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. Patients were identified from the ROH database who had been treated with a new presentation of myxofibrosarcoma. The size, grade and depth of the tumour at presentation was noted. Those patients who had suffered local or distal disease relapse or died were highlighted. Analysis was undertaken by Kaplan Meier survival curves for univariate and cox regression for multivariate analysis.Introduction
Methods
The National Institute of Clinical Excellence (NICE) published clinical guidelines in 2006 defining urgent referral criteria for soft tissue sarcoma to help improve the diagnostic accuracy and overall outcome. Despite these guidelines inadvertent excisions of soft tissue sarcomas continue to occur with alarming frequency potentially compromising patient outcomes. We reviewed the East Midlands Sarcoma Service experience of treating inadvertent excision of STSs and highlight the patient profile, referral pattern, subsequent management and oncological outcome associated with inadvertent resection.Background
Objective
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.
Sixty eight consecutive patients underwent proximal humeral replacement with a fixed fulcrum massive endoprosthesis for tumour between 1997 and 2007. The mean age was 46 years (7-87). Ten patients were lost to follow up and 16 patients died. The 42 surviving patients were assessed using the Musculoskeletal Tumour Society (MSTS) Score and the Toronto Extremity Salvage Score (TESS). The mean MSTS score was 72.3 % and the mean TESS was 77.2 %. Four of 42 patients received a new constrained humeral liner to reduce the risk of dislocation. This sub group of 4 patients had a mean MSTS score of 77.7 % and a mean TESS of 80.0%. Endoprosthetic replacement for tumour of the proximal humerus using this prosthesis is a reliable operation yielding good functional results without the documented problems of unconstrained prostheses. The performance of this prosthesis is expected to improve further with the new constrained humeral liner.
To look at the effect of width and tissue at surgical margins, together with the use of adjuvant therapy on locally recurrent disease and disease free survival. The distance (mm) and tissue has been regularly reported for 5 years, prior to this it was reported as wide, marginal or intra-lesional. It is known from previous studies that 90% of locally recurrent disease for osteosarcoma and Ewing's occurs within 2 years from surgery. Therefore all primary bone sarcomas of the pelvis and appendicular skeleton that underwent surgery between 1/1/2005 and 1/1/2008 were entered, giving at least 2 years follow up. The pathology records were reviewed and the margins and percentage tumour necrosis were recorded. Those patients who had suffered local or distal disease relapse or died were highlighted. Analysis was undertaken by Kaplan Meier survival curves with univariate and multivariate analysis.Introduction
Methods
Fibromatosis is a disorder characterised by a spectrum of biological behaviour from relative indolence to aggressive local infiltration. With aimed to describe the pre and post-operative functional status of these patients managed with surgery and analyse the effect of radiotherapy on functional outcome. 43 patients were analysed in the upper and lower limb fibromatosis database in which functional data was available pre-op and at a minimum of two years post-op. Any plantar, palmer, chest or abdominal lesion was excluded as were hormonal or chemotherapy treated patients.Introduction
Methods
To evaluate the rise in impedance during percutaneous radiofrequency thermo coagulation (PRFTC) of osteoid osteomas as a predictor of local recurrence. A prospective study of 23 patients (24 PRFTC procedures) with minimum of 2.25-year follow-up (average 3.3 years). Average age 19.6 years (range 4–44), sex ratio 15□:□8 (male□:□female), 16 non-diaphyseal, 7 diaphyseal.Objective
Design and Patients
It is generally held that blood tests are not particularly helpful in establishing the diagnosis of bone tumours but may be useful in prognosis. We reviewed the results of blood tests taken at the time of diagnosis to establish the frequency of abnormalities in common blood tests and whether this was significant in staging or prognosis. Blood test results on all newly diagnosed patients with bone tumours from 2005 – 2010 were exported and abnormalities identified. This was matched to diagnosis, clinical features and prognosis.Introduction
Method
