Endoprosthetic replacement of the proximal femur may be required to treat primary bone tumours or destructive metastases either with impending or established pathological fracture. Modular prostheses are available off the shelf and can be adapted to most reconstructive situations for this purpose. We have assessed the clinical and functional outcome of using the METS (Stanmore Implants Worldwide) modular tumour prosthesis to reconstruct the proximal femur in 100 consecutive patients between 2001 and 2006. We compared the results with the published series for patients managed with modular and custom-made endoprosthetic replacements for the same conditions.

There were 52 males and 48 females with a mean age of 56.3 years (16 to 84) and a mean follow-up of 24.6 months (0 to 60). In 65 patients the procedure was undertaken for metastases, in 25 for a primary bone tumour, and in ten for other malignant conditions. A total of 46 patients presented with a pathological fracture, and 19 presented with failed fixation of a previous pathological fracture. The overall patient survival was 63.6% at one year and 23.1% at five years, and was significantly better for patients with a primary bone tumour than for those with metastatic tumour (82.3% vs 53.3%, respectively at one year (p = 0.003)). There were six early dislocations of which five could be treated by closed reduction. No patient needed revision surgery for dislocation. Revision surgery was required by six (6%) patients, five for pain caused by acetabular wear and one for tumour progression. Amputation was needed in four patients for local recurrence or infection.

The estimated five-year implant survival with revision as the endpoint was 90.7%. The mean Toronto Extremity Salvage score was 61% (51% to 95%). The implant survival and complications resulting from the use of the modular system were comparable to the published series of both custom-made and other modular proximal femoral implants.

We conclude that at intermediate follow-up the modular tumour prosthesis for proximal femur replacement provides versatility, a low incidence of implant-related complications and acceptable function for patients with metastatic tumours, pathological fractures and failed fixation of the proximal femur. It also functions as well as a custom-made endoprosthetic replacement.

We treated 98 patients with peri-acetabular tumours by resection and reconstruction with a custom-made pelvic endoprosthesis. The overall survival of the patients was 67% at five years, 54% at ten years and 51% at 30 years. One or more complications occurred in 58.1% of patients (54), of which infection was the most common, affecting 30% (28 patients). The rate of local recurrence was 31% (29 patients) after a mean follow-up of 71 months (11 to 147). Dislocation occurred in 20% of patients (19). Before 1996 the rate was 40.5% (17 patients) but this was reduced to 3.9% (two patients) with the introduction of a larger femoral head. There were six cases of palsy of the femoral nerve with recovery in only two. Revision or excision arthroplasty was performed in 23.7% of patients (22), principally for uncontrolled infection or aseptic loosening. Higher rates of death, infection and revision occurred in men.

This method of treatment is still associated with high morbidity. Patients should be carefully selected and informed of this pre-operatively.

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.

Introduction: The population of the UK is getting older. Patients over the age of 80 (the older old) are increasingly presenting with musculoskeletal tumours that require major surgery. We have investigated the success or otherwise of endoprosthetic replacements after tumour excision in this population.

Methods: We looked at an oncological database to identify patients over the age of 80 who had an endprosthetic replacement after tumour excision. We reviewed the records of all patients over the age of 80 who had an endoprosthetic replacement to assess the oncological and functional outcomes of the procedure.

Results: 17 patients over the age of 80 had an endoprosthesis over the past 10 years. The main indication was for metastatic disease (9 patients) but 6 had primary malignant bone tumours. The most common site was the distal femur in 9 followed by the proximal femur in 5. Most of the patients had associated co-morbidity (12 were ASA 2 or 3). There were no perioperative deaths but 2 patients had early complications with one having a paralytic ileus and one a chest infection. The median survival of the patients was 2 years with death being due to progressive metastases in most. 3 had late complications to do with the prosthesis including one infection in a proximal tibial replacement and a late dislocation at 2 years in a proximal femoral replacement. Two patients developed local recurrence treated by local excision and radiotherapy. There were no amputations or revisions.

Conclusions: Endoprosthetic replacements have a useful role to play in the surgical management of elderly patients. Although they have significant comorbidity most do well. Functional results are less good than in the younger population but most patients regain their independence and are free of pain.

We report our experience of treating 17 patients with benign lesions of the proximal femur with non-vascularised, autologous fibular strut grafts, without osteosynthesis. The mean age of the patients at presentation was 16.5 years (5 to 33) and they were followed up for a mean of 2.9 years (0.4 to 19.5). Histological diagnoses included simple bone cyst, fibrous dysplasia, aneurysmal bone cysts and giant cell tumour. Local recurrence occurred in two patients (11.7%) and superficial wound infection, chronic hip pain and deep venous thrombosis occurred in three. Pathological fracture did not occur in any patient following the procedure.

We conclude that non-vascularised fibular strut grafts are a safe and satisfactory method of treating benign lesions of the proximal femur.

We investigated whether our policy of routine re-excision of the tumour bed after an unplanned excision of a soft-tissue sarcoma was justified.

Between April 1982 and December 2005, 2201 patients were referred to our hospital with the diagnosis of soft-tissue sarcoma, of whom 402 (18%) had undergone an unplanned excision elsewhere. A total of 363 (16.5%) were included in this study. Each patient was routinely restaged and the original histology was reviewed. Re-excision was undertaken in 316 (87%). We analysed the patient, tumour and treatment factors in relation to local control, metastasis and overall survival.

Residual tumour was found in 188 patients (59%). There was thus no residual disease in 128 patients of whom 10% (13) went on to develop a local recurrence. In 149 patients (47%), the re-excision specimen contained residual tumour, but it had been widely excised. Local recurrence occurred in 30 of these patients (20%). In 39 patients (12%), residual tumour was present in a marginal resection specimen. Of these, 46% (18) developed a local recurrence. A final positive margin in a high-grade tumour had a 60% risk of local recurrence even with post-operative radiotherapy.

