Osteosarcomas represent a heterogeneous group of primary bone tumours that affect predominantly the long bones of patients in the first two decades of life. We aim to describe the secondary effects of a poor response (⋋90% necrosis) to chemotherapy on the effectivity of other treatment outcomes, local recurrence and survival rates.

182 cases of osteosarcoma with necrosis of less than 90% and no metastases at diagnosis have been seen at our institution over 24 years. There were 60 amputations. 122 patients underwent limb salvage, with 105 marginal margins and 17 contaminated. There was no difference in size or location between the two groups. In the 122 patients with LSS, 21 had adjuvant radiotherapy and 101 did not. In the entirety of patients with ⋋90% necrosis, survival was 64% at 2 years and 37% at 5 years. When LSS Marginal resections were compared with amputation there was a significant (P=0.006) difference in survival. LSS with a marginal margin had a 25% risk of LR. In these patients there was 25% survival, whereas the absence of a local recurrence, conferred a benefit of a 40% survival XRT was used in 21 of the 122 who underwent limb salvage. The decision to use XRT was made by the local oncologist at the treating unit. There was a 24% rate of recurrence in the XRT group and 25% with no XRT.

These data demonstrated that patients who had a poor response to chemotherapy and underwent an amputation faired poorly when compared to patients with LSS. There is a selection bias in patients selected to undergo amputation. Additionally, patients who underwent amputation had a lower rate of local recurrence, but still had a poorer survival when compared to LSS.

Introduction

The role of adjuvants in curettage for giant cell tumours (GCT) is still controversial. Our aim was to determine if adjuvant cementation lowers local recurrence (LR) rates for GCTs treated with curettage.

Aim

To investigate the effectiveness of silver coated prostheses in preventing periprosthetic infection in a high-risk group.

Introduction

Myxofibrosarcoma is described by WHO as comprising of a spectrum of malignant fibroblastic lesions with variably myxoid stoma, pleomorphism and with a distinctively curvilinear vascular pattern. They are reported to be one of the commonest sarcomas of elderly patients. It has previously been reported to have a high rate of locally recurrent disease (50-60%). The aim of the study was to investigate the ROH series of tumours to determine prognostic factors for survival and local recurrence.

The aim was to identify when primary amputation was used as primary treatment and to describe outcomes in patients managed with modern chemotherapy. A detailed review of the electronic patient records was undertaken. Statistical analysis was performed with univariate analysis using Kaplan-Meier curves and Chi² testing, whilst multivariate analysis was performed using Cox regression analysis.

There were 354 osteosarcomas. 93 patients presented with metastases and 192 subsequently developed metastases at a mean of 46 months. Amputation was performed as the primary surgical treatment in 101 patients. Endoprosthetic reconstruction was used in 253 patients. Amputation was performed as a secondary procedure on 15 patients.

The 5 and 10 year survival data for all patients, including those with metastatic disease were 60% and 60% for amputation with good chemotherapy response (>89% necrosis), 65% and 63% for limb salvage and good response, 21% and 21% for amputation and poor response (⋋90% necrosis) and 51% and 30% for limb salvage with poor response.

Local recurrence occurred both with amputation (10.8%) or limb salvage (9%), with no significant differences between the two.

Univariate analysis demonstrated that the extent of response to chemotherapy induced necrosis significantly affected survival, whether the patient had an amputation or not.

Whether or not amputation or LSS was used in the surgical management of patients, local recurrence rates where similar between the two groups. Further assessment of chemotherapy-induced necrosis is a key factor in determining subsequent limb salvage or amputation management strategies.

Chondrosarcoma of bone is a surgical disease and excision with wide margins is the optimum treatment. Sometimes the size or location of the tumour at the time of diagnosis mean that only a marginal excision can be achieved. The effect of the margin of excision on outcome is investigated.

Aim

To determine the overall survival of patients with Pelvic Ewing's Sarcoma treated in our unit and to identify prognostic factors in pelvic primaries that could be used to select patients who would most likely benefit from high intensity treatment.

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.

The outcome for patients with Ewing's sarcoma recurrence is poor. Local recurrences occur in 8%-25%of these patients. The aim of the study was to analyze the patients who had a local recurrence to identify factors predicting the local recurrence and if it could be prevented

There is currently no standard follow up protocol for patients who have been diagnosed with and treated for high-grade osteosarcoma. We therefore investigated the possibility of creating a risk based follow-up protocol for patients with primary osteosarcomas.

313 patients diagnosed with primary osteosarcomas were studied. The identified risk factors for local recurrence included poor necrosis, inadequate margins and high risk tumour site in the bone. The risk factors for metastases were poor necrosis, inadequate margins, extra-compartmental stage and tumour size ≥5cm.

The risk of local recurrence and/or metastases within three years of diagnosis increases as the number of risk factors increase. Patients were grouped according to their number of risk factors. The cumulative risk of metastases for patients with 0, 1, 2, 3 and 4 risk factors is 0%, 12%, 21%, 54% and 60% respectively (p=<0.0001). Risk of local recurrence for patients with 0, 1, 2 and 3 risk factors is 5%, 14%, 25% and 20% respectively (p=0.0025).