Enchondromas located in the phalangeal bones may be more cellular than non-digital locations necessitating clinical and radiological correlation to determine diagnosis. Atypical enchondromas have increased cellularity and atypia relative to simple enchondromas but no evidence of permeation. Chondrosarcomas of the phalanges are thought to have a more indolent course than chondrosarcomas in other locations. The aim of the study was to determine the outcome of atypical enchondromas and grade 1 chondrosarcomas of the phalanges treated surgically. Data was collected prospectively on patients with a cartilage lesion of the phalanges. Typical enchondromas, grade 2 or 3 chondrosarcomas and patients with Ollier's disease were excluded.Introduction
Methods
A GP may only encounter one sarcoma in their professional career. Early diagnosis and treatment will improve the outcomes of this rare and malignant disease. Guidelines designed to lead to earlier diagnosis of the most common cancers were introduced by NICE in 2000 and were updated in 2005. These advise and assist in the early referral of potential bone and soft tissue sarcoma. Prior to 2000 only ~10% of GP referrals were proven to be malignant. In a referral region of ∼3m, we reviewed the referral patterns of suspected sarcoma by General Practitioners since 2005 in an effort to determine whether the published NICE guidelines had influenced an improvement in the diagnosis and management of malignant disease.Introduction
Methods
Earlier diagnosis is one of the key aims in achieving improved outcomes for patients with cancer. In general, the earlier a tumour is diagnosed, the easier it will be to treat and the greater the chance of cure. We have investigated how tumour size at diagnosis and duration of symptoms, both of which may act as a proxy for delay in diagnosis have varied over a 25 year period and whether there is evidence of improvement. Data were available for 2568 patients with primary bone sarcomas and 2366 with soft tissue sarcomas. The mean size at diagnosis was 10.7 cm for bone tumours and 9.9cm for soft tissue sarcomas. The size of bone sarcomas had not changed with the passage of time but there had been a slight decrease in the size of soft tissue sarcomas (10.3 cm before 2000 vs 9.6cm after 2000, p=0.03). The duration of symptoms reported by patients varied widely with a median of 16 weeks for bone sarcomas and 26 weeks for soft tissue sarcomas. The median duration of symptoms for bone sarcomas had actually increased since 2000 (16 weeks before to 20 weeks after 2000, p⋋0.01), whilst it remained unchanged for soft tissue sarcomas. Further analysis showed that females tended to present with smaller tumours than males and that slower growing tumours (eg. liposarcoma and chondrosarcoma) tended to be larger and have a longer duration of symptoms than other tumours. 15% of patients with a soft tissue sarcoma had undergone a previous inadvertent excision – and this % has not changed over 20 years. Younger patients had smaller soft tissue soft tissue sarcomas than older patients but there was little difference for bone sarcomas. This data shows there is huge room for improvementConclusion
Our Unit has been treating large volume soft tissue sarcomas involving the sciatic nerve with epineurectomy for over a decade. The aim of this study was to quantify the functional outcome of patients who were known to have sciatic nerve involvement pre-operatively and went on to have nerve preserving surgery utilising a planned marginal excision with epineurectomy. 20 patients with soft tissue sarcomas involving the sciatic nerve were studied treated between 1997 and 2010. Nineteen underwent surgery with extended epineurectomy of the sciatic nerve and planned marginal excision. All patients underwent staging and follow up at our Sarcoma Clinic with functional assessment and TESS evaluation.Introduction
Methods
Periarticular metastasis may be treated with endoprosthetic reconstruction. The extensive surgery required may not, however, be appropriate for all patients. Our aim was to establish if the outcome of locking plate fixation in selected patients with periarticular metastases. Prospective data collection was performed. Twenty one patients underwent surgery for periarticular metastatic tumours. The median duration of follow-up for surviving patients was one year. There have been no cases of implant failure and no requirement for revision surgery. Pain relief was excellent or good in the majority of patients. Patients who had sustained a fracture prior to fixation had restoration of their WHO performance status. All patients had a dramatic improvement in their MSTS scores. The median pre-operative score was 15% (0%-37%) improving to a median score of 80% (75% -96%) post operatively. Locking plates provide reliable fixation and excellent functional restoration in selected patients suffering from periarticular metastatic bone disease.
