The aim of this study was to investigate the local recurrence rate at an extended follow-up in patients following navigated resection of primary pelvic and sacral tumours. This prospective cohort study comprised 23 consecutive patients (nine female, 14 male) who underwent resection of a primary pelvic or sacral tumour, using computer navigation, between 2010 and 2012. The mean age of the patients at the time of presentation was 51 years (10 to 77). The rates of local recurrence and mortality were calculated using the Kaplan–Meier method.Aims
Patients and Methods
Bone tumours of the foot are rare, representing 3–6% of all bone tumours. Of these 15–25% are thought to be malignant. Obtaining clear surgical margins remains an important factor in improving outcome from tumours. However, the anatomical complexity of the foot can lead to an inadequate resection, particularly if the operating surgeon is attempting to preserve function. The aim of this paper is to identify the clinical course of patients suffering from malignant bone tumours of the foot. A prospective tumour registry over a 30 yr period was used to identify patients with a malignant bone tumour of the foot. Patient demographics along with the site of primary malignancy, region of the foot involved and clinical management were recorded.Introduction
Method
The aim of this study was to evaluate the prognostic
and therapeutic factors which influence the oncological outcome
of parosteal osteosarcoma. A total of 80 patients with a primary parosteal osteosarcoma
were included in this retrospective study. There were 51 females
and 29 males with a mean age of 29.9 years (11 to 78). The mean follow-up was 11.2 years (1 to 40). Overall survival
was 91.8% at five years and 87.8% at ten years. Local recurrence
occurred in 14 (17.5%) patients and was associated with intralesional
surgery and a large volume of tumour. On histological examination,
80% of the local recurrences were dedifferentiated high-grade tumours.
A total of 12 (14.8%) patients developed pulmonary metastases, of
whom half had either a dedifferentiated tumour or a local recurrence.
Female gender and young age were good prognostic factors. Local
recurrence was a poor prognostic factor for survival. Medullary
involvement or the use of chemotherapy had no impact on survival. The main goal in treating a parosteal osteosarcoma must be to
achieve a wide surgical margin, as inadequate margins are associated
with local recurrence. Local recurrence has a significant negative
effect on survival, as 80% of the local recurrences are high-grade
dedifferentiated tumours, and half of these patients develop metastases. The role of chemotherapy in the treatment of parosteal osteosarcoma
is not as obvious as it is in the treatment of conventional osteosarcoma.
The mainstay of treatment is wide local excision. Cite this article:
The purpose of this study was to evaluate the
long-term outcome of patients with a sacral chordoma and the surgical
management of locally recurrent disease. Between October 1990 and August 2013 we operated on 54 consecutive
patients with a sacral chordoma. There were 34 men and 20 women
with a mean age of 60 years (25 to 86). The mean maximum diameter
of the tumour was 9.3 cm (3 to 20). The mean follow-up was 7.8 years (2 months to 23.4 years). The
disease-specific survival was 82% at five years, 57% at ten years
and 45% at 15 years. The local recurrence-free survival was 49%
at five years, 37% at ten years and 20% at 15 years. Local recurrence
occurred in 30 patients (56%) at a mean of 3.8 years (3 months to
13 years) post-operatively. Survival after the treatment of recurrence was 89% at two years,
56% at five years and 19% at ten years. Of nine patients who had
complete resection of a recurrence, one died after 72 months and
eight remain disease-free. Incomplete resection of recurrent disease
resulted in a survival of 54% at two years and 36% at five years. For 12 patients with a local recurrence who were treated palliatively,
survival was 81% at two years and 31% at five years. A wide margin of resection gave the best chance of long-term
survival and complete resection of local recurrence the best chance
of control of disease. Cite this article:
Bone sarcomas are rare cancers and orthopaedic
surgeons come across them infrequently, sometimes unexpectedly during
surgical procedures. We investigated the outcomes of patients who
underwent a surgical procedure where sarcomas were found unexpectedly
and were subsequently referred to our unit for treatment. We identified
95 patients (44 intra-lesional excisions, 35 fracture fixations,
16 joint replacements) with mean age of 48 years (11 to 83); 60%
were males (n = 57). Local recurrence arose in 40% who underwent
limb salvage surgery Cite this article:
We reviewed the outcome of patients who had been
treated operatively for symptomatic peri-acetabular metastases and
present an algorithm to guide treatment. The records of 81 patients who had been treated operatively for
symptomatic peri-acetabular metastases between 1987 and 2010 were
identified. There were 27 men and 54 women with a mean age of 61
years (15 to 87). The diagnosis, size of lesion, degree of pelvic
continuity, type of reconstruction, World Health Organization performance
status, survival time, pain, mobility and complications including
implant failure were recorded in each case. The overall patient survivorship at five years was 5%. The longest
lived patient survived 16 years from the date of diagnosis. The
mean survival was 23 months (<
1 to 16 years) and the median
was 15 months. At follow-up 14 patients remained alive. Two cementoplasties
failed because of local disease progression. Three Harrington rods broke:
one patient needed a subsequent Girdlestone procedure. One ‘ice-cream
cone’ prosthesis dislocated and was subsequently revised without
further problems. We recommend the ‘ice-cream cone’ for pelvic discontinuity
and Harrington rod reconstruction for severe bone loss. Smaller
defects can be safely managed using standard revision hip techniques. Cite this article:
We present our experience of the coned hemi-pelvis (‘ice-cream’ cone) implant, using an extended posterior approach to the hip joint, in the management of pelvic bone loss and pelvic discontinuity. Retrospective study conducted utilising a prospectively collected database. Patients who underwent an ice-cream cone reconstruction between August 2004 – September 2011 were identified. All had a posterior approach to the hip. Femur prepared in the standard fashion. A variety of femoral components used. Demographic data was recorded along with the indication for surgery and outcomes.Introduction
Methods
To estimate the risk of bone malignancy arising in premalignant conditions. There are quite a number of possible premalignant conditions with considerable uncertainty about the actual risk of a bone sarcoma developing. The incidence of these malignant conditions was identified from a prospective database containing 3000 primary bone sarcomas.Aim
Methods
After 25 years in orthopaedic oncology the author wishes to set a challenge for the next generation by posing 10 questions which he believes still do not have answers and which may improve outcomes for patients with sarcomas Why are sarcomas diagnosed so late? Can we ever decide what is a safe margin? What is the role of neoadjuvant chemotherapy for STS? What can we do to decrease the risk of infection after limb salvage surgery? What is the significance of local recurrence on outcome? What really is the best treatment for Ewing's sarcoma of the pelvis? Is cross sectional imaging essential as part of patient follow up? Is it possible to evaluate outcomes combining survival and function? Why can't we run a surgical trial in orthopaedic oncology? How can we evaluate surgical success? The author suggests ways these questions may be answered.
It is generally accepted that there is a high rate of local recurrence following surgical excision of chordoma of the sacrum, even if the margins of excision appear clear. There is uncertainty as to whether the addition of postoperative radiotherapy may decrease the risk of local recurrence, particularly if there are close or involved margins. We aimed to determine the effect of conventional radiotherapy, in the post-operative setting, on the effect of local recurrence, metastases and patient survival in a multi-centre study. 57 patients were identified from the combined databases of the RNOH and ROH, who underwent surgical excision of a primary sacral chordoma and who had a minimum of three years follow-up. There were 17 women and 40 men, with a median age of 64 (25-81 range). Median tumour length was 10cm (2-20 range). 22 of the 57 patients died. Survival was 60% at 5 years and 45% at ten years. 28 of the 57 patients developed local recurrence (49%) and margins of excision did not affect the rates of local recurrence. Adjuvant radiotherapy was used in 9 patients, to with intra-lesional resections, five marginal and two wide. Without radiotherapy the local recurrence rate was 45% at five years. With radiotherapy, the local recurrence rate was 62.5% at five years. There were no statistically significant differences between the two.Methods
Results
The aim of this study is to evaluate the functional and oncological outcome of extracorporeally irradiated autografts as a method of pelvic reconstruction after internal hemipelvectomy. The study included fifteen patients with primary malignant bone tumours of the pelvis. There were 10 males and 5 females with a mean age of 21.5 years (range, 8 to 46 years). Six patients had Ewing's sarcoma, six osteosarcoma, and three chondrosarcoma.Introduction
Methods
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.