Metastases developed in 24% (86). The overall survival was 77% at five years. Survival was related to the grade of the tumour and the finding of residual tumour at the time of re-excision.

We concluded that our policy of routine re-excision after unplanned excision of soft-tissue sarcoma was justified in view of the high risk of finding residual tumour.

We have investigated the oncological outcome of 63 patients with soft-tissue sarcomas of the hand managed at three major centres in the United Kingdom. There were 44 males and 19 females with a mean age of 45 years (11 to 92). The three most common diagnoses were synovial sarcoma, clear cell sarcoma and epithelioid sarcoma. Local excision was carried out in 45 patients (71%) and amputation in 18 (29%). All those treated by amputation had a wide margin of excision but this was only achieved in 58% of those treated by local excision. The risk of local recurrence was 6% in those treated by amputation compared with 42% for those who underwent attempted limb salvage. An inadequate margin of excision resulted in a 12 times greater risk of local recurrence when compared with those in whom a wide margin of excision had been achieved. We were unable to demonstrate any role for radiotherapy in decreasing the risk of local recurrence when there was an inadequate margin of excision. Patients with an inadequate margin of excision had a much higher risk of both local recurrence and metastasis than those with wide margins. The overall survival rate at five years was 87% and was related to the grade and size of the tumour and to the surgical margin.

We have shown that a clear margin of excision is essential to achieve local control of a soft-tissue sarcoma in the hand and that failure to achieve this results in a high risk of both local recurrence and metastastic disease.

Pathological fractures due to metastasis with destruction of the acetabulum and central dislocation of the hip present a difficult surgical challenge. We describe a series using a single technique in which a stable and long-lasting reconstruction was obtained using standard primary hip replacement implants augmented by strong, fully-threaded steel rods with cement and steel mesh, where required.

Between 1997 and 2006, 19 patients with a mean age of 66 years (48 to 83) were treated using a modified Harrington technique. Acetabular destruction was graded as Harrington class II in six cases and class III in 13.

Reconstruction was achieved using three 6.5 mm rods inserted through a separate incision in the iliac crest followed by augmentation with cement and a conventional cemented Charnley or Exeter primary hip replacement. There were no peri-operative deaths. At the final follow-up (mean 25 months (5 to 110)) one rod had fractured and one construct required revision. Of the 18 patients who did not require revision, 13 had died. The mean time to death was 16 months (5 to 55). The mean follow-up of the five survivors was 31 months (18 to 47). There were no cases of dislocation, deep infection or injury to a nerve, the blood vessels or the bladder.

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.

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 identified 42 patients who presented to our unit over a 27-year period with a secondary radiation-induced sarcoma of bone. We reviewed patient, tumour and treatment factors to identify those that affected outcome. The mean age of the patients at presentation was 45.6 years (10 to 84) and the mean latent interval between radiotherapy and diagnosis of the sarcoma was 17 years (4 to 50). The median dose of radiotherapy given was estimated at 50 Gy (mean 49; 20 to 66). There was no correlation between radiation dose and the time to development of a sarcoma. The pelvis was the most commonly affected site (14 patients (33%)). Breast cancer was the most common primary tumour (eight patients; 19%). Metastases were present at diagnosis of the sarcoma in nine patients (21.4%). Osteosarcoma was the most common diagnosis and occurred in 30 cases (71.4%). Treatment was by surgery and chemotherapy when indicated: 30 patients (71.4%) were treated with the intention to cure. The survival rate was 41% at five years for those treated with the intention to cure but in those treated palliatively the mean survival was only 8.8 months (2 to 22), and all had died by two years. The only factor found to be significant for survival was the ability to completely resect the tumour. Limb sarcomas had a better prognosis (66% survival at five years) than central ones (12% survival at five years) (p = 0.009).

Radiation-induced sarcoma is a rare complication of radiotherapy. Both surgical and oncological treatment is likely to be compromised by the treatment received previously by the patient.

We investigated whether improvements in design have altered the outcome for patients undergoing endoprosthetic replacement of the distal femur after resection of a tumour. Survival of the implant and ‘servicing’ procedures have been documented using a prospective database, review of the design of the implant and case records. In total, 335 patients underwent a distal femoral replacement, 162 having a fixed-hinge design and 173 a rotating-hinge. The median age of the patients was 24 years (interquartile range 17 to 48).

A total of 192 patients remained alive with a mean follow-up of 12 years (5 to 30). The risk of revision for any reason was 17% at five years, 33% at ten years and 58% at 20 years. Aseptic loosening was the main reason for revision of the fixed-hinge knees while infection and fracture of the stem were the most common for the rotating-hinge implant. The risk of revision for aseptic loosening was 35% at ten years with the fixed-hinge knee, which has, however, been replaced by the rotating-hinge knee with a hydroxyapatite collar. The overall risk of revision for any reason fell by 52% when the rotating-hinge implant was used.

Improvements in the design of distal femoral endoprostheses have significantly decreased the need for revision operations, but infection remains a serious problem. We believe that a cemented, rotating-hinge prosthesis with a hydroxyapatite collar offers the best chance of long-term survival of the prosthesis.

We have investigated the significance of the method of treatment on the oncological and functional outcomes and on the complications in 184 patients with soft-tissue sarcomas of the adductor compartment managed at three international centres. The overall survival at five years was 65% and was related to the grade at diagnosis and the size of the tumour. There was no difference in overall survival between the three centres. There was, however, a significant difference in local control with a rate of 28% in Centre 1 compared with 10% in Centre 2 and 5% in Centre 3.

The overall mean functional score using the Toronto Extremity Salvage Score in 70 patients was 77% but was significantly worse in patients with wound complications or high-grade tumours. The scores were not affected by the timing of radiotherapy or the use of muscle flaps.