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. All patients with newly diagnosed primary chondrosarcoma of bone and without metastases at the time of diagnosis were identified from a prospective database. Their outcome was investigated to assess whether the margin of excision affected outcome. 492 patients were included in the analysis with a mean age of 48. The mean tumour size was 11cm and 59% were male. The 10 year tumour specific survival was 85% for clear cell (N=7) and grade 1 (N=210), 60% for grade 2 (N=180), 47% for grade 3 (N=59) and 16% for dedifferentiated (N=36). The 10 year local recurrence free survival was 86% (clear cell), 73% (grade 1), 67% (grade 2), 36% (grade 3) and 56% dedifferentiated. Local recurrence was strongly related to older age (p=0.0065), grade (p⋋0.0001) and margins (p⋋0.0001). Patients who developed local recurrence had a 43% survival at 10 years compared with 76% for those who did not (p⋋0.0001). They also had a 49% risk of developing metastases compared to a 17% risk for those without local recurrence (p⋋0.0001). However most of these patients had metastases before developing LR(57%). 16 of 65 patients with local recurrence but no metastases, died due to local progression (usually pelvic or spinal tumours). There was however no relation of the margin of excision to either the risk of developing metastases or survival for any grade of tumour.Method
Results
Fibromatosis represent a highly heterogeneous group of tumours in growth pattern, location and management. Our aim was to describe the demographics of the patient population who had undergone surgical resection and to identify predictors of local recurrence. Any lesion that was infiltrating the chest or abdominal cavity was excluded. Patients were also not included if they had a plantar or palmar lesions or had received hormonal or chemotherapy. 67 men and 88 women aged from 16 to 77 with a median age of 39 were analyzed. 121 patients had no prior resective operative intervention. 34 patients had undergone an attempted resection procedure at another unit of which 30 had locally recurred. 3 were located in the abdominal wall, 5 chest wall, 15 paraspinal, 56 lower and 76 upper limb. 40 patients did not receive XRT, 18 in the post-operative period and 97 in the pre-operative period. 67 operations produced margin negative resection, 85 were positive and 3 in which the margin status was unknown. Follow-up ranged from 1 day post op to 23.3 years. 23 patients had a local recurrence. Following subsequent re-resections, the total number of patients who were alive with evidence of disease was 16. 6 pts had deceased. 149 were alive with no evidence of disease. No factors were found to be statistically significant for predicting local recurrence, including the use of radiation (0.06) and margin status (0.81). Although radiation, given either pre or post-operatively did trend towards preventing local recurrence (HR 0.40; 95% CI 0.15 to 1.06; p = 0.06). The retention of critical structures whilst resecting fibromatosis continues to be an appropriate management strategy, as local recurrence rates seem to be independent of margin status. Although not statistically significant, the use of XRT did tend towards reducing local recurrence.Conclusions
We analyzed the results of extracorporeal radiated (ECRT) autogenous tumour bone for reconstruction of diaphyseal defects after tumour resection at our institute. Sixteen diaphyseal bone tumours operated between March 2006 to March 2008 were reconstructed with ECRT bone after appropriate oncologic resection. These included 10 cases of Ewing's sarcoma, 5 of Osteosarcoma and 1 Adamantinoma. Nine involved femur, 5 tibia and 2 humerus. Suitable internal stabilisation (14 cases plate fixation, 2 intramedullary nails) was used after re-implanting ECRT bone. Average resection length was 18.9 cm (range, 11 to 30 cm).Introduction
Methods
To identify factors leading to the success or failure of extracorporeal irradiation and re-implantation of bone (ECIR). Review of experience of this technique since 1996 documenting successes and failures.Aim
Method