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. Detailed curettage has been the principal treatment for GCT for the past 30 years. Cement was used from 1996 onwards for tumours where there was concern about structural stability. We investigated factors affecting LR and also the incidence of complications for treatment with or without cement.Introduction
Methods
To investigate the effectiveness of silver coated prostheses in preventing periprosthetic infection in a high-risk group. We have used silver coated prostheses in 48 endoprosthetic replacements in whom there would be a high expected risk of infection.Aim
Methods
Current practice in the UK is to perform clinical follow up and CXR on patients with sarcomas with the use of cross-sectional imaging reserved for high clinical suspicion of recurrence. We aimed to assess the effectiveness of this policy by investigating patients who developed local recurrence (LR) after previous treatment of a bone or soft tissue sarcoma (STS). Additionally, we aimed to determine whether size and extent of LR at diagnosis of recurrence affected subsequent management. We reviewed clinical records and imaging of all patients in the previous 5 years (01/01/2005–01/07/2010) with diagnosed LR to investigate: how it was diagnosed, site and size of recurrence and management. A value judgement was made as to whether earlier diagnosis may have altered treatment and/or outcome.Introduction
Method
Death within one year has been shown for some cancers to be an indicator of late presentation. They may have widespread disease by the time of diagnosis and many will have experienced delays in diagnosis. The aim of this study is to identify if this holds true for patients with bone and soft tissue sarcomas. A prospective sarcoma database was used to identify all patients with a newly diagnosed bone or soft tissue sarcoma and to compare those who had died within one year with those who had survived for at least that time.Background
Method
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 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.
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
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
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
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
To evaluate functional and oncological outcomes following sacral resection Retrospective review of 97 sacral tumours referred to spinal or oncology units between 2004 and 2009.Objective
Methods
Infected mega-endoprostheses are difficult to treat with systemic antibiotics due to encapsulation of the implant by fibrous tissue, formation of biofilms and antibiotic resistant bacteria. Modifying the implant surface by incorporating a bactericidal agent may reduce infection. Infection rates are typically in the range of 8% to 30%. This study describes a novel process method of “stitching-in” ionic silver into the implant surface, A novel process has been developed to “stitch in” ionic silver into the upper surface of titanium alloy (Ti6Al4V). The process produces a modification by anodisation of the titanium alloy in dilute phosphoric acid, followed by absorption of silver from an aqueous solution. The engineered surface modification is therefore integral with the substrate and loaded with silver by an ion exchange reaction. Using this technique the maximum inventory of silver for typical a mega-prosthesis is 6mg and this is greater than 300 times lower than the No Observable Adverse Affects Level (NOAEL). Scanning electron microscopy revealed that the silver was concentrated in pits and forming reservoirs of ionic silver exposed to the body tissues. Laboratory-based studies focusing on the safety and efficacy of silver as a bactericidal agent have included investigation into cytotoxicity using fibroblast and osteoblast cell lines, the impact of silver in reducing corrosion and laboratory testing to establish if the modified surface has an effect on the wear and mechanical characteristics. A range of fatigue, static, tensile pull off tests were performed. The silver elution profiles for both silver loaded and HA coated over a silver loaded surface have been examined. Histological studies were also performed to examine the impact of the silver on osseointegration. The To date (May 10) 147 silver treated mega-prostheses have been implanted since March 2006. The majority of implants were distal femoral (29%), proximal tibial (23%) or hemiplevic (10%). The most common indication was revision of a failed limb salvage reconstruction (58%), with the dominant cause of failure being infection. The next most common indication was bone tumour (31%) and the large majority were used in the high risk skeletal locations of the tibia (44%) and the pelvis (27%). Early clinical results are encouraging indicating a significant reduction in the incidence of infection. Three implants have been retrieved. An analysis of a proximal humeral replacement that had been This novel process of “stitching-in” silver appears to be a safe and effective surface treatment in helping to control infections of mega-prostheses. This technology has the potential to be transferred to other arthroplasty joints.
To evaluate functional and oncological outcomes following resection of sacral tumours and discuss the strategies for instrumentation. Primary malignant tumours of the sacrum are rare, arising from bony or neural elements, or bone marrow in haematological malignancies. Management of such lesions is dictated by anatomy and the behaviour of tumours. Three key issues which arise are the adequacy of tumour resection, mechanical stabilisation and the need for colostomy. Stabilisation is often extensive and can be challenging.Objective
Introduction
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
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
The aim of this study was to investigate the results of a series of cases from a single institution with respect to local disease control and patient survival to determine prognostic factors. Electronic patient records were reviewed on all patients with STS between February 1963 and January 2007. 2445 patients had over 30 types of STS. 1639 (67%) had not received any treatment prior to presentation, however, 770 patients (32%) had undergone a previous attempted excision. Survival analyses were done using Kaplan Meier and Cox regression analyses, however, for prognostic factor analysis, only patients presenting without prior treatment were included.Introduction
Methods
Neoadjuvant chemotherapy for osteosarcoma improves outcomes for the majority, but if the chemotherapy does not work then the dilemma often arises as to whether to do limb salvage with a marginal (or worse) margin of excision or to do an amputation. If limb salvage is carried out with a close margin, does post operative radiotherapy make any difference? This study aims to address these questions. All patients with limb osteosarcoma, no metastases, a poor response to chemotherapy and either a marginal excision or primary amputation were identified from a prospective database. This group were investigated in terms of overall survival and local control. There were 182 patients in this category of whom 60 had an amputation, 105 limb salvage with marginal margins and 17 with an intralesional margin. Local recurrence (LR) arose in 41% of those with an intralesional margin, 22% of those with a marginal margin and 13% of those with an amputation. Radiotherapy was used in 21 of the 122 patients and the risk of LR was the same as in those who did not have radiotherapy. Neither age nor sex of the patient, size or site of the tumour affected the risk of LR. The overall survival for this group was 42% at 10 years. The survival was best in those with marginal margins (38%) than those with an amputation (28%) and worst for those with an intralesional margin (20%). Survival was worst in those who did develop LR, but no worse than in those having amputationMethod
Results
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 A retrospective analysis of 650 patients who had a diagnosis of Ewing's sarcoma treated between 1975 and 2009 at a single institution was performed and 64 patients (10%) who had a local recurrence were identified and analysed. Fifteen patients had metastases at diagnosis.20 patients had chemotherapy and radiotherapy only while 44 had chemotherapy and surgery +/− post op radiotherapy. Thirteen patients who were suitable for post –operative radiotherapy could not receive the treatment due to various reasons like biological reconstruction. The estimated 5 years survival for the patients was 15%. The risk of local recurrence is higher if the tumour is located in the axial skeleton, treatment with chemotherapy and radiotherapy alone [location and size of the tumour precluding surgery]. The risk of local recurrence is higher if the tumour was in the fibula or radius. One out of three patients who have good response to chemotherapy still went on to develop a LR. The use of biological reconstruction and younger age group often resulted in deferral of post-operative radiotherapy. Location and type of treatment can predict LR. Surgery with clear margins and post-operative radiotherapy given when indicated may reduce the incidence of LR.Methods
Results
Bone and Soft Tissue Sarcomas represent approximately 1% of all malignant tumours. Delays in diagnosis are frequent and the average size of Sarcomas at diagnosis has averaged 10cm for many years. In 1999 guidance was produced by NICE with the aim of leading to the earlier diagnosis of common cancers – including Sarcomas. We have attempted to analyze whether this guidance has had any impact on either the size of the tumours at diagnosis or the symptom duration prior to diagnosis experienced by the patients. Data for patients referred to the Royal Orthopaedic Hospital in Birmingham between 1992 and 2007 with Bone Sarcomas (n=1592) and Soft Tissue Sarcomas (n=2004) were analysed to determine the effect of the guidance. For Bone Sarcomas the mean size of the tumours decreased from 11.2cm prior to the guidance to 10.7cm after the guidance but the change was not statistically significant (p=0.09). The mean duration of symptoms increased from 18 to 21.2 weeks (p=0.01). For Soft Tissue Sarcomas, mean size fell from 10.8cm to 9.5cm (p<0.001), however the duration of symptoms actually increased from 27.3 to 32.1 weeks (p=0.01). Statistical modelling using restricted cubic splines confirmed these trends in the data. These results show that whilst there may have been a slight improvement in the size at diagnosis of Soft Tissue Sarcomas, overall most patients still experience a long delay between the onset of symptoms and diagnosis and commencement of treatment. It is difficult to conclude that the early diagnosis guidance produced in 1999 has had a significant effect on the basis of this study. Strategies to improve awareness of the symptoms and clinical features of Bone and Soft Tissue Sarcomas are still urgently required.