Our investigation shows that by grouping patients together according to their number of identified risk factors, it is possible to identify groups of patients that are most at risk. This information can be used to design an evidence based follow up protocol which would have important implications for clinical practice.

Solitary plasmacytomas in the appendicular skeleton are rare monoclonal expansions of plasmacytoid cells. They are two main hazards; local destruction of bone with resultant loss of function and possible fracture, and progression to Myeloma.

Between February 1988 and July 2005 seven patients (4 male, 3 female) were treated for solitary plasmacytoma with surgical resection and endoprosthetic reconstruction. The median age was 46.7 (35-75). The site was: distal humerus (2), proximal humerus (2) proximal femur (2) proximal tibia (1). Three patients had sustained a pathological fracture. Five patients had received pre-operative radiotherapy and three received post-operative radiotherapy. Mean follow-up is 8.6 years. Two cases became infected at 2 and 5 years post-operatively and have had revisions of their endoprosthesis. Both remain functional at 18 and 15 years. No patient has suffered a local recurrence. Two patients have progressed to multiple Myeloma but no patients have died. Literature review shows that the progression of solitary bone plasmacytoma to Myeloma is around 53% despite radiotherapy, in an average period of 2-4 years. With resection and endoprosthetic reconstruction, the progression in this series has been 28% despite an average follow up of 8.6 years.

Although the numbers are small, due to the rarity of the condition, surgical resection and endoprosthetic reconstruction reduces disease progression than radiotherapy alone. This produces far superior results compared to the intramedullary nailing of the long bones for this condition. Endoprosthetic reconstruction after resection should be given consideration in cases of solitary plasmacytoma of the appendicular skeleton when there is extensive bone destruction present. The optimal timing of local radiotherapy to be combined with surgery is still to be established.

We performed a retrospective review of 98 patients with malignant tumours of the periacetabular region, treated by resection and endoprosthetic reconstruction between 1971 and 2005 at the Royal National Orthopaedic Hospital, Stanmore and The Royal Orthopaedic Hospital, Birmingham.

The mean age of the patients was 43.6 years (10 to 76). 53 patients (54%) were male. The age distribution demonstrated peaks in the 2nd and 6th decades. The mean follow up was 65 months (2 to 405. The overall 10-year survival rate was 56% (determined by the Kaplan-Meier method).

54 patients (58.1%) had one or more complications. Infection was the most common (30%) with the majority occurring in the first 2 years. Dislocation occurred in 19 (20%) of our patients all in the first year following surgery. All were recurrent with a mean of 2.9 (2 to 6) episodes. Operations performed before 1994 were associated with a dislocation rate of 40.5% and after 1996 the rate was only 3.9% (p < 0.001).

Function was assessed according the Toronto Extremity Salvage Sore (TESS). The overall TESS was 59.4. In patients that had a major complication the TESS was 37.1 but in the group that did not the TESS was 70.3 (p < 0.001).

Men experienced higher rates of death, infection and revision than women (p < 0.05). The local recurrence rate was 31% with high grade tumours associated with higher recurrence rates (p < 0.05). Tumour resection in the ilium is associated with higher rates of infection than tumours located in the periacetabular region alone (p < 0.05).

This method of reconstruction is still associated with high complication rates. Function is good if a major complication can be avoided. The lower dislocation rate more recently is probably the result of improved surgical technique and the use of larger femoral heads.

Soft tissue sarcomas (STS) arising in the adductor compartment of the thigh are frequently large before clinical detection, posing particular challenges with surgical resection and associated with a high risk of wound complications. This study compares oncological and functional outcomes and complications following treatment of adductor compartment soft tissue sarcomas from three international centres with different treatment philosophies.

184 patients with new primary, non-metastatic, deep STS in the adductor compartment diagnosed between 1990 and 2001 were identified from the centres' local databases. The Toronto Extremity Salvage Score (TESS) was used to assess function in patients.

There were 94 male and 90 female patients, with ages ranging from 13 to 88 years (median age 57 years). The period of follow-up ranged from 1 to 162 months. The overall survival was 65% at 5 years and related to grade and size of the tumour. There was no difference in overall survival or systemic relapse between the three centres. There was however a significant difference in local control (28% LR in centre 1 compared to 10% in centre 2 and 5% in centre 3, which appeared to be principally related to the use of radiotherapy and surgical margins.)

66 patients (36%) from the three centres developed wound complications post-operatively and it was shown to be associated with high grade and large tumours (>10cm).

Functional scores averaged 78% but were significantly worse for patients with wound complications or high-grade tumours; however, they were not affected by timing of radiotherapy or use of prophylactic free muscle flaps.

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.

Aim

To identify patient, tumour or treatment factors that influence outcome in patients with radiation induced sarcoma of bone.

Primary malignant bone tumours frequently arise in children close to the knee, hip or shoulder. Resection of the tumour will often require excision of the epiphysis and frequently one side of the involved joint. In these children an extendable endoprosthesis is usually required to allow for maintenance of limb length equality.

