use of cement were independent significant factors associated with local recurrence. The use of cement was associated with a higher risk of the subsequent need for joint replacement. In patients without local recurrence, 18.1% (13 of 72) of those with cement needed a subsequent joint replacement compared to 2.3% (4 of 173) of those without cement (p = 0.001). In patients who developed local recurrence, 75.0% (9 of 12) of those with previous cementation required a joint replacement, compared with 45.2% (33 of 73) of those without cement (p = 0.044).

We aimed to identify the incidence, outcome and prognostic factors associated with spindle cell sarcomas of bone (SCSB). We studied 196 patients with a primary non-metastatic tumour treated with the intent to cure. The results were compared with those of osteosarcoma patients treated at our hospital during the same period. The overall incidence of SCSB was 7.8% of all patients with a primary bone sarcoma. The five- and ten-year survival rates were 67.0% and 60.0%, respectively, which were better than those of patients with osteosarcoma treated over the same period. All histological subtypes had similar outcomes. On univariate analysis, factors that were significantly associated with decreased survival were age > 40 years, size > 8 cm, the presence of a pathological fracture, amputation, involved margins and a poor response to pre-operative chemotherapy. Multivariate analyses showed that age > 65 years, amputation and involved margins were all statistically significant prognostic factors. Involved margins and poor response to pre-operative chemotherapy were associated with an increased risk of local recurrence. SCSB has a better prognosis than osteosarcoma when matched for age. Most prognostic factors for osteosarcoma also seem to apply to SCSB. Patients with SCSB should be treated in the same way as patients of the same age with osteosarcoma.

We investigated the eventual diagnosis in patients referred to a tertiary centre with a possible diagnosis of a primary bone malignancy.

We reviewed our database from between 1986 and 2010, during which time 5922 patients referred with a suspicious bone lesion had a confirmed diagnosis. This included bone sarcoma in 2205 patients (37%), benign bone tumour in 1309 (22%), orthopaedic conditions in 992 (17%), metastatic disease in 533 (9%), infection in 289 (5%) and haematological disease in 303 (5%). There was a similar frequency of all diagnoses at different ages except for metastatic disease. Only 0.6% of patients (17 of 2913) under the age of 35 years had metastatic disease compared with 17.1% (516 of 3009) of those over 35 years (p < 0.0001). Of the 17 patients under 35 years with metastatic disease, only four presented with an isolated lesion, had no past history of cancer and were systematically well.

Patients under the age of 35 years should have suitable focal imaging (plain radiography, CT or MRI) and simple systemic studies (blood tests and chest radiography). Reduction of the time to biopsy can be achieved by avoiding an unnecessary investigation for a primary tumour to rule out metastatic disease.

Endoprosthetic replacement of the pelvis is one of the most challenging types of limb-salvage surgery, with a high rate of complications. In an attempt to reduce this and build greater versatility into the reconstruction process, a new type of pelvic endoprosthesis was developed in 2003, based on the old McKee-Farrar prosthesis. This study reviews the outcomes in 27 patients who had an ice-cream cone pelvic prosthesis inserted at two different specialist bone tumour centres in the United Kingdom over the past six years. The indications for treatment included primary bone tumours in 19 patients and metastatic disease in two, and six implants were inserted following failure of a previous pelvic reconstruction. Most of the patients had a P2+P3 resection as classified by Enneking, and most had resection of the ilium above the sciatic notch. The mean age of the patients at operation was 49 years (13 to 81). Complications occurred in ten patients (37.0%), of which dislocation was the most common, affecting four patients (14.8%). A total of three patients (11.1%) developed a deep infection around the prosthesis but all were successfully controlled by early intervention and two patients (7.4%) developed a local recurrence, at the same time as widespread metastases appeared. In one patient the prosthesis was removed for severe pain.

This method of treatment is still associated with high morbidity, but early results are promising. Complications are diminishing with increasing experience.

We report a case of spontaneous physeal growth arrest of the distal femur in a nine-year-old child with Ewing’s sarcoma of the proximal femur treated with chemotherapy and endoprosthetic replacement. Owing to the extent of disuse osteoporosis at the time of surgery, the entire intramedullary canal up to the distal femoral physis was filled with cement. Three years later, the femur remained at its pre-operative length of 19 cm. Pre-operative calculations of further growth failed to account for the growth arrest, and the initial expandable growing prosthesis inserted has been revised to a longer one in order to address the leg-length discrepancy.

To our knowledge, this is the only reported case of distal femoral physeal growth arrest following cemented endoprosthetic replacement of the proximal femur.

The aim of this study was to identify whether there was any difference in patient, tumour, treatment or outcome characteristics between patients with skeletal or extra-skeletal Ewing’s sarcoma. We identified 300 patients with new primary Ewing’s sarcoma diagnosed between 1980 and 2005 from the centres’ local database. There were 253 (84%) with skeletal and 47 (16%) with extra-skeletal Ewing’s sarcomas. Although patients with skeletal Ewing’s were younger (mean age 16.8 years) than those with extra-skeletal Ewing’s sarcoma (mean age 27.5 years), there was little difference between the groups in terms of tumour stage or treatment. Nearly all the patients were treated with chemotherapy and most had surgery. There was no difference in the overall survival of patients with skeletal (64%) and extra-skeletal Ewing’s sarcoma (61%) (p = 0.85), and this was also the case when both groups were split by whether they had metastases or not.