Recently a great deal of interest has emerged in new techniques for resection of bone tumours, such as the use of computer guided surgery, joint sparing prostheses and epiphysiolysis. However, all the techniques may require narrower margins at resection than the traditional Enneking wide margins. The aim of the study was 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 quality in terms of precise distance (mm) and tissue has been regularly reported by our pathologists for 5 years, prior to this it was reported as wide, marginal or intralesional. It is known from previous studies that 90% of locally recurrent disease for osteosarcoma and Ewings 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 into the study group, giving at least 2 years follow up. The pathology records were reviewed and the quality of 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
To evaluate functional and oncological outcomes following resection of primary malignant bone tumours. Primary malignant tumours of the sacrum are rare, arising from bony or neural elements, or bone marrow in haematological malignancies. Management of these lesions is dictated by anatomical considerations and the behaviour of tumours. The three key issues which arise are the adequacy of tumour resection, mechanical stabilisation and the need for colostomy. A retrospective review of the surgical management of primary malignant sacral tumours from 2004 - 2009. The study included 46 patients (34 males, 12 females) with an average age of 49 (range 7 – 82). Median duration of symptoms before presentation was 26 months. 10 patients had inoperable tumours at presentation. 6 patients had chemotherapy. 2 patients opted for palliative radiotherapy. 1 patient was unfit for surgery. 25 patients (54%) underwent surgical resection. 8 underwent instrumented stabilisations with fibula strut graft vs. 17 uninstrumented. Colostomy was performed in 10 patients (40%). Mean follow post-operatively was 19.0 months. Wound healing problems were present in 5/25 (20%). There was no difference in infection rates between definitive surgery with and without colostomy. Mechanical failure of stabilisation was noted in 75%. There was one peri-operative death. Local recurrence occurred in 12%(3/25) of operated patients although follow-up period was noted to be short. Mechanical stabilisation for extensive lesions in the sacrum are particularly challenging in tumour surgery. Despite radiological failure in 7/8 instrumented stabilisations, patients were relatively asymptomatic and only 1/8 required revision stabilisation surgery. Ethics approval: None: Audit Interest Statement: None
The pelvis has always been a difficult area for surgeons, with high complication rates from surgery and the perception of poor oncological outcomes. The aim of the study was to look at the surgical and oncological outcomes of pelvic tumours treated at our centre. From the 3100 primary bone tumours seen at the ROH. Information was retrieved on 539 patients seen with a primary bone tumour of the pelvis. The demographic details, oncological and surgical outcomes were reviewed.Introduction
Methods
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.
Bone tumours rarely involve the joint surface as cartilage is thought to be a good barrier to tumour spread. When the tumour does cross the surface the surgeon is faced with the dilemma of whether to amputate the limb, resect it without reconstruction or reconstruct with an implant. This paper aims to investigate the oncological and functional outcomes of patients undergoing an extra-articular resection and reconstruction with an endoprosthesis. 3100 patients have been seen in ROH with primary bone tumours. Patients were identified who had an extra-articular resection considered pre-operatively and the notes and imaging was reviewed. This group was subdivided into a group who did have an extra-articular resection (EAR) and those who either had an amputation or traditional through joint resection. The outcomes of the three groups (group 1 = no joint involvement, group 2 = EAR considered but not done and group 3 = EAR) were then compared in terms of oncological outcome, surgical margins and complications.Introduction
Method
Pigmented villonodular synovitis is a monoarticular proliferative process most commonly involving the synovium of the knee joint. There is considerable debate with regards to diagnosis and effective treatment. We present our experience of managing PVNS of the knee joint over a 12 year period. Twenty-eight patients were reviewed. MRI was used to establish recurrence in symptomatic patients rather than routine screening and to identify posterior disease prior to surgery. Eight patients had localised disease and were all treated with open synovectomy and excision of the lesion, with no evidence of recurrence. Twenty patients had diffuse disease, eight treated arthroscopically and twelve with open total synovectomy. Nineteen patients (95%) had recurrence on MRI, however, only five (25%) had evidence of clinical recurrence. There were no significant complications following arthroscopic synovectomy. Open synovectomy, in contrast, was associated with three wound infections and two thrombo-embolisms. Three patients had Complex regional pain syndrome. We believe diffuse disease should be treated with arthroscopic synovectomy which is associated with minimal morbidity and can be repeated to maintain disease control. Radiotherapy is helpful in very aggressive cases. TKR was used when there was associated articular erosion.
To assess the affects of a delay in diagnosis on the survival rates of Bone Sarcoma (BS) using size and symptom duration as measures of delay. All patients diagnosed with a Primary BS from 1970 to 2005 were included. Demographic data concerning age, sex, diagnosis and tumour site were recorded. The data were collected retrospectively from a prospective database, with 2573 patients included. Symptom duration is defined as the length of time the patient was experiencing symptoms for until the date of diagnosis. Survival analysis was performed using Kaplan-Meier curves and Cox Regression was carried out to identify variables affecting outcome.Objectives
Methods
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.
Sarcomas are a rare group of tumours, which pose numerous problems regarding correct diagnosis and appropriate management. This study aimed to examine whether symptom duration and tumour size at diagnosis have changed over time, using size and symptom duration as methods of comparison. All patients diagnosed with sarcoma were identified retrospectively from a prospective database from 1963 to 2005. Demographic data concerning age at diagnosis, sex and diagnosis were recorded. Data were also collected on duration of symptoms and size of tumour at diagnosis. Symptom duration is defined as the length of time the patient was experiencing symptoms for until the date of diagnosis. Changes in size and symptom duration over time were compared by grouping date of diagnosis into time periods and comparing them against each other using non-parametric statistical analysis.Objective
Methods
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 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.