The purpose of this study was to compare the outcome, complications and survival of the three most commonly used surgical reconstructions of the proximal humerus in adult patients after trans-articular tumour resection. Between1985 and 2005 thirty-eight consecutive proximal humeral reconstructions using either, allograft-prosthesis composite (n=10), osteoarticular allograft (n=13) or a modular tumour prosthesis (n=14), were performed in our clinic. Their mean follow-up was ten years (nine months to 25 years). Of these, twenty-seven were disease free at latest follow-up (Mean follow-up 16.8 years) and ten had died of disease (4.2 years). Complications and implant survival with revision surgery as end-point are presented for the total group of patients, functional scores for surviving patients only. The endoprosthetic group presented the smallest complication rate of 21%, compared to 40% in the allograftprosthesis- composite and 62% in the osteoarticular allograft group. Only one revision was performed in the endoprosthetic group, in a case of shoulder instability. Infection after revision (n=3), pseudoarthrosis (n=2), fracture of the allograft (n=3), and shoulder instability (n=4) were major complications of allograft use in general. Kaplan-Meier-analysis showed a significantly better implant survival for the endoprosthetic group (log-rank p=0.002). At final follow-up the MusculoSkeletal Tumour Society scores averaged at; 72% for the allograft-prosthetic-composite (n=7, mean follow-up 19 years), 76% for the osteoarticular allograft (n=3, 16 years), and 77% for the endoprosthetic reconstruction (n=10, 6 years) groups.Method
Results
In proximal humerus reconstructions, osteoarticular massive allograft (OA) allows a biologic reattachment of tendineous structures of the host providing stability and functional recovery. Allograft-prosthesis composites (APC) are more technically demanding, but functional advantages with new prosthetic design are expected with lower fracture and failure rate with time. We compared the outcome of patients treated with OA reconstruction (group A) and patients treated with an APC (group B) after humerus resection for bone tumours. Thirty six patients treated between 1996 and 2009 were included in the study (18 group A; 18 group B). The diagnosis was a malignant bone tumour in 28 cases, an aggressive benign bone tumour in 6 and a bone metastasis in 2. Three total humerus resections were performed (two in group A and one in group B). Three different type of prosthesis were used (conventional, reverse and resurfacing). In group A, at an average follow-up of 66 months (2-116), 16 patients were continuously disease free. Ten patients (56%) had no complication; in 6 cases (33%) we observed a fracture of the graft and in 3 cases (17%) a pseudoarthrosis. In group B, at an average follow up of 44 months (3-164), 16 patients were continuously disease free. Thirteen patients (72%) had no complication, in 2 cases (11%) stem loosening, in 2 cases (11%) dislocation, in 1 case pseudoarthrosis and in 1 case resorption and detachment of allograft's great tuberosity was observed. Functional outcome was evaluated using MSTS scoring system: a mean score of 24 (13-30) in group A and 23 (15-27) in group B was observed. In our experience, proximal humerus APC showed functional results comparable to OA allograft with lower complication rate. In conclusion, in biologic reconstruction of the proximal humerus, APC seems to provide a more reliable solution than OA allograft at long term
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. Between 1977 and 2009, 80 male and 66 female patients aged 2 to 60 (mean, 18) years with Pelvic Ewing's Sarcomas were retrospectively reviewed from the Royal Orthopaedic Hospital Oncology Service Registry. Treatments included surgery, radiotherapy, chemotherapy, or any of them in combination. Event-free (from presentation to recurrence) and overall (from presentation to death/latest follow-up) survival rates were calculated using the Kaplan- Meier method. Influence of various factors (age at diagnosis, gender, tumour site, metastasis at presentation, surgery (and surgical margins), radiotherapy, and type of treatment on survival was assessed using SPSS 14.0 statistical software.Aim
Method