This large series has shown that the oncological outcomes of Ewing’s sarcoma are related to tumour characteristics and patient age, and not determined by whether they arise in bone or soft tissue.

Introduction: Postoperative infection rates following endoprosthetic surgery are currently around 11%. In comparison with routine orthopaedic infection rates (0.5–2.0%) this is high and warrants investigation. Antibiotic prophylaxis at the time of surgery has been proven to reduce postoperative infection rates. We are looking to see if this is being administered correctly.

Methods: We sought the records of 183 patients undergoing primary EPRs at the ROH within an 18 month period. We were unable to locate 57 (69%) leaving 126 to analyse. We recorded details of the surgeon conducting the operation, the perioperative antibiotics administered, and whether the patient developed a SSI postoperatively. We grouped the patients into three groups, those who received the correct regime as defined by the department (1.5g Cefuroxime at induction followed by 3 doses of 750mg Cefuroxime at 8, 16 and 24 hours post-op), those who received cefuroxime but in an incorrect regime and those not receiving cefuroxime. We further looked at the frequency individual surgeons were administering the correct antibiotics. Finally we applied a Chi-squared anaylsis to see if there was a significant difference in infection rates between those receiving the correct antibiotics and those not.

Results: 52 patients (41%) received cefuroxime in the correct regime, 27 (22%) received cefuroxime in an incorrect regime and 47 patients (37%) did not receive cefuroxime. The different surgeons at the hospital administered the correct regime in varying frequencies (0%–65%). Giving the correct antibiotic dose is associated with a reduction in the incidence of postoperative SSI (0.1> p> 0.05).

Discussion: A large proportion of EPRs are carried out without the recommended prophylactic antibiotic cover. The rate of correct antibiotic administration varies widely between surgeons from the same department. This lack of appropriate prescribing could be contributing to a high rate of postoperative infection and therefore needs addressing.

Background: And Aims Pathological fractures of the proximal femur due to primary bone sarcomas are difficult to treat. The aim of the study was to assess the factors determining the outcomes following pathological fractures of the proximal femur due to primary bone sarcomas.

Methods: 93 patients with a pathological fracture of the proximal femur due to primary bone sarcomas were studied. The patient, tumour and treatment factors in relation to overall survival were analysed.

Results: There were 55 male and 38 female patients. The mean age was 47 years. The diagnoses were Chondrosarcoma -34, Osteosarcoma – 21, spindle cell sarcoma – 25, Ewing’s sarcoma -13. 74 patients had a pathological fracture at diagnosis and 19 patients had a fracture after the diagnosis. 17 patients had metastases at diagnosis. 24 patients had an intracapsular fracture. Limb salvage was possible in 60 patients (65%), 18 patients had an amputation and 15 patients had palliative treatment. 27% of the patients were referred after an unplanned surgery. The mean follow up was 49 months [range 0–302]. Twenty one patients [23%] had a local recurrence -10 patients had a diagnosis of chondrosarcoma, four patients had osteosarcoma and seven had spindle cell sarcoma. The overall five years survival was 37% [Ewing’s sarcoma 60%, Chondrosarcoma 57%, spindle cell sarcoma 28%, osteosarcoma 13% and dedifferentiated Chondrosarcoma 0% (p-0.002)]. Metastasis at diagnosis was a significant factor (p-0.04) affecting survival.

Conclusion: We conclude that a pathological fracture of the proximal femur due to osteosarcoma and dedifferentiated chondrosarcoma. carry a poor prognosis.

Of 3000 patients diagnosed with primary malignant bone tumours and treated at our unit over the past 25 years, 234 (7.8%) were considered to be spindle cell sarcomas of bone (ie not osteosarcoma, chondrosarcoma, Ewing’s, chordoma or adamantinoma). We have analyzed their management and outcomes.

The diagnosis of these cases varied with fluctuations in the popularity of conditions such as MFH, fibrosarcoma and leiomyosarcoma with the passage of time. Treatment was with chemotherapy and surgery whenever possible. 36 patients had metastases at diagnosis and 17 had palliative treatment only because of age or infirmity. The most common site was the femur followed by the tibia, pelvis and humerus. The mean age was 45 and the mean tumour size 10.2cm at diagnosis. 25% of patients presented with a pathological fracture. Chemotherapy was used in 70% of patients the most common regime being cisplatin and doxorubicin. 35% of patients having neoadjuvant chemotherapy had a good (> 90% necrosis) response. The amputation rate was 22% and was higher in patients presenting with a fracture and in older patients not having chemotherapy.