We have investigated whether improvements in design have altered the outcome for patients undergoing endoprosthetic replacement of the distal femur following tumour resection. Survival of the implant and ‘servicing’ procedures have been documented using a prospective database and review of the implant design records and case records. A total of 335 patients underwent a distal femoral replacement with 162 having a fixed hinge design and 173 a rotating hinge with most of the latter group having a hydroxyapatite collar at the bone prosthesis junction. The median age of the patients was 24 years (range 13-82 yrs). With a minimum follow up of 5 years and a maximum of 30 years, 192 patients remain alive with a median follow-up of 11 years. The risk of revision for any reason was 17% at 5 years, 34% at 10 years and 58% at 20 years. One in ten patients developed an infection and 42% of these patients eventually required an amputation. Aseptic loosening was the most common reason for revision in the fixed hinge knees whilst infection and stem fracture were the most common reason in the rotating hinges. The risk of revision for aseptic loosening in the fixed hinges was 32% at ten years compared with nil for the rotating hinge knees with a hydroxyapatite collar. The overall risk of revision for any reason was halved by use of the rotating hinge, and for patients older than 40 years at time of implant. Improvements in design of distal femoral replacements have significantly decreased the risk of revision surgery. Infection remains a serious problem for these patients.Conclusion
EPRs are the treatment of choice following resection of tumours. These have been used for 39 years in our institution. There has been concern regarding the long term survival of endoprosthesis; this study investigates the fate of the reconstruction. Between 1966 and 1995, 3716 patients were seen with a suspected neoplasm and 776 patients underwent EPRs. Patients receiving growing endoprostheses were excluded from the study as they invariably require revision, leaving 667 replacements. Insufficient data was available in 6 cases, leaving 661 patients in the study group. Information was reviewed concerning the diagnosis, survival of implant and patient, subsequent surgery, complications and functional outcome. Kaplan-Meier survival analysis was used for implant survival with end points defined as revision for mechanical failure (aseptic loosening, implant fracture, instability, avascular necrosis, periprosthetic fracture, pain and stiffness) and revision for any cause (infection, local recurrence and mechanical failure). Mean age at diagnosis was 34 years. Overall patient survival was 52.7% at 10 years and 45.7% at 20 years. The mean follow-up for all patients was 9 years, and for those patients who survived their original disease, the mean follow-up was 15 years. 227 (34%) patients underwent revision surgery, 75 patients for infection (33%), 36 patients for locally recurrent disease (16%) and mechanical failure in 116 patients (51%). With revision for mechanical failure as the end-point, implant survival was 75% at 10 years and 52% at 20 years. With revision any cause as an end-point implant survival was 58% at 10 years and 38% at 20 years. Overall limb salvage was maintained in 91% of patients at 10 years from reconstruction and 79% at 20 years. There was a significant difference between survival of implant between implantation sites, with the proximal humeral implant survival being the best and tibial reconstructions being the worst. The MTSS functional score was available on 151 patients, with a mean score of 25/30 (83%) at last follow-up visit.Methods
Results
Current guidelines suggest that most lumps less than 5cm in size will not be malignant. We reviewed our experiences of small lumps at the Royal Orthopaedic Hospital, Birmingham, in order to try and identify factors associated with malignancy. All referrals to our clinic were included and data on size, symptoms, depth and diagnosis were analysed. We identified 325 patients, with 57 (17.5%) malignant and 268 (82.5%) benign lumps. Size >3cm was the most accurate predictor of malignancy (35.8% malignant vs 9.9% <3cm). 23.6% of lumps reported as increasing size were found to be malignant, compared to 8.6% of those that were not. Pain was found to be a poor predictor of malignancy, with only 15.5% of painful lumps found to be malignant and 18.9% of painless lumps found to be malignant. ‘Size >3cm’ has a sensitivity of 73.6% and a specificity of 56.3%, ‘Depth’ has a sensitivity of 73.6% and a specificity of 30.2%, ‘Increasing in Size’ a sensitivity of 71.4% and a specificity of 56.7% and ‘Pain’ a sensitivity of 33.3% and a specificity of 57.1%. If a lump has none of the above features then one can be 89.5% certain that it is not malignant. If it has 1 feature there is a 16.1% chance of its becoming malignant, 2 features a 58% chance, 3 features an 87% chance and if a lump has all 4 features there is a 93.5% chance of its being found to be malignant. The main limitation with this study is that the data set is skewed because all lumps referred to the Oncology Department are by their nature suspicious. This study identifies those factors which suggest malignancy in small lumps and provides the basis for carrying out a large, community-based study on all lumps, to identify features associated with malignancy.
Chondroblastomas arise in the epiphyseal area of bones. In the femoral head this can cause considerable difficulty in obtaining access as the epiphysis is entirely intra-articular. We have reviewed management and outcome of 10 patients with chondroblastoma of the femoral head to identify outcome and complications. The mean age was 14 years and all presented with pain (frequently in the knee) and a limp. All were diagnosed on plain Xray and MRI. Five younger children were treated by curettage by a lateral approach up the femoral neck (to try and minimise damage to the epiphysis) and five by a direct approach through the joint. Two of the five patients with a lateral approach developed local recurrence whilst none of the direct approaches did. Both local recurrences were cured with a direct curettage. One patient developed overlengthening of the leg by 1cm but there was no case of growth arrest or osteoarthritis. We recommend a direct approach to the lesion whenever possible to give the best chance of cure with a low risk of complications.
Despite the advances in adjuvant chemotherapy and surgical techniques, the diagnosis of a bone tumour still carries with it a significant risk of mortality. This study investigates factors affecting survival, in patients treated for malignant tumours of bone using Endoprosthetic replacement (EPR). Our tertiary referral musculoskeletal tumour unit has taken referrals over 40 years. Electronic patient records have been prospectively kept on all patients seen since 1986 and data has been entered retrospectively for patients seen between 1966 and 1986. A consecutive series of 1264 patients underwent endoprosthetic reconstruction; after 158 patients were excluded, 1106 patients were left in the study group. Factors including diagnosis, size of tumour, surgical margins, percentage tumour necrosis following chemotherapy, tumour site, local recurrence, decade of reconstruction, fracture and post-operative deep infection were analysed.Introduction
Methods
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. This large series of adductor compartment STS has shown that survival factors do not vary across international boundaries but that treatment factors affect complications, local recurrence and function. Centralisation of adjuvant treatment like radiotherapy may have an important role in improving local control.Conclusion
Propionibacteria are organisms of low virulence, although they do cause deep periprosthetic infections. The aim of this study was to show that Propionibacteria do not always cause a significant rise in ESR and CRP. Between May 2001 and May 2004, we identified 77 patients with prosthetic joint replacements colonised with Propionibacteria, 47 males and 30 females. There were 47 hip joint replacements, 27 knee joint replacements, 2 endoprosthetic replacements of the femur and 1 shoulder joint replacement. We retrieved successfully the medical records of 66 patients in order to identify the number of patients treated for an infected prosthetic joint arthroplasty. The pre-operative values of ESR and CRP were recorded. For the purposes of this study, an ESR rate of 30mm/hr or higher and a CRP level of 10mg/lt or higher were considered to be suggestive of infection and were deemed a positive result. All of the 77 patients had both ESR and CRP measured pre-operatively. In only 16 (21%) both ESR and CRP were higher than 30mm/hr and 10mg/l respectively. In 33 patients (43%) with prosthetic joint replacements colonised with Propionibacteria, the pre-operative values of ESR and CRP were normal. 23 patients were treated for an infected prosthetic joint arthroplasty. In 7 (30%) of those patients both ESR and CRP were normal. This suggests that normal pre-operative values of ESR and CRP in suspected failed prosthetic joint replacements might not exclude infection, if the causative organism is of low virulence such as Propionibacteria.