This large series of soft-tissue sarcomas of the adductor compartment has shown that factors influencing survival do not vary across the international boundaries studied, but that methods of treatment affect complications, local recurrence and function.

The Control of Infection Committee at a specialist orthopaedic hospital prospectively collected data on all episodes of bacteriologically-proven deep infection arising after primary hip and knee replacements over a 15-year period from 1987 to 2001.

There were 10 735 patients who underwent primary hip or knee replacement. In 34 of 5947 hip replacements (0.57%) and 41 of 4788 knee replacements (0.86%) a deep infection developed. The most common infecting micro-organism was coagulase-negative staphylococcus, followed by Staphylococcus aureus, enterococci and streptococci. Of the infecting organisms, 72% were sensitive to routine prophylactic antimicrobial agents.

Of the infections, 29% (22) arose in the first three months following surgery, 35% between three months and one year (26), and 36% (27) after one year. Most cases were detected early and treated aggressively, with eradication of the infection in 96% (72). There was no significant change in the infection rate or type of infecting micro-organism over the course of this study.

These results set a benchmark, and importantly emphasise that only 64% of peri-prosthetic infections arise within one year of surgery. These results also illustrate the advantages of conducting joint replacement surgery in the isolation of a specialist hospital.

We undertook this retrospective study to determine the rate of recurrence and functional outcome after intralesional curettage for chondroblastoma of bone. The factors associated with aggressive behaviour of the tumour were also analysed. We reviewed 53 patients with histologically-proven chondroblastoma who were treated by intralesional curettage in our unit between 1974 and 2000. They were followed up for at least two years to a maximum of 27 years.

Seven (13.2%) had a histologically-proven local recurrence. Three underwent a second intralesional curettage and had no further recurrence. Two had endoprosthetic replacement of the proximal humerus and two underwent below-knee amputation after aggressive local recurrence. One patient had the rare malignant metastatic chondroblastoma and eventually died. The mean Musculoskeletal Tumour Society functional score of the survivors was 94.2%.

We conclude that meticulous intralesional curettage alone can achieve low rates of local recurrence and excellent long-term function.

A case of pencil-gore granuloma occurring 45 years after the initial injury is described. The clinical and radiological presentation was suggestive of a soft-tissue sarcoma. The diagnosis was confirmed by biopsy, which also revealed foreign body fragments which, on spectometric analysis, had high concentrations of carbon and aluminium.

We studied 100 patients who had undergone endoprosthetic replacement of the proximal humerus between 1976 and 1998. The outcome was assessed with regard to the survivorship of the patients, the salvaged limbs and the prostheses. Function was determined in the 47 surviving patients, of whom 30 were assessed using the Musculo-Skeletal Tumour Society (MSTS) rating scale and 38 completed the Toronto Extremity Salvage Score (TESS) questionnaire.

The median age of the patients was 34 years (10 to 80). The mean follow-up period for surviving patients was nine years (2 to 20). The mean MSTS score at follow-up was 79% and the mean TESS score was 72%. The length of bone which was resected influenced the functional outcome. Abduction of the shoulder was to 45° in most patients. The overall survival was 42% at ten years and that of the limb without amputation 93%. The survival of the prostheses using mechanical failure as the endpoint was 86.5% at 20 years.

Endoprosthetic replacement of the proximal humerus is a predictable procedure providing reasonable function of the arm with a low rate of complications at long-term follow-up.

Ten patients underwent endoprosthetic replacement of the distal humerus for bone tumours over a period of 30 years. There were eight primary and two secondary tumours in four men and six women with a mean age of 47.5 years (15 to 76). The mean follow-up was eight years (9 months to 31 years). Four patients required further surgery, three having revision for aseptic loosening; two of these and one other later needing a rebushing. There were no cases of postoperative nerve palsy, infection, local recurrence or mechanical failure of the implant. Four patients died from their disease, all with the prosthesis functioning satisfactorily. At follow-up the mean flexion deformity of the elbow was 15° (0 to 35) and the mean range of flexion was 115° (110 to 135). The functional results showed a mean Toronto extremity salvage score of 73% of normal. Endoprosthetic replacement of the distal humerus and elbow is a satisfactory method of treating these rare tumours.

Our centre has used a specially designed custom-made endoprostheses with curved stems to reconstruct femoral defects in patients with residual short proximal femur after excision of primary bone sarcoma over the last 18 years. Two designs of endoprostheses with curved intramedullary stems were used: the rhinohorn stem type and the bifid stem type. We report the safety, survival and functional outcome of this form of reconstruction.

Twenty six patients who had these special endoprosthesis reconstruction were studied. The median age was 16 years (range 7 to 60 years). Prostheses with rhino horn stems were used in 15 patients and bifid-stem in 1 1 patients. Twenty patients had the prostheses inserted as a primary procedure after excision of primary bone sarcoma, and in six patients the prostheses were inserted after revision surgery of failed distal femur endoprostheses. Seventeen patients (65%) were alive and free of disease at a median follow-up of 98 months (12 to 203 months) and nine patients had died of metastatic disease. Local recurrence developed in two patients (1 0%) out of the 20 patients. Surgical complications occurred in five patients (191/o). Deep infections occurred in two patients (8%) requiring revision surgery in one patient. Prosthetic failure, occurred in nine patients (35%). The cumulative survival of prostheses was 69% at five years and 43% at 10 years. Musculoskeletal Tumour Society mean functional score was 83% (53% to 97%).

In conclusion, preservation of a short segment of the proximal femur and the use of endoprostheses with curved stems for reconstruction of the femur is technically possible. There is an increased risk of fracture of the prostheses decreasing the survival rate. Functional outcome of patients with this form of reconstruction is not significantly different from the functional outcome of patients who have proximal femur or total femur endoprosthetic reconstruction. This operation is particularly desirable in skeletally immature patients and allows normal development of the acetabulum.