With a mean follow up of 8 years the overall survival was 64% at 5 yrs and 58% at 10 yrs. Adverse prognostic factors included the need for amputation, older age and poor response to chemotherapy as well as a pathological fracture at presentation. The few patients with angiosarcoma fared badly but there was no difference in outcomes between patients with other diagnoses.

We conclude that patients with spindle cell sarcomas should be treated similarly to patients with osteosarcoma and can expect comparable outcomes. The histological diagnosis does not appear to predict behaviour.

The two week wait has been established as a potential means of diagnosing malignant tumors earlier and thus hopefully leading to improvements in outcome. There remains controversy as to whether these clinic achieve this end or whether they just speed up treatment of patients already diagnosed (eg by imaging)

Aim: The aim of this study is to evaluate the diagnoses of all patient referred with a suspicious soft tissue lump to a two week wait clinic, to assess the diagnostic ‘hit rate’ and how many of these had already had imaging leading to a possible diagnosis of malignancy.

Method: Review of the diagnoses and referral criteria for all patients referred to a soft tissue sarcoma early diagnosis clinic.

Results: ** patients were referred under the 2 week wait criteria with a possible soft tissue sarcoma. ** (**%) turned out to have a malignant diagnosis. Of these, *8 were STS, ** were other soft tissue malignancies and ** were bone malignancices (** being….) Of the patients with a malignant diagnosis, only ** were referred directly to this hospital as a two week wait without previous investigation. Of the remainder *8 had undergone imag-9ing prior to referral leading to a suspicion of malignancy and ** were referred after investigation at another hospital. The average size of STS diagnosed after 2 week wait referral was ** which compared with **cm in those not referred via this route over the same time period.

Conclusion: Patients referred to a soft tissue 2 week wait clinic had a **% risk of malignancy. Of these, ** were new diagnoses suspected purely on clinical as opposed to imaging grounds.

Endoprosthetic replacement of the pelvis is one of the most challenging types of limb salvage surgery with a high rate of complications. In order to try and decrease the high risk of complications and to allow greater versatility in the reconstruction options, a new concept of pelvic endoprosthesis was developed in 2003. Since then 20 of these ice cream cone pelvic prostheses have been inserted at our centre incorporating antibiotic laden cement around the prosthesis to minimize infection risk.

Aim: To review the outcomes of the ice cream cone prostheses and to learn lessons from this.

Method: retrospective review of records and Xrays of patients having an ice cream cone type prosthesis at our centre.

Results: 20 ice cream cone prostheses were inserted in the past 5 years. Six of the implants were inserted following failure of a previous pelvic reconstruction (one for hydatid disease, one following a excision arthroplasty for chondrosarcoma, three following failed pelvic EPRs). Of the primary tumours, there were 9 chondrosarcomas, 2 Ewings, one each of osteosarcoma, epithelioid sarcoma and GCT. All of the patients had at the least had a P2+P3 resection with most having resection of the ilium above the sciatic notch. The average age of patients at operation was 50.5yrs [range 13–81yrs]. Ten patients (50%) had one or more complication following surgery, of which dislocation was the most common, affecting 5 patients (25%), of whom two have permanent dislocations. Four patients (20%) developed a deep infection of the prosthesis but all had this controlled with early intervention. Two patients (15%) developed a local recurrence, both at the time of widespread metastases. Only one patient has had the prosthesis removed, for severe pain. There were six deaths, four due to metastatic disease and two from cardiovascular complications. The complication was significantly lower for Surgeon A (who did 15 of the 20 procedures).

Conclusion: This method of treatment is still associated with high morbidity but early results are promising. Complications are much lower with increasing experience.

Introduction: Malignant tumours of the foot and ankle are rare. The aim of the study was to document one of the largest series of malignant tumours affecting the foot and ankle and to assess the outcomes following limb salvage and amputation.

Methods: The study was a retrospective review of the patients with a malignant tumour of the foot and ankle. Demographic details, diagnosis, treatment and outcomes were retrieved from the electronic patient records containing information on over 20 000 patients seen over a 25 year period.

Results: Two hundred and twenty five patients had malignant tumours affecting the foot and ankle. It was common in the fifth decade (35 patients). The mean age was 46 years. The commonest diagnosis was synovial sarcoma (40 patients) followed by chondrosarcoma (23 patients) and Ewing’s sarcoma (21 patients). The mean tumour size was 5.6 cm (0.8 to 17.5 cm). 82 patients (37%) underwent an unplanned excision and 13% (29 patients) presented with metastases at diagnosis. Primary bone tumours were 28% (64 patients), soft tissue sarcomas were 62% and metastatic tumours were 8% while lymphoreticular malignancies were (1%). Limb salvage was possible in 71% (156 patients). 29% (65 patients) had a below knee amputation. 7% (15 patients) had a local recurrence. The 5 years survival was 63%. The 5 years survival for the patients who had limb salvage was 68% compared with 54% for the patients who had an amputation (p 0.03).

Conclusion: Though amputation can provide better local control, limb salvage surgery improves survival.