Fifty-six patients with stage II-B osteosarcoma around the knee were followed-up for a minimum of 92 months. The percentage of tumour cells expressing VEGF/MMP-9 was assessed using immunohistochemistry. The relationship between VEGF/MMP-9 expression and survival was assessed using Kaplan-Meier and Cox regression models. Patients with tumours expressing VEGF in >25% of their cells had shorter overall (p=0.019) and disease-free survival (p=0.009). Patients with tumours expressing MMP-9 had shorter overall (p=0.0042) and disease-free survival (p=0.0004). There was an association between VEGF and MMP-9 expression (p=0.021). The negative effects of VEGF/MMP-9 expression on survival were independent of traditional prognostic factors.
To identify patient, tumour or treatment factors that influence outcome in patients with radiation induced sarcoma of bone. A retrospective review of an oncology database supplemented by referral back to original records.Aim
Method
Dedifferentiated chondrosarcoma is a rare but highly malignant manifestation that can occasionally arise in patients with cartilage tumours. There remains uncertainty as to the best treatment for this condition and in particular whether chemotherapy may have a role in improving prognosis. Members of EMSOS were invited to contribute data on patients, tumours, treatment and outcomes of patients with dedifferentiated chondrosarcoma. Eight centres contributed data on 317 patients from 7 countries. The mean age was 59 (range 15 to 89) and the most common site was the femur (46%) followed by the pelvis (28%). 25% of patients presented with a pathological fracture and the most common high grade component identified was MFH. 23% had metastases at diagnosis and these patients had a median survival of 5 months. 30% of patients received chemotherapy, with 47% under 60 having chemotherapy compared with 10% over 60. One third of this group had neoadjuvant chemotherapy and the rest had adjuvant reatment. 88% had surgery with limb salvage in 80% of this group. The overall survival was 38% at 2 years and 24% at 5 years but in patients without metastases at diagnosis these figures were 44% and 28% respectively. Poor prognostic factors for survival were: metastases at diagnosis, amputation or no operation, local recurrence, age over 60 and pathological fracture at presentation. We were unable to identify any group in whom chemotherapy appeared to have a survival benefit. Dedifferentiated chondrosarcoma carries a dismal prognosis. Although 30% of patients received chemotherapy in this study we were not able to prove that it improved survival. Early diagnosis and complete surgical excision still offer the best prognosis for this condition.
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. We recommend a radical procedure for patients who present with a solitary renal metastasis, particularly those with a disease-free interval of more than one year.Conclusion
Non-invasive expandable prostheses for limb salvage tumour surgery were first used in 2002. These implants allow ongoing lengthening of the operated limb to maintain limb-length equality and function while avoiding unnecessary repeat surgeries and the phenomenon of anniversary operations. A large series of skeletally immature patients have been treated with these implants at the two leading orthopaedic oncology centres in England (Royal National Orthopaedic Hospital, Stanmore, and Royal Orthopaedic Hospital, Birmingham). An up to date review of these patients has been made, documenting the relevant diagnoses, sites of tumour and types of implant used. 74 patients were assessed, with an age range of 7 – 16 years and follow up range of 4 – 88 months. We identified five problems with lengthening. One was due to soft tissue restriction which resolved following excision of the hindering tissue. Another was due to autoclaving of the prosthesis prior to insertion and this patient, along with two others, all had successful further surgery to replace the gearbox. Another six patients required mechanism revision when the prosthesis had reached its maximal length. Complications included one fracture of the prosthesis that was revised successfully and six cases of metalwork infection (two of which were present prior to insertion of the implant and three of which were treated successfully with silver-coated implants). There were no cases of aseptic loosening. Overall satisfaction was high with the patients avoiding operative lengthening and tolerating the non-invasive lengthenings well. Combined with satisfactory survivorship and functional outcome, we commend its use in the immature population of long bone tumour cases.
The overall patient survival was 69% at 5 years and 62% at 10 years. Local recurrence arose in 4 cases, 3 of whom already had metastatic disease. None of the LR arose in the irradiated bone. The rate of non union was 9% at the 58 osteotomies, the greatest risk being in the tibia. There were four graft fractures of which 3 needed fixation and all united. There is one case of convincing graft resorbtion after 12 years. Two patients developed infections but there have been none since the done was routinely immersed in antibiotics whilst being irradiated.
The purpose of our study was to examine the survival and functional outcome of endoprosthetic replacements for non-oncology limb salvage purposes. Although initially designed for bone tumours, such is the versatility of these implants they can be used to salvage failed joint replacements, peri-prosthetic fractures, failed internal fixation and non-union. Thirty eight procedures were identified from September 1995 to June 2007 from a prospectively kept database, including 17 distal femoral replacements, 12 proximal femoral replacements, 4 proximal humeral replacements, 2 distal humeral replacements, 2 hemi-pelvic replacements and 1 total femoral replacement. The quality of patients’ mobility was used to assess functional outcome and the survival of the prosthesis was calculated using a Kaplan-Meier survival curve. The Kaplan-Meier implant survival was 91.3% at 5 years, 68.5% at 10 years and 45.7% at 20 years. The limb salvage survival for all reconstructions was 75% at 10 years. The best survival was as follows pelvic (n=0/2) and total femoral prostheses where there was no failure in either group (n=0/1). Distal femoral replacements survival was 91% at 5 years, a single humeral prosthesis failed at 11 years post surgery, and proximal femoral replacements had a survival at 87.5% at 5 years. Three implants failed, two as a result of infection and required staged revisions and 1 failed as a result of aseptic loosening. Two patients dislocated their proximal femoral replacements, both were treated successfully by closed reduction. Endoprosthetic replacement appears to be effective and the medium term survival is encouraging. The aim of a pain free functional limb is achievable with this technique. The complication rates are acceptable considering the salvage nature of these patients. We recommend referral of complex cases to a tertiary centre with expertise in this type of surgery.
We aim to establish the likely functional scores following different types of surgery for bone tumours. All living patients who had undergone treatment for a bone tumour were contacted and invited to complete a TESS self-report questionnaire. The TESS contains 32 items of day-to-day activities which the patient scores themselves against. The final score is a percentage, with 100% being ‘normal’. The project was approved by the local ethical committee. 723 responses were obtained from just over 1000 letters that were sent. Thirty seven sets of data were incomplete leaving a total of 686. There were 369 males and 317 females, with an average age of 49 (range 15 to 93). The mean interval from their initial operation was 12 years (range 1 to 42). 590 of the procedures were in the lower limb and 96 in the upper limb. 126 patients had an amputation and 560 limb salvage (82%). Mean scores obtained for each procedure were: EPR mid-femur 86%; EPR distal femur 77%; EPR proximal tibia 74%; EPR proximal humerus 71%; EPR proximal femur 71%; Below knee amputation 70%; EPR total femur 63%; Disarticulation of the hip 61%; Above knee amputation 60%; Hindquarter amputation 57%; EPR pelvis 54%. Younger patients had much better scores (80% for those under 30 vs. 69% for those over 30, p<
0.0001) than those over 30. Additionally, the scores for those that had had an amputation following a EPR procedure were no worse than those who had had an amputation originally. Significantly, EPR proximal tibia was better than AKA (78% vs. 67% p = 0.0003), as was EPR distal femur (80% vs. 67% p <
0.0001). This data from a large series is helpful in indicating likely outcomes following surgical treatment of sarcomas, both for patients and surgeons.
