Low grade central osteosarcoma is a rare intramedullary bone producing tumour. It accounts for only 1–2% of all osteosarcomas. Due to the indolent nature of low grade central osteosarcoma, achieving a correct and prompt diagnosis is the real challenge both from imaging and histology, particularly as it may resemble a benign condition, i.e. Fibrous Dysplasia.

We have reviewed 15 cases of low grade central osteosarcoma with long term follow-up (2 to 22 years) to identify problems in diagnosis and treatment and to assess outcome.

There were 7 females and 8 males with a mean age of 37 yrs (range 11 to 72 years); 13 cases arose in the lower limb (8 femur, 4 tibia, 1 os calcis), 1 in the pelvis and 1 in the upper limb. The average duration of symptoms prior to presentation was over 2yrs. A primary diagnosis of low grade central osteosarcoma was achieved for only 6 cases (4 open and 2 needle biopsies), in the other 9 the primary diagnoses were GCT, cystic lesion or fibrous lesion (both benign and malignant) and all of them had undergone treatment (usually curettage with or without bone grafting for this). Definitive treatment was with surgery attempting to obtain wide margins. Marginal excision was associated with local recurrence in three cases but there were no local recurrences in patients who had a wide excision, even in those with prior treatment. Only one patient has died following the development of multiple metastases after 9 years. The survival rate is 90% at 15 years.

We present this study to show the difficulties in diagnosing this rare type of osteosarcoma and to highlight the importance of wide surgical margins to obtain local control.

Aim: To investigate the effectiveness of silver coated titanium prostheses in preventing periprosthetic infection in a group of very high risk patients.

Methods: Periprosthetic infection is one of the main problems in limb salvage surgery, especially for tibial and pelvic prostheses or following revision surgery, particularly if it has been done for a previous infection. We have used silver coated prostheses in 21 cases on a named patient basis and have now assessed the results.

Results: Between July 2006 and June 2008 21 patients had a silver coated prosthesis inserted. 11 patients were having a second stage revision after a previous infection, 6 were having a pelvic prosthesis inserted, 3 were having a primary tibial replacement and one a one stage proximal tibial revision. Three patients developed a postoperative infection, two of the pelvic replacements and one infected revision (a total femur replacement). Of these only one patient required removal of the prosthesis (for overwhelming coliform infection in a pelvic replacement) whilst the other two infections both settled with antibiotics and washout.

Discussion: The anticipated risk of infection in this high risk group would have been around 20%. The actual infection rate was 14% but two of the infections completely resolved with relatively modest treatment. This suggests that the silver coating may not only have a role in preventing infection but also enhancing control of infection should it arise. There were no other side effects and we believe that these preliminary results are encouraging and should lead to a further evaluation of silver for preventing infection around prostheses.

Aim: To review our experience of managing patients with aneurysmal bone cysts (ABC)

Method: We reviewed the medical records and radiographs of all patienst with aneurismal bone cyst treated at our unit over a 25 year period. During that time the policy of the unit was to treat ABCs with biopsy/curettage without use of adjuvants or bone grafting. Patients were followed up with regular Xrays until healing had taken place. Local recurrences were again treated with curettage, occasionally supplemented with embolisation or bone grafting.

Results: 237 patients (128 female, 109 male), with a median age of 14 yrs (range 1 to 76) received treatment. The cyst size varied from 1 to 20 cm and the median duration of symptoms was 16 weeks (range 0 to 8 yrs). The most common sites were the tibia (55) followed by the femur (41) then the pelvis (29) and humerus (27). 35 (15%) of the patients presented with a pathological fracture. Primary treatment was by curettage alone in 195, curettage and bone grafting in 7, aspiration and injection of steroids or bone marrow in 7, excision in 5 and observation alone in 17. The rate of local recurrence requiring further surgery was 12% with all local recurrences (bar one) arising within 18 months. Local recurrence was not related to site, age, sex or whether the patient had previous treatment or not. Local recurrences were managed with curettage alone in 19 of the 23 cases, with one having embolisation, one excision and 2 curettage and bone grafting. This was successful in all but 3 cases who were controlled with a third procedure.

Conclusion: The local control rate of ABCs with simple curettage is 88% which is as good as those published for any other technique. We recommend biopsy in all cases and limited curettage at the same time, many ABCs will heal with this simple procedure. Full curettage is needed for those showing no signs of healing within 4 weeks. Local recurrence is very unusual after 18 months.

Introduction: Parosteal osteosarcoma is a rare, mostly low grade tumor arising from the surface of the bone. The aim of the study was to establish risk factors for local recurrence (LR) and death.

Method: Retrospective review of a prospectively collected database with complete up-to-date follow up.

Results: 63 patients were diagnosed and treated at our centre from 1978 to 2007. The most common site was the femur (37) followed by the tibia (10) and humerus (9). The median size was 7cm (2 – 16). At the time of diagnosis 27 (43%) were low grade, 13 (21%) were low grade but had invaded the medullary canal and 8 (13%) had a high grade component; the data was missing in 15 patients (24%). All patients were operated.

LR arose in 11 patients (17.5%) at a mean of 38 months and was related to surgical margins. There were no LR in the 26 wide resections, but the risk of LR was 20% in those with a marginal and 46% in those with an intralesional excision. LR appeared as a higher grade than the primary tumor in 3 patients (27%).

9 (14.3%) patients developed metastases at a median of 22 months (6 – 123). Five patients developed both LR and metastases. Seven patients died from the tumor, 2 patients are alive with metastatic disease.