Deep prosthetic infections are a significant cause of failure after arthroplastic surgery. Superficial wound infections are a risk factor for deep infections. We aimed to quantify the risk of deep infection after superficial wound infections, and analyse the microbiology of organisms grown.

We defused Superficial Infection according to the definition used by the Centre for Disease Control, and Deep Infection according to the Swedish Hip Register. We retrospectively analysed the results of 6782 THR and TKRs performed consecutively from 1988–1998. We analysed patient records, radiology and microbiological data. The latter collected prospectively by our infection control team.

We identified 81 (1. 2%) superficial wound infections, however we had to exclude 3 due to poor follow-up. Of the 78 patients studied, mean age was 71 (23–89), 50 were female, 28 male, 41 THR, 37 TKR and follow-up was a mean 49 months (12–130). The majority (81%) of organisms grown in the superficial wound infections were gram positive Staphylococci. These organisms were most frequently sensitive to Erythromycin or Flucloxacillin. All the superficial infections were treated with antibiotics, 66% settled with less than 6 weeks therapy. Deep prosthetic infections occurred in 10% of superficial infections in both THR and TKR. In 80% of cases the organism in the superficial infection caused the deep infection. Wound dehiscence, haematoma, post-op pyrexia and patient risk factors had no affect on onset of deep infection. However patients who had a wound discharge with positive microbiology and those patients in whom there was clinical doubt about the diagnosis of deep infection and thus had antibiotic therapy for more than six weeks had increased risk of deep sepsis.

Inadvertent excision of lumps which turn out to be soft tissue sarcomas is still unfortunately quite common. It is known as the “whoops” procedure. Determining whether there is residual disease is key to deciding subsequent management. The value of MRI has been assessed.

All new patients referred to our unit with a potential diagnosis of a “whoops” lesion were routinely reassessed with MR1 6 weeks after the initial operation.

Notwithstanding the result of the scan all patients underwent a further wide excision of the involved area shortly after the MRI. The scans of these patients have been reviewed and classified into positive, equivocal or negative. These results have been compared with the histological assessment of the re- excision specimen to determine the accuracy of MR1 in predicting the presence of residual tumour.

Of 887 patients with newly diagnosed soft tissue sarcomas seen in an 8 year period, 140 (11 %) had previously had a ‘whoops” procedure. Of these 111 had re-evaluation MR1 scans and had also undergone a further re-excision. There was residual tumour in 63 (57%) patients, whilst 48 (43%) had no residual tumour.

The sensitivity of MRI in predicting tumour was 64% but specificity 93%. Positive predictive value was 93% and negative predictive value 67%. Overall accuracy was 77%.

MRI is useful in identifying residual tumour after a whoops procedure but a negative result by no means excludes it. Re-excision remains essential despite the MRI results in most cases to ensure tumour clearance. Preventing the “whoops” procedure is clearly the best option of all!

Endoprosthetic Replacements are one of the most commonly used types of limb salvage following surgical excision of bone tumours. The advantages of Endoprosthetic Replacements are their initial reliability and the rapid restoration of function along with their ready availability. The problems with Endoprosthetic Replacements are the long term problems of wear, loosening, infection and mechanical failure. Increasing and insolvable problems may lead to the necessity for amputation. This paper assesses the risk of amputation following Endoprosthetic Replacement.

A total of 1262 patients have undergone Endoprosthetic Replacement surgery at our centre in the past 34 years. They have a total of 6507 patient years of follow up. A total of 112 patients have had subsequent amputation (8. 9%). The reasons for amputation were local recurrence in 71(64. 4%), infection in 38(33. 9%), mechanical failure in 2(1. 8%) and continued pain in 1 case (0. 8%). The risk of amputation was greatest in the proximal tibia 15. 5% (n=38/246), followed by pelvis 10. 2%(5/49), and femur 7. 4% (n=58/784), whilst the risk of amputation was least in the humerus at 6. 4% (n= 1l/182). The time to amputation varied from 2 days to 16. 3 7 years, with a mean of 31 months. The risk of amputation decreased with time although 10% of the amputations took place more than 5 years after implantation.

The greatest risk of amputation is in the first 5 years and is due to local recurrence, whilst infection poses the next greatest threat. The risk decreases with time. Attempts to control both local recurrence and infection will decrease the need for amputation. Late failure of the endoprosthetic replacements, even in young patients does not seen to be a major cause of amputation thus far.

Limb Salvage surgery is the preferred treatment for malignant tumours of bone. This may require resection – arthrodesis, endoprosthetic replacement or allograft reconstruction.

We have re-implanted the patient’s bone for reconstruction of the defect after debulking the tumour and irradiation in ten patients. All had grade IIB sarcomas of the pelvis, humerus, tibia and metacarpal.

Median overall survival was 24 months (maximum 69 months). Four patients are alive at the most recent follow-up. One of them has metastatic disease and local recurrence while others remain free of disease. One patient has had pathological fracture through the irradiated bone that healed with conservative measures. One developed avascular necrosis of the femoral head and required resurfacing arthroplasty of the hip.

The pelvic sarcoma continues to be a challenge. Resection, extracorporal irradiation and re-implantation may offer some hope but remain experimental.

The purpose of this retrospective study was to analyze the risk factors, causes, bacteriology of deep infection following extensible endoprosthetic replacement for bone tumours in children and to review our experience in the treatment of 20 patients with infected prostheses.

123 patients with extensible endoprostheses were treated between 1983 and 1998. Three types of prostheses, which differed in the lengthening mechanism used, were implanted. 20 of these were diagnosed to have deep infection. Patients were divided into 3 groups: group I 5 patients were treated with a single stage revision, group 11– 13 patients were treated with a two stage revision procedure, group Ill- 2 patients had a primary amputation. Control of infection was assessed clinically and with inflammatory markers. Function was assessed using the MSTS score.