Patients who undergo soft tissue sarcoma excision accumulate serosanguinous fluid, potentially resulting in a seroma. This can lead to wound complications and delay post-op radiotherapy. The purpose of this preliminary report is to assess the impact of routine application of Tisseel sealant prior to closure. We investigated whether the sealant Tisseel is effective as a sealing agent to reduce the duration and volume of serosanguinous fluid drainage. Results were compared with individually matched controls. Patients were split into 2 groups: those receiving 10mls sealant and those not receiving the sealant. Efficacy was evaluated by the number of days required for wound drainage, the volume of fluid drainage and the length of stay compared with matched controls. The preliminary findings suggest that application of the sealant reduced the duration and quantity of fluid drainage after excision of the STS, allowing earlier discharge from hospital. We present this work to suggest that the members of the British Orthopaedic Oncology Society should consider using this in a randomised controlled trial setting to evaluate its efficacy nationally.
Chondrosarcoma is treated with respect by oncology surgeons, given that it is relatively resistant to chemotherapy and radiotherapy. The aim was to study the outcomes of surgery for chondrosarcoma and determine the role of initial surgical margins and local recurrence on outcome. Electronic patient records were retrieved on all patients seen with chondrosarcoma of bone with a minimum of two years follow up. A total of 532 patients were seen with Chondrosarcoma between 1970 and 2006. Patients were excluded if they had initial treatment in another unit (20 patients), a subdiagnosis of dedifferentiated chondrosarcoma (due to very poor prognosis, 43 patients), metastases at presentation (30 patients), if they presented with disseminated metastases prior to local recurrence (12 patients) or were not offered surgery, leaving 402 patients in the study group. The mean age was 48 years old (range 6–89 years) with the most commonly sites of presentation being in the pelvis in 132 patients (29%), proximal femur in 81 patients (18%), distal femur in 40 patients (9%) and proximal humerus in 40 patients (9%). Grade at presentation was grade 1 in 44%, grade 2 in 44% and grade 3 in 12%. Surgical margins were radical in 3%, wide in 44%, marginal in 29%, planned incisional in 13% and unplanned incisional in 11%. Local recurrence occurred in 87 patients (22%). Local recurrence rates were significantly different for surgical margins on Fisher exact testing (p=0.003), which held true even when stratified by presenting grade of tumour. Surgery for local control was successful in 62% of cases. Complex relationships exist between surgical margins, local recurrence and survival. Long term survival is possible in 1/3 patients who have local recurrence in intermediate and high grade chondrosarcomas and therefore ever effort should be made to regain local control following local recurrence.
We have compared the cost:benefit ratio of the new type of non invasive extendable prostheses with the old type which required lengthening under general anaesthetic with an invasive procedure. Over the past four years we have inserted 27 non invasive endoprostheses (cost £14,000). Two have failed to lengthen due to problems with the inbuilt motor. So far there have been no infections, no loosenings and no patient has required revision. The lengthenings are painless and take half an hour. In the past 25 years we inserted 175 extendable endoprostheses (cost £7,000). All lengthenings were performed under a general anaesthetic. The risk of infection was initially 20% at ten years but had decreased to 8%. Pain and stiffness arose in about 10% requiring physiotherapy or occasionally manipulation under anaesthetic. Assuming the following costs (current NHS cost) are accurate and appropriate, then the non invasive extendable prosthesis becomes cost effective when Cost EPR <
Cost old EPR + (Additional risk physio(P) x cost) + (additional cost x number of lengthenings (L)) + (additional cost of revision for infection x risk of infection (R)). 14000 <
7000 + (300 x P) + (1500 x L) + (20000 x R). Assuming a 10% need for physiotherapy, four lengthenings and a 10% risk of infection gives: 14000 <
7000 + (30) +(6000) + (2000) = 15030. Given the high complication rate of the old type of extendable procedure and assuming there are few if any with the non invasive type, then the non-invasive endoprostheses becomes cost effective if more than three lengthenings are required. They are certainly more popular with parents and children alike!
Pelvic reconstruction after tumour resection is challenging. Pelvic replacements are usually custom made at considerable expense and then need very careful positioning at the time of surgery. They have a very high rate of complications with up to 30% risk of infection and 10% dislocation. In 2003 we developed a new type of pelvic replacement which would be simple to make, simple to use and which would hopefully avoid the major complications of previous pelvic replacements whist being versatile to use even when there was very little pelvis remaining. The concept is based on the old design of Ring stemmed hip replacement and has become known as the ice-cream cone prosthesis. It is inserted into the remnant of pelvis or sacrum and is surrounded by bone cement containing antibiotics. One of the main advantages is it’s flexibility, allowing insertion after resection at a variety of levels. It is also suitable for patients with metastatic disease. We have inserted 12 of these implants in the past 4 years, resolving very difficult reconstruction problems. There was one case that became infected but was cured with washout and antibiotics. In one patient there was excessive leg lengthening resulting in a sciatic and femoral nerve palsy and the prosthesis had to be revised. All patients can walk with one stick or less. These results are encouraging and suggest that this versatile implant may be the way forward for pelvic reconstruction because of it’s flexibility of use and low complication rate.
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 (but 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.
Of 3000 patients diagnosed with primary malignant bone tumours and treated at our unit over the past 25 years, 234 (7.8%) were considered to be spindle cell sarcomas of bone (ie not osteosarcoma, chondrosarcoma, Ewing’s, chordoma or adamantinoma). We have analyzed their management and outcomes. The diagnosis of these cases varied with fluctuations in the popularity of conditions such as MFH, fibrosarcoma and leiomyosarcoma with the passage of time. Treatment was with chemotherapy and surgery whenever possible. 36 patients had metastases at diagnosis and 17 had palliative treatment only because of age or infirmity. The most common site was the femur followed by the tibia, pelvis and humerus. The mean age was 45 and the mean tumour size 10.2cm at diagnosis. 25% of patients presented with a pathological fracture. Chemotherapy was used in 70% of patients the most common regime being cisplatin and doxorubicin. 35% of patients having neoadjuvant chemotherapy had a good (>
90% necrosis) response. The amputation rate was 22% and was higher in patients presenting with a fracture and in older patients not having chemotherapy. With a mean follow up of 8 years the overall survival was 64% at 5 yrs and 58% at 10 yrs. Adverse prognostic factors included the need for amputation, older age and poor response to chemotherapy as well as a pathological fracture at presentation. The few patients with angiosarcoma fared badly but there was no difference in outcomes between patients with other diagnoses. We conclude that patients with spindle cell sarcomas should be treated similarly to patients with osteosarcoma and can expect comparable outcomes. The histological diagnosis does not appear to predict behaviour.
Enchondromatosis is a non-hereditary disease, characterised by the presence of multiple enchondromas. While Ollier Disease is typified by multiple enchondromas, in Maffucci Syndrome they are combined with haemangioma. Due to the rarity of these diseases, systematic studies on clinical behaviour providing information how to treat patients are lacking. This study intends to answer the following questions: What are predictive factors for developing chondrosarcoma? When is extensive surgery necessary? How often patients die due to dedifferentiation or metastasis? Twelve institutes in eight countries participated in this descriptive retrospective EMSOS-study. 118 Patients with Ollier Disease and 15 patients with Maffucci Syndrome were included. Unilateral localization of disease was found in 60% of Ollier patients and 40% of patients with Maffucci Syndrome. One of the predictive factors for developing chondrosarcoma is the location of the enchondromas; the risk increases especially when enchondromas are located in the scapula (33%), humerus (18%), pelvis (26%) or femur (15%). For the phalanges, this risk is 14% in the hand and 16% in the feet. The decision whether or not to perform extensive surgery is difficult, especially in patients who suffer multiple chondrosarcomas. Malignant transformation was found in fourty-four patients with Ollier Disease (37%) and eight patients with Maffucci Syndrome (53%). Multiple synchronous or metachronous chondrosarcomas were found in 15 patients. Nine patients died (range 21–54 yrs). Seven of them died disease related due to pulmonary metastasis (2 humerus, 2 pelvis, 3 femur). Two patients died from glioma of the brain. In conclusion, one important predictive factor for developing chondrosarcoma is the location of the enchondromas; interestingly, only patients with chondrosarcoma outside the small bones died of their disease. In this series, no dedifferentiation of chondrosarcoma was seen. A first design flow-chart how to approach chondrosarcoma in patients with Ollier Disease and Maffucci Syndrome is in preparation.