None of the patients with a low grade tumor and wide or marginal margins died of tumor, but 2 patients (18%) operated intralesional died of tumor.

Discussion: There was a statistically significant relationship between surgical margins and LR (p< 0.001). All patients with low grade parosteal osteosarcoma operated with wide margins have been cured. LR appeared often in higher grade than the primary tumor. Patients with low grade tumor and intralesional margins had 18% risk to tumor-ralated death.

Ewing’s sarcoma principally arises in bone but can also present as a soft tissue tumour. Very few studies have assessed the outcomes of extra-skeletal Ewing’s sarcomas. This study compares the oncological outcomes of the two forms of Ewing’s sarcomas to see if there is any difference in prognostic factors.

198 patients with primary, non metastatic Ewing’s sarcoma diagnosed between 1980 and 2005 were identified from our database. There were 118 males and 80 females with a median age of 15 years. The three most common sites of diagnosis were the femur (24%), pelvis (15%) and tibia (13%). There were 169(85%) bony Ewing’s and 29 (15%) extra-skeletal Ewing’s sarcomas. All patients received chemotherapy. 86% of the patients had surgery for local control but 28(14%) patients had radiotherapy.

The overall survival at five years was 89% and was related to the age of patient (92% < 16years p=0.005), size (p=0.03) and site of tumour (p=0.004) as well as the response to chemotherapy. There was no difference in the overall survival of patients with bony Ewing’s (90%) and extra-skeletal Ewing’s (85%) (p=0.85). There was a 10% risk of local recurrence at 5 years with site of tumour (p=0.01) and surgical excision (p=0.05) being significant prognostic factors. The risk of local recurrence was also not related to the type of Ewing’s sarcoma.

This large series has shown that the oncological outcomes of Ewing’s sarcoma is related to tumour characteristics, patient age and treatment factors and not determined by the tissue component.

Aim: To identify the clinical features of patients who present with soft tissue metastases (from a cancer elsewhere) and to identify the source of the cancer.

Method: In a database containing details of 7242 patients referred to our unit for investigation of a soft tissue lump, only 100 of these patients were found to have a soft tissue metastasis (1.4%). We analysed their presenting features and identified the site of the primary malignancy.

Results: The most common presentation of soft tissue metastases was a painless lump The lumps ranging from 2 to 35 cm (mean 8.3cm) with 78% of the lumps located deep to the fascia. The mean age at presentation was 62 and there were equal males and females. 53 had a past history of malignancy. Of these 53, 52 had metastases from the same primary (lung 11, melanoma 10, kidney 9, GI tract 4, breast 6, bladder 4 and others in 9). The other 47 patients had no past history of malignancy and the metastasis was the first presentation of malignancy. The primary sites in these cases were: lung in 19, GI tract 4, kidney 2, melanoma 9, other 3 and unknown (despite investigations) in 10. There was no correlation of the site of the metastases with the primary tumour.

Of the 7242 patients with soft tissue lumps, 476 had a past history of malignancy. Of these patients, only 12% actually had a soft tissue metastasis while 28% had a benign diagnosis, 55% a soft tissue sarcoma and 5% other malignancy.

Conclusion: Patients with a past medical history of malignancy and a new soft tissue lump have a 12% chance of it being a soft tissue metastasis. If a lump proves to be a soft tissue metasasis, the lung is the most common primary site.

In our database of 7935 patients referred for investigation of a soft-tissue mass, only 100 were found to have a soft-tissue metastasis (1.3%). Our aim was to define the clinical features of such patients and to identify the site of their primary tumour.

The most common presentation was a painful lump, deep to the fascia, ranging between 2 cm and 35 cm (mean 8.3 cm) with 78% of the lumps located deep to the fascia. The mean age of the patients at presentation was 64 years (22 to 84) and there were almost equal numbers of men and women. Of 53 patients with a history of malignancy, 52 had metastases from the same primary (lung in 12, melanoma in ten, kidney in nine, gastrointestinal track in four, breast in five, bladder in four, and others in eight). The other 47 had no history of malignancy and the metastasis was the first presentation. The primary sites in these cases were the lung in 19, gastro-intestinal track in four, kidney in two, melanoma in nine, other in three, and unknown (despite investigations) in ten. There was no correlation between the site of the metastases and the primary tumour.

Of the 7935 patients, 516 had a history of malignancy. Of these, only 10% had a soft-tissue metastasis, 29% had a benign diagnosis, 55% a soft-tissue sarcoma and 6% another malignancy. Patients with soft-tissue metastases have similar clinical features to those with soft-tissue sarcomas and should be considered for assessment at appropriate diagnostic centres for patients with suspicious soft-tissue lumps.

We undertook a cemental unipolar proximal femoral endoprosthetic replacement in 131 patients with a mean age of 50 years (2 to 84). Primary malignant tumours were present in 54 patients and 67 had metastatic disease. In addition, eight patients had either lymphoma or myeloma and two had non-oncological disorders. The mean follow-up was 27 months (0 to 180). An acetabular revision was required later in 14 patients, 12 of whom had been under the age of 21 years at the time of insertion of their original prosthesis. The risk of acetabular revision in patients over 21 years of age was 8% at five years compared with 36% in those aged under 21 years. All the unipolar hips in this younger age group required revision within 11 years of the initial operation.

We conclude that unipolar replacement should not be used in younger patients and should be avoided in patients with a life expectancy of more than five years.

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.

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.

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 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.