The overall incidence of infection was 16%. The incidence of infection at the proximal tibia and distal femur was 27% and 14% respectively. Staphylococcus epidermidis was the most common organism. The most common clinical features were pain and swelling around the prostheses. Infection in most cases was immediately preceded by an operative procedure or by distant a focus of infection. The number of operative procedures and the site of the prosthesis were significant risk factors. The success rate was 20% in Group 1 and 84% in Group II. Amputation was the salvage procedure of choice for failed revision procedures. The mean MSTS functional score was 83% in patients in whom the infection was controlled.

The incidence of deep infection is high following extensible endoprostheses. The site of the prosthesis and the number of operative procedures are significant risk factors. The type of prosthesis used is not a risk factor. Two-stage revision is successful in controlling infection in a majority of these cases.

This is a retrospective study of 70 patients with chondroblastoma treated between 1973 to 2000. Of these 70 patients, 53 had their primary procedure performed at our unit in the form of an intralesional curettage. The purpose of this study was to determine the rates of recurrence and the functional outcomes following this technique. Factors associated with aggressive tumour behaviour were also analysed. The patients were followed up for at least 22 months, up to a maximum of 27 years. 6 out of these 53 cases (11. 3%) had a histologically proven local recurrence. Three patients underwent a second intralesional curettage procedure and had no further recurrences. Two patients had endoprosthetic replacement of the proximal humerus and one patient underwent a below knee amputation following aggressive local recurrences. One patient had the rare malignant metastatic chondroblastoma and died eventually. The mean MSTS score was 94. 1%. We conclude that meticulous primary intralesional curettage without any additional procedure can achieve low rates of local recurrence and excellent long-term functional results.

Between 1975 and 2000, we treated 404 patients with Ewings sarcoma; 350 had osseous and 54 had extraosseous forms. The 5 year survival for osseous was 65%, and for extraosseous, 63%. There was a statistically significant difference in the average age of the 2 groups. However, there was no difference in the prognosis with regard initial blood results, excision margin, response to chemotherapy, or the presence of metastases at diagnosis. The overall rate of metastases was the same in both groups. We would therefore suggest that both osseous and extraosseous Ewings sarcoma are manifestations of the same disease.

Malignant peripheral nerve sheath tumours (MPNSTs) constitute 10% of soft tissue sarcomas. A significant proportion arise in neurofibromatosis type 1 (NF1). Several publications have compared MPNST survival in sporadic and NF1 patients, without consensus on whether NF1 is an independent factor for poor prognosis.

Clinical and histological data from 135 proven MPNSTs were analysed from 2 national centres for soft tissue tumour surgery diagnosed from 1979 to 2000. 129 patients had follow-up data from 6 months to 21 years. 35 were from patients with NF1. Local treatment involved surgery in surgery in 95%, radiotherapy in 44% and chemotherapy in 21%.

NF1 patients were younger than those with sporadic tumours (median age 26 years vs 53 years, p< 0. 001). Overall MPNST survival was almost identical to that in soft tissue sarcomas as a whole, but was worse in NF1 than in sporadic tumours (33% vs 72% at 30 months [p< 0. 01], 17% vs 39% at 60 months, 6% vs 21% at 120 months). A trend towards shorter time to local recurrence was seen in NF1, but not time to metastasis. Superficial tumours gave improved prognosis. Tumour volume over 100ml was associated with worse survival (46% vs 91% at 30 months, p< 0. 02), as was histological grade (80% high grade vs 25% low grade at 60 months, p< 0. 01). In terms of location, a non-significant over-representation of NF1 MPNSTs in the sciatic and brachial plexii was identified.

NF1 and sporadic MPNSTs exhibited no difference in depth or tumour volume profile, although NF1 tended towards higher grade. Analysis of survival in only high grade tumours, however, still resulted in a significant survival disadvantage in NF1 (33% vs 70% at 30 months, p< 0. 01). Removal of brachial and sciatic plexus tumours from analysis did not affect survivorship profiles in NF1 and sporadic MPNSTs.

Grade, volume and tumour depth correlate with survival; only 7 of 45 patients with deep high grade tumours over 100ml volume were observed to survive beyond 2 years. MPNST survival is worse in NF1 than sporadic tumours. Grade, depth, site and volume differences could not explain this disadvantage.

Few studies of wound complications following limb salvage surgery for soft tissue sarcomas separate anatomical compartments. Forty-nine patients with adductor compartment sarcomas underwent limb salvage surgery, 43% developing significant wound complications, 25% requiring further surgery and 20% had delays in adjuvant radiotherapy as a result. Prior surgery by non tumour surgeons and previous radiotherapy led to an increased risk of wound healing problems. In this particular group of patients, special attention should be made to prevent wound healing complications, possibly involving plastic surgeons at an earlier stage of management.

To assess whether primary spindle cell sarcomas of bone behave like other primary bone sarcomas.

185 patients with primary spindle cell sarcomas of bone, that is non-osteosarcoma, non-chondrosarcoma and non-Ewings sarcoma of bone, were identified from the patient database of the Orthopaedic Oncology Service in Birmingham, UK. This database contains information on over 10, 000 patients treated in Birmingham since 1970 and collected prospectively since 1986.

Spindle cell sarcomas of bone are primary bone tumours which share the histological spindle shaped cells. They are a heterogeneous group including the cytological diagnoses of malignant fibrous histiocytoma (MFH), spindle cell sarcoma, leiomyosarcoma, fibrosarcoma, angiosarcoma and secondary sarcoma.

119 (64%) of the patients were male. The mean age was 47 years with 116 (63%) older than 40 years. Only 6 patients were low grade and 34 had metastases at presentation. 12 patients developed tumours secondarily to other pathology such as Pagets or radiotherapy. The patient’s limb was affected in 155 (84%) of cases with the distal femur being the most common site.