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.
Synovial Sarcoma of the foot is the most common soft tissue sarcoma to present in the foot. Despite this, diagnosis is often delayed and treatment may be difficult. The aim of this paper is to review the presenting features, management and outcome of synovial sarcoma of the foot and to try and identify areas for improvement. 33 patients with synovial sarcoma were treated at out unit over a 25 year period. The average duration of symptoms was 125 weeks. The age range at presentation was 11 to 80 years (mean 44). The mean size of the tumour at diagnosis was 5cm in diameter (range 1–10cm). 75% of the tumours were deep at the time of diagnosis. Treatment was by amputation in 21 patients and limb salvage in 11 with 1 patient receiving palliative chemotherapy. Only one patient had local recurrence and presented with lung metastases. Four patients had metastases at diagnosis and nine developed them subsequently. Overall survival at 10 years was 53%. These results have shown that late diagnosis is common for synovial sarcoma of the foot and that by the time of diagnosis 75 % have invaded extra-compartmentally leading to a high risk for amputation. 20 patients had an inadvertent excision of the tumour before referral to our unit. Local control was best achieved with amputation. Overall survival was surprisingly poor despite successful local control. Earlier aggressive investigation of patients with foot pain or swelling but no other features may change their long term prognosis.
Disappearing bone disease is also known as vanishing bone disease, phantom bone disease, massive osteolysis, Gorham’s disease or Gorham-Stout disease. Basically, it is characterised by osteolysis in (contiguous) bone segments, due to localised proliferation of thin-walled vascular channels in the bone and surrounding soft tissues. The etiology and pathophysiology of this condition remain poorly understood and largely unclear, but there is increasing evidence that disordered lymphangiogenesis plays a role. It is an extremely rare cause of osteolysis, so all other differential diagnoses should be considered and ruled out before retaining the diagnosis of disappearing bone disease. Treatment is fairly disappointing and no single treatment modality has proven effective in actually arresting the disease. Conservative treatment includes ant-resorptive agents (bisphosphonates), immunomodulating substances and radiation therapy, whereas surgical treatment options include resection and reconstruction with bone grafts and/or prostheses versus amputation. We report on the only two cases that were identified in our database between 1984 and 2008, both affecting the lower limb (one tibia, one femur). In an attempt to limb salvage, these patients initially underwent endoprosthetic replacement of the affected bone segment, but due to disease progression both eventually ended up with a hip disarticulation.
Given that the incidence of bone sarcomas is 9/million population per year, our 3000 patients represent 333 million population years. When the incidence of a condition is known in the population this allows an estimation of the risk of malignancy compared with the normal population. Retinoblastoma for instance is known to arise in 1 in 16000 births. The 7 malignancies we saw thus represents a risk to individuals with retinoblastoma of 336/million/yr – a figure 37 times the risk of the normal population. Approximate figures of risk have been calculated for other entities.
Malignant tumours of the radius compose only 3% of all upper limb tumours. Owing to their rarity they are often difficult to manage satisfactorily. Of the options for fixation available, endoprosthetic replacements have been scarcely utilized despite their success in limb preservation with malignant tumours in other parts of the body. At our centre we have used these when biological solutions (eg fibula graft) were not indicated due to extensive disease or the need for radiotherapy. We performed four endoprosthetic replacements of the distal radius in three males and one female with ages ranging from 19–66 years (average= 42.25 years of age). Two were performed for varieties of osteosarcoma (parosteal and osteoblastic osteosarcomas), one for a large destructive giant cell tumour (GCT) and one for destructive renal metastases. Three were right sided (75%) and one left sided (25%). Medical records were evaluated for information on local recurrence, metastases, complications and functional outcome using the Toronto Extremity Salvage Score (TESS). Follow up ranged from 22 to 205 months (average= 116.5 months). The average TESS score was 58.1% (range= 44.6–74.5%). Neither case of osteosarcoma recurred. The GCT recurred twice and the patient with renal metastases had nodules removed from his affected wrist on two further occasions. There were no cases of infection, but the two earlier cases had problems with metacarpal stems cutting out and jointsubluxatinos. The two earlier cases have since died at 205 (parosteal osteosarcoma) and 189 months (GCT) respectively of other disease. We conclude that although this is a very small series of endoprosthetic replacement of the distal radius, the technique is a useful addition to the surgical options, with acceptable postoperative functional results and complication rates when a biological solution or preservation of the wrist joint is not indicated.
The 38 procedures were identified from September 1995 to June 2007 and included 17 distal femoral replacements, 12 proximal femoral replacements, 4 proximal humeral replacements, 2 distal humeral replacements, 2 hemi-pelvic replacements and 1 total femoral replacement. EPR survivorship was calculated using a Kaplan-Meier survival curve. The quality of patients’ mobility and performance of activities of daily living was used to assess functional outcome.
87.4% of patients who underwent a lower limb EPR achieved a satisfactory or very satisfactory functional outcome. 100% of patients achieved a satisfactory or very satisfactory functional outcome in the upper limb EPR group. 3 implants failed, 2 as a result of infection and required staged revisions, 1 eventually requiring amputation, and 1 failed as a result of aseptic loosening. 2 patients dislocated their proximal femoral replacements, both were treated successfully by closed reduction. Despite the salvage surgery subsequent amputation was only required in one patient.
Four operations were secondary procedures following previous non-grower implant failures (1 infection of previous EPR, 1 IM nail non-union, 1 failed allograft and a revision of a proximal femoral EPR to a total femoral prosthesis). Five patients required revision of the primary prosthesis (2 with motor failures, 3 due to prosthesis infections). Mean time to start lengthening from surgery was 12.2 months. The mean number of lengthenings was 4 with an average total length of 30 mm achieved, mean leg length difference was 0.8 cm. All lengthenings were undertaken with the patient fully alert, no adverse incidents occurred at the time or after lengthening.
We could not show that delay in diagnosis led to a worse prognosis.
We conclude that METS modular tumour prosthesis for proximal femur provides versatility; low implant related complications and acceptable function lasting the lifetime of the patients with metastatic tumours of the proximal femur providing a cost effective solution.