We have used 180 extendable endoprostheses in 176 children since 1975. The indication for use of an extendable prosthesis was if there was more than 30mm of growth remaining in the resected bone. The age of the patients ranged from 2 to 15 and 99 were boys. The sites of the endoprostheses used were: distal femur in 91, proximal tibia in 42, proximal femur in 11, total femur in 6 and proximal or total humerus in 26. 131 of the operations were for osteosarcoma and 34 for Ewing's.

Five types of lengthening mechanism have been used. Two designs used a worm screw gear, one type used a C collar, one type a ball bearing mechanism and the latest uses a non invasive lengthening system whereby a motor inside the prosthesis is activated by an electromagnetic field.

Of the 176 patients, 59 have died and of the remainder, 89 have reached skeletal maturity. 19 patients had an amputation, 11 due to local recurrence and 8 due to infection. The risk of infection was 19% in surviving patients. Most of the skeletally mature had equal leg lengths. The average number of operations was 11 but ranged between 2 and 29. Most operations were for lengthening but younger children always needed revisions of the prosthesis. Functional scores were 77%.

Extendable endoprostheses are demanding both for the patient and the surgeon. The high complication rate should be decreased by non invasive lengthening prostheses.

All patients referred to our unit with previously untreated metastatic renal cancer were included in this review. We investigated likely prognostic factors including age, sex, site, synchronous or metachronous metastasis, stage of the disease and the type of treatment received.

From 1976 until 2004, a total of 198 patients were treated by our unit for renal metastases. 15 patients were excluded because they were referred after failure of previous treatment or only had advice. 96 patients were already known to have renal metastasis with their diagnosis having been made between 0.2 and 17 years from the diagnosis of primary cancer (mean 4 years). 33 patients presented to us with a pathological fracture and were found to have renal cancer. A total of 54 patients had multiple metastases and 129 had a solitary metastasis. The cumulative survival from the time of diagnosis of the bone metastasis is 70 percent at 1 year, 40% at 3 years and 18% at 5 years. In patients with a solitary metastasis, the overall survival was 74% at 1 year and 45% at 3 years, whereas in patients with multiple metastases it was 55% at 1 year and 22% at 3 years. (p=0.02) In patients with a solitary metastasis treated by excision of the metastasis, the survival at 1 year was 86% as compared to 38% for those that were treated with just a local procedure. Cox multivariate analysis shows that survival was better in those with solitary metachronus metastasis who underwent a radical procedure.

Background: There is currently no standard follow up protocol for patients who have been diagnosed with and treated for high-grade osteosarcoma. We therefore investigated the possibility of creating a risk based follow-up protocol for patients with primary osteosarcomas.

Methods: 313 patients diagnosed with primary osteosar-comas were studied. The identified risk factors for local recurrence included poor necrosis, inadequate margins and high risk tumour site in the bone. The risk factors for metastases were poor necrosis, inadequate margins, extracompartmental stage and tumour size ≥5cm.

Results: The risk of local recurrence and/or metastases within three years of diagnosis increases as the number of risk factors increase. Patients were grouped according to their number of risk factors. The cumulative risk of metastases for patients with 0, 1, 2, 3 and 4 risk factors is 0%, 12%, 21%, 54% and 60% respectively (p=< 0.0001). Risk of local recurrence for patients with 0, 1, 2 and 3 risk factors is 5%, 14%, 25% and 20% respectively (p=0.0025).

Conclusion: Our investigation shows that by grouping patients together according to their number of identified risk factors, it is possible to identify groups of patients that are most at risk. This information can be used to design an evidence based follow up protocol which would have important implications for clinical practice.

Introduction: Primary bones sarcomas account for 5% of childhood cancers; however the introduction of neo-adjuvant chemotherapy and the development of surgical techniques have resulted in reduced mortality and a longer length of survival. Consequently improving post operative functional outcomes has become an important focus of research. The aim of this study was to investigate and compare differences in the complications and functional outcomes of EPR and rotationplasty in skeletally immature patients.

Methods: This is a retrospective case control study of twenty-four patients, of whom twelve received rotation-plasty and twelve received EPR. Patients were selected at random and matched according to age at diagnosis, sex, site of disease and date of surgery. The Musculoskeletal Tumour Society (MSTS) score was used to evaluate functional outcome, and surgical complications were assessed qualitatively.

Results: Five patients (42%) treated with endoprosthe-ses experienced some form of post-operative surgical complication compared to three patients (25%) treated with rotationplasty. However this difference was not found to be statistically significant. The average MSTS score in the EPR cohort was 22.7 and 18.9 in rotation-plasty patients. Mann Whitney U testing confirmed this difference to be statistically significant (p=0.05).

Discussion: The study showed that patients who received EPR suffered more surgical complications than rotationplasty patients. However the results demonstrated superior functional outcomes in patients who received EPR.

The theoretical benefit historically attributed to rota-tionplasty lies in the provision of a functional and durable hinge joint, however these results suggest that this advantage has been negated by modern endoprostheses, probably due to improvements in surgical experience and prosthesis technology.

Conclusion: Our experience shows that patients with EPR are more likely to suffer more surgical complications but have similar, if not better functional outcomes compared to rotationplasty.

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.