Treatment was with chemotherapy and surgery where indicated. Chemotherapy was with adriamycin and cis-platin most commonly and was neoadjuvant in 75% of those who had it. Of the 150 patients who had surgery, 113 (75%) had limb salvage.

Overall five year survival was 52 %. Survival in patients who had high grade limb tumours, with no metastases at presentation and which weren’t secondary tumours had a five year survival of 60 %. These results are comparable to previous studies looking at similar tumour groups. Good prognostic factors included treatment since 1980, good response (> 90% necrosis) to neo-adjuvant chemotherapy, limb tumours, age < 40 and local control. 73% of patients with local recurrence died with a mean survival of 11 months. There was no difference in outcome or behaviour between the different diagnoses although prognosis was slightly better than those of aged matched patients with osteosarcoma.

Spindle cell sarcomas of bone respond in a similar way to, if not better than, osteosarcoma when treated in the same way with chemotherapy and surgery. Consequently this should be the preferred method of management.

Current survival rates for cancer in the UK are perceived to be worse than those in mainland Europe. In order to asses this we investigated the prognostic value of patient and treatment parameters in the management of osteosarcoma, and whether these parameters are equally important across international boundaries.

Retrospective, cross-sectional study of patients (n=428) diagnosed with non metastatic distal femur or proximal tibia osteosarcoma, between 1990–1997 at two specialist orthopaedic oncology centres; Birmingham, UK and Bologna, Italy. Disease free survival (DFS) and overall survival (OS) were assessed by Kaplan-Meier, Fisher’s PLSD and Cox proportional hazard regression. Results : DFS and OS were 43% and 60% at 5 years in Centre 1 and 56% and 73% at Centre 2 respectively. Median survival was 108 weeks at Centre 1 and 136 weeks at Centre 2. A significant difference in DFS and OS was demonstrated between the centres (p=0. 0019 and p=0. 0280 respectively). The most important prognosticators were raised alkaline phosphatase (p=0. 002 and p=0. 0019), degree of chemotherapy induced necrosis (p=0. 0001 and p=0. 0002) and tumour volume > 150cm³ (p=0. 0037 and p=0. 0057).

The most significant combination of prognosticators was alkaline phosphatase and tumour necrosis. 75% of patients in centre 2 had a good chemotherapy response (> 90% necrosis) compared to only 29% in Centre 1. The other prognostic indicators were evenly matched. Chemotherapy regime was found to have significantly different outcome in DFS and OS.

This is a retrospective study designed to explore possible reasons for differences in survival between two international centres. It would appear that all known patient factors were matched between the centres but that the main difference was in the effectiveness of chemotherapy. Further international prospective studies are needed to confirm these findings.

Reconstruction of the shoulder joint following resection of the proximal humerus for bone tumours remains controversial. We report the long term functional results of the simplest form of reconstruction – an endoprosthesis.

One hundred patients underwent endoprosthetic replacement of the proximal humerus between 1976 and 1998. Thirty eight had osteosarcoma, 17 had chondrosarcoma, 16 had metastases and 9 had Ewing’s sarcoma. Mean age was 36 years (range 10 to 80 yrs). Survivorship of patients and prostheses were calculated. Function was assessed using the Musculoskeletal tumour society (MSTS) and Toronto extremity salvage (TESS) scoring systems. Thirty patients could come to the clinics for MSTS scoring and 38 out of 49 alive patients replied to the TESS questionnaires sent out to them.

The overall survival of the patients was 42% at 10 years. Local recurrence (LR) arose in 16 patients, being most common in chondrosarcoma (26%) and osteosarcoma (22%) and arose in 50% of patients with these tumours who had marginal excisions. Of these 16 patients, 8 had forequarter amputations whilst the remainder had excisions and radiotherapy. Mean time to LR was 12 months and all but two of these 16 patients subsequently died within a mean of 18 months.

The prostheses proved reliable and dependable. Only 9 required further surgery of any sort, 2 needing minor surgery to correct subluxation and 7 needing revisions, one for infection after radiotherapy and six for loosening – three after trauma. The survivorship of the prosthesis without any further surgery was 86. 5% at 20years. The survivorship of the limb without amputation was 93% at 20 years.

The functional outcome was very predictable. Most patients had only 45 degrees of abduction although three patients had normal movements. The mean MSTS functional score was 79% and the mean TESS score was also 79%. There was a high level of patient satisfaction but difficulty was encountered especially in lifting and in all activities above shoulder height. Endoprosthetic replacement of the proximal humerus is a predictable procedure providing reasonable function of the arm below shoulder height. The endoprostheses have proved highly dependable with a low re-operation rate. There is a high risk of local recurrence after inadequate surgery which should be avoided if possible.

This retrospective clinical study describes our experience of the use of growing endoprostheses in children with primary malignant tumours of the proximal femur and analyses the results.

Between 1983 and 1996 we treated nine children with primary bone tumors of the proximal femur by resection and proximal femoral extensible replacements. Outcomes measured were function of the limb using Musculoskeletal Tumor Society score, oncologic outcome, complications and equalization of limb length. Results: Four patients died as a result of pulmonary metastases. The remaining five patients were observed for an average follow-up period of 7. 6 years (range 11–12. 7 years). One patient had a hindquarter amputation for uncontrolled infection. In these five patients we performed an average of 10. 2 operative procedures per patient (range of 3–17 procedures) including 5 lengthening procedures (range of 1–8 procedures) and a mean total extension of 69. 7 mm per patient. Acetabular loosening and hip dislocations were the most frequent complications. Only two patients have not had a revision or a major complication. Despite this, 4 children are alive with a functioning lower limb and a mean Musculoskeletal Tumour Society functional score of 77. 6%. The limb length discrepancy was less than 1 0 mm in three of these patients. The remaining patient has a discrepancy of 50 mm and is awaiting further limb equalization procedures.