Improving outcomes is important for any patient with any disease. Defining the outcome measure will effect what steps need to be taken to improve it. In 2006 the National Institute of Clinical Excellence (NICE) published evidence based Guidance on measures to be taken in the UK to improve outcomes for patients with sarcomas. The Guidance established the following principles:
That all patients with sarcomas must be treated by a recognised and properly constituted multi disciplinary team Early diagnosis is a key to better outcomes and clear referral pathways to diagnostic centres should be established for any patient with a suspected possible sarcoma All patients with a suspected diagnosis of a sarcoma must have the diagnosis confirmed by a recognised sarcoma specialist pathologist who participates in quality assurance. All centres treating sarcomas should collaborate in establishing treatment protocols and developing appropriate clinical trials for patients with sarcomas All definitive surgery for patients with sarcomas should be carried out at by appropriately trained sarcoma surgeons who regularly audit their results There should be a national registry of sarcoma cases with treatment and outcome data collected Complex cases such as pelvic, retroperitoneal and head and neck cases should be even more centralised to one or two centres All patients should have a keyworker assigned to them who would usually be a specialist nurse and who can act as a point of contact Effective follow up regimes need to be investigated Patients need access to reliable and relevant information at every step of their pathway How this Guidance has been implemented and what implications this may have for other countries will be discussed.
The definition of a ‘safe’ tumour margin remains controversial. Enneking’s original definition of intralesional, marginal, wide and radical margins was based on a study of just 40 patients of whom only 12 had limb salvage surgery. Since that time thre have been numerous attempts to try and define tumour margins more clearly based on anatomical structures and distances of the tumour from the cut edge. Whilst all can agree on what is a radical margin (usually an amputation) and what is an intralesional margin (tumour exposed) there remains a lack of clear definition of what comprises a wide margin and what is a marginal margin. As an example three large tumour units were asked to assess their margins for adequacy. While the rated of adequate excision varied from 49% to 70%, all 3 units had near identical local recurrence rates of 17–20%. The rate of local recurrence is also modified by adjuvant therapy. A suggestion for an International study assessing tumour margins is made, investigating both anatomical and biological factors.
Overall cumulative patient survival was 58% at 5 years and 44% at 10 years. Locally recurrent disease occurred in 350 patients (14%), 204 patients (8%) presented with and 720 patients (30%) subsequently developed metastatic disease. Prognostic factors for locally recurrent disease were arm tumours (p=0.003, HR=0.3), hip tumours (p=0.01, HR=0.31), thigh tumours (p=0.002, HR=0.52), intralesional margins (p<
0.0001, HR=3.7), high grade tumours (p=0.03, HR=1.8), tumour size 3–6cm (p=0.04, HR=0.54) and tumour size 6–10cm (p=0.03, HR=0.63). Prognostic factors for patient survival were deep location (p=0.02, HR=1.6), high grade tumours (p<
0.0001, HR=4.7), intermediate grade tumours (p<
0.0001, HR=3.4), surgical margins (p=0.04), age at diagnosis (p<
0.0001, HR=1.02), size of tumour <
3cms (p=0.04, HR=0.29), 3–6cms (p<
0.0001, HR=0.41), 6–10cms (p=0.007, HR=0.63), no locally recurrent disease (p=0.0001, HR=0.59).
those 7 whose LR was inoperable due to size, those 7 patients with LR_5cm who had concomitant metastases and in the 5 patients who already had maximum doses of radiotherapy.
We reviewed the treatment and clinical outcome of 32 consecutive patients with Ewing’s sarcoma who presented with or developed pathological fracture after biopsy between 1984 and 2004. The minimum follow-up was 18 months. The mean age at diagnosis was 20 years (5 – 51). There were 18 males and 14 females. All patients were newly diagnosed and had localized disease at the time of diagnosis. 21 patients presented with pathological fracture while 11 patients developed fracture during the course of chemotherapy. The femur was the most common location in 15 patients. All the patients had chemotherapy according to the protocol current at the time of treatment. 6 patients had radiotherapy alone while 26 patients underwent surgical excision and reconstruction. Of the patients who had surgery, 7 patients had adjuvant radiotherapy. Fracture healing was the norm after pre-operative chemotherapy. Surgical margins were wide in 17 patients, marginal in 4 and intralesional in 3 patients. Local recurrence developed in one patient (3%). Metastases occurred in 12 patients (37%). At the time of review 16 patients were free of disease, 3 were alive with disease and 13 patients had died of disease. The cumulative 5 year metastases free and overall survival in all the patients was 58% and 61 % respectively and similar to patients with Ewing’s sarcoma without fracture treated at our centre. The prognosis of patients who presented with fracture was exactly similar to those who developed fracture in the course of treatment. We conclude that limb preserving surgery is perfectly safe in patients with Ewing’s sarcoma who have associated pathological fracture and survival is not in any way compromised. Survival of patients who present with fracture is similar to those who develop fracture in the course of treatment. The exact role of adjuvant radiotherapy in these patients needs to be clarified.
We have investigated whether improvements in design have altered outcome for patients undergoing endoprosthetic replacement of the distal femur following tumour resection. Survival of the implant and ‘servicing’ procedures has been documented using a prospective database and review of the implant design records and case records. A total of 335 patients underwent a distal femoral replacement with 162 having a fixed hinge design and 173 a rotating hinge. The median age of the patients was 24 years (range 13–82yrs). With a minimum follow up of 5 years and a maximum of 30 years, 192 patients remain alive with a median follow up of 11 years. The risk of revision for any reason was 17% at 5 years, 33% at 10 years and 58% at 20 years. Aseptic loosening was the most common reason for revision in the fixed hinge knees whilst infection and stem fracture were the most common reason in the rotating hinges. The risk of revision for aseptic loosening in the fixed hinges was 35% at ten years but appears to have been abolished by the use of a the rotating hinge knee with a hydroxyapatite collar. The overall risk of revision for any reason was halved by use of the rotating hinge. Improvements in design of distal femoral replacements have significantly decreased the risk of revision surgery but infection remains a serious problem for all patients. We believe that a cemented, rotating hinge prosthesis with a hydroxyapatite collar offers the best chance of long term prosthesis survival.
We reviewed the treatment and clinical outcome of 32 consecutive patients with Ewing’s sarcoma who presented with or developed pathological fracture after biopsy between 1984 and 2004. The minimum follow-up was 18 months. The mean age at diagnosis was 20 years (5 – 51). There were 18 males and 14 females. All patients were newly diagnosed and had localized disease at the time of diagnosis. 21 patients presented with pathological fracture while 11 patients developed fracture during the course of chemotherapy. The femur was the most common location in 15 patients. All the patients had chemotherapy according to the protocol current at the time of treatment. 7 patients had radiotherapy alone while 25 patients underwent surgical excision and reconstruction. Of the patients who had surgery, 7 patients had adjuvant radiotherapy. Fracture healing was the norm after pre-operative chemotherapy. Surgical margins were wide in 17 patients, marginal in 4 and intralesional in 3 patients. Local recurrence developed in one patient (3%). Metastases occurred in 12 patients (37%). At the time of review 16 patients were free of disease, 3 were alive with disease and 13 patients had died of disease. The cumulative 5 year metastases free and overall survival in all the patients was 58% and 61 % respectively and similar to patients with Ewing’s sarcoma without fracture treated at our centre. The prognosis of patients who presented with fracture was exactly similar to those who developed fracture in the course of treatment. We conclude that limb preserving surgery is perfectly safe in patients with Ewing’s sarcoma who have associated pathological fracture and survival is not in any way compromised. Survival of patients who present with fracture is similar to those who develop fracture in the course of treatment. The exact role of adjuvant radiotherapy in these patients needs to be clarified.
Synovial sarcoma is a morphologically well-defined neoplasm that most commonly occurs in soft tissue accounting for 5% to 10 % of all soft tissue sarcomas. We reviewed 156 patients with synovial sarcoma of soft tissues treated at a supra-regional centre to determine survival and prognostic factors. There were 77men and 79 women with mean age at presentation of 38 years (3 to 84). Follow-up periods ranged from 3 to 494 months (median 43 months). Tumor was located in lower extremities in 111patients, upper extremities in 34 patients, and trunk