Extendible endoprostheses of the proximal femur in selected children is a viable reconstructive procedure. It allows for equalization of limb length and the ability to walk without the use of mobility aids.

Between 1982 and 1997, twenty-six children between the age of 2 and 15 (mean age 10. 6 years) underwent proximal femoral replacement. Twenty have survived and all but three have reached skeletal maturity.

Sequential radiographs have been reviewed with particular reference to acetabular development and fixation of the prostheses. Initially a cemented acetabular component was inserted, but recently uncemented implants and unipolar femoral heads that exactly fit the acetabulum have been used.

In older children the acetabulum develops normally and the components remain well fixed. One of nine children over thirteen years with a cemented acetabulum needed revision for loosening and one suffered recurrent dislocations.

In younger children the acetabulum continues to develop at the triradiate cartilage, so a cemented acetabulum grows away from the ischiopubic bar. As the component is fixed proximally, it becomes increasingly vertical and will almost inevitably loosen. In our study six of eight children under 13 years of age with a cemented acetabulum needed revision for loosening.

Unipolar replacements in younger children tend to erode the superior acetabular margin. Femoral head cover is difficult to maintain, and of four unipolar implants in children under thirteen, two required acetabular augmentation.

Cemented cups may be unsuitable for children under thirteen years but our results are not statistically significant. In this age group, unipolar implants may be more appropriate but they have serious potential complications. In children over thirteen, cemented implants survive longer. The number of uncemented implants in our study is too small to comment on long-term survival.

34 two-stage revision procedures were carried out between 1989 and 1998 for controlling deep infection following resection of bone tumours and reconstruction with endoprostheses. In 4 cases the procedure failed with early recurrent infection. In six others infection reappeared after further operative procedures. Six of these ten patients required amputation. The success of the procedure in controlling infection was 75% at 5 years. Two stage revision procedures have proved effective in controlling infection in massive endoprostheses but the risk of re-infection appears to be much greater in patients requiring further surgery for any cause.

Although pathological fractures in the aged are usually due to metastasis, solitary lesions with undetected primary should be treated with caution. Assumption of such lesions as metastatic and their subsequent internal fixation could lead to completely inappropriate treatment if the lesion turns out to be a primary sarcoma of bone.

Referrals to our bone tumour service over a four year period were analysed. There were 62 pathological fractures of which 11(17. 8%) were primary sarcomas that were treated as a metastasis. The limb salvage was compromised and survival rates poor in these group.

Although it is believed that primary sarcomas are rare in the aged, our database confirms that 14% of primary sarcomas affect this age group. The survival figures of primary sarcomas in the aged (> 60 years) treated at our centre was a mean of 43 months with a 5-year survival of 22%. The presence of a pathological fracture did not significantly alter the long-term survival of these patients. On the other hand, metastasis had a poor survival with a mean of 19 months and a 5-year survival of 4% showing a significant difference. In addition, these patients underwent major inappropriate surgeries, which rendered limb-salvage difficult, worsened the morbidity and caused mental distress to patients.

Any pathological fracture in the aged presenting as a solitary osseous lesion with an undetected primary or even remote primary should be treated with caution. Their diagnosis needs to be established by biopsy whatever the age of the patient before any form of internal fixation is undertaken. The temptation to carry out biopsy and internal fixation at the same sitting or even a prophylactic fixation should be avoided when the diagnosis is not clear. Standard principles of musculoskeletal oncology need to be followed.

Local recurrence (LR) following treatment of a sarcoma is generally accepted to be a poor prognostic sign and an indicator both of inadequate local control as well as of the failure of adjuvant treatment.

Of 2589 patients with non metastatic Ewings, osteo-sarcoma, chondrosarcoma or a soft tissue sarcoma, 316 have developed LR at some stage following initial treatment of their tumour. 120 were already known to have metastases elsewhere when they developed LR or were found to have them at time of restaging but 196 developed LR as the first sign of relapse.

The mean time to development of LR was 24 months and 72% had arisen within the first two years. Overall survival following LR was 25% at 5 years and 20% at 10 yrs. In patients with metastases at the time of LR or who were found to have them at the time of restaging the median survival was 6 months with only 12% being alive at 2 years. In those with LR as first sign of relapse median survival was 3 years with 30% long term survivors. Patients with low grade tumours had a better outcome than those with high grade – 50% being cured by further surgery. Of the high grade tumours without metastases at time of diagnosis relapsed Ewing’s had the worst prognosis with median survival of 8 months compared with 22 months for osteosarcoma, 36 months for STS and 36 months for chondrosarcoma, despite which overall survival was 16% for both Ewing’s and osteo-sarcoma patients but was 30% for chondrosarcoma and STS.

LR following sarcoma surgery is due to a combination of aggressive disease with inadequate surgery and ineffective adjuvant treatment. In isolated LR aggressive further treatment is justified with an outcome similar to that of metastatic disease.

Survival in the wake of hindquarter amputation and oncological treatment is improving; the hindquarter amputee population is increasing. Some amputees function well, others do not. To assess quality of life and function of hindquarter amputees.

21 amputees (10 females and 11 males) consented to take part in the study. Ethics approval was sought. Assessment was performed using postal questionnaires; SF36 for quality of life; TESS (Toronto Extremity Salvage Score) for physical function and mobility; IEFF( International Index for Erectile Function) for male sexual function; a prosthetics questionnaire to assess prosthetic use.

Mean age of the group was 55 with the mean survival of 7 years post amputation.

Quality of life results were compared to normal subjects and patients for long term illness. Amputees had a significant reduction in quality of life concerning physical function and pain. Social function, mental health and energy levels were equivalent to patients with long term illness.

The mean TESS result was 56.9 with females having a mean score of 61.7 and male of 48. 50% of the group considered themselves as severely to completely disabled; 50% considered themselves moderately disabled. Five were in full time employment.

Six males responded to the sexual function questionnaire. Impotence was universally experienced.

Only five amputees used their prosthesis regularly. All amputees have experienced and 20 continue to experience phantom pain.

This study illustrates that hind quarter amputees have poor physical function and a low quality of life. Phantom pain is universally experienced. Male amputees experience impotence. Addressing these areas would improve the life of current and future hind quarter amputees.

Due to advances in the assessment and treatment of patients with metastatic bone disease, the survival has improved in recent years.

It was the aim of this met-analysis to assess the outcome of patients with metastatic bone disease across Europe.

Five major bone tumour treatment centres participated in the met-analyses. Data had been collected prospectively and was retrieved from the databases for the purpose of this study. All patients were referred to the bone tumour centre for assessment and possible surgical treatment.

The data of almost 2500 patients were analysed. The average age at diagnosis was 60 years and the male to female ratio was 1:1.07.

The overall 5-year survival was 35%, but this was significantly better in metastatic disease of the thyroid and breast as well as multiple myeloma.

The number and site of metastases or the presence of a pathological fracture were not prognostic factors in terms of survival. Patients who were female or younger than 50 years of age at time of diagnosis had a better outcome.

This registry will be expanded and more information analysed to try and provide useful information about prognostic factors and outcome for patients with meta-static bone disease.

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.

From the European Osteosarcoma Intergroup study 202 patients were assessed with respect to their surgical treatment. Although treated in three different centres the survival of the three groups was identical (57% at five years). Two of the centres had rates of limb salvage of 85% and 83%, respectively, while the third had a rate of 49%. The corresponding risks of local recurrence were 13.3%, 6.8% and 2.5%, with all local recurrences arising in limbs with attempted limb salvage. Local recurrence was closely related to the adequacy of the margins of excision and to the chemotherapeutic response. Patients who had undergone limb-salvage surgery and who developed local recurrence still survival at five years). Of patients who relapsed, 31% of those with local recurrence alone were cured by further treatment, as compared with only 10% of those with metastases. Limb-salvage surgery with effective chemotherapy remains the optimum treatment for osteosarcoma.

We studied 153 patients with non-metastatic chondrosarcoma of bone to determine the risk factors for survival and local tumour control. The minimum follow-up was for five years; 52 patients had axial and 101 appendicular tumours. Surgical treatment was by amputation in 27 and limb-preserving surgery in 126.

The cumulative rate of survival of all patients, at 10 and 15 years, was 70% and 63%, respectively; 40 patients developed a local recurrence between 3 and 87 months after surgery and 49 developed metastases. Local recurrence was associated with poor survival in patients with concomitant metastases but not in those without.

On multivariate analysis independent risk factors for rates of survival include extracompartmental spread, development of local recurrence and high histological grade. Independent risk factors for local recurrence include inadequate surgical margins and tumour size greater than 10 cm. Location within the body, the type of surgery and the duration of symptoms are of no prognostic significance. Surgical excision with an oncologically wide margin provides the best prospect both for cure and local control in these patients.

Limb salvage is now customary in the treatment of primary bone tumours. The proximal tibia is a frequent site for these neoplasms but reconstruction, especially in children, is a formidable challenge. We reviewed 20 children with extendible replacements of the proximal tibia, all with a minimum follow-up of five years. Five died from their disease and, of the remaining 15, four had above-knee amputations for complications. Infection occurred in seven patients; in five it was related to the lengthening procedure. Aseptic loosening is inevitable in the younger children and only two have avoided a revision, amputation or other major complication; both were aged 12 years at the time of the initial surgery. Despite this, 11 children are alive with a functioning leg and a mean Musculoskeletal Tumour Society functional score of 83%. The lengthening mechanisms used in our series required extensive open operations. We are now using a simpler, minimally invasive, technique which we hope will decrease the incidence of complications. At present, the use of extendible prostheses of the proximal tibia remains an experimental procedure.

We carried out excision of a solitary bony metastasis from renal-cell carcinoma in 25 patients in the hope that this would produce a prolonged disease-free interval. Two patients had excisions only, five had amputations and 18 had excision and endoprosthetic replacement. The one-, three- and five-year cumulative survival rates were 88%, 54% and 13%, respectively.

There were three complications. One patient developed a local recurrence and three had problems related to the endoprosthesis.

We recommend radical excision of a solitary bony metastasis from renal-cell carcinoma to achieve local control of the tumour for the remainder of the patient’s life.

Dedifferentiated chondrosarcoma is a rare, highly malignant variant of chondrosarcoma in which a high-grade spindle-cell sarcoma coexists with a lower-grade chondroid tumour. We have reviewed our experience with this neoplasm in 22 patients, all of whom were treated using modern oncological principles of planned resection and chemotherapy. Despite this the median survival was under nine months and only 18% were alive at five years. Those patients who received chemotherapy, and in whom wide margins of excision were achieved at operation, did best. It is essential to have an accurate preoperative diagnosis in order to plan treatment which may offer a better prospect of cure.

Over a 25-year period we have treated 36 patients with osteosarcoma of the pelvis. Of the tumours, 24 (67%) were primary osteosarcomas and 12 (33%) arose either after irradiation or in association with Paget’s disease. Six patients had a hindquarter amputation and 12 were treated by a limb-salvage procedure with intrapelvic excision.

The five-year survival rate of all the patients with pelvic osteosarcoma was 18%, while for 17 treated by chemotherapy and surgery it was 41%. The prognosis for patients presenting with metastases or with secondary osteosarcoma was appalling and none survived after 29 months. No patient over the age of 50 years when seen initially survived for a year. Youth and a good response to chemotherapy along with complete surgical excision offer the best chance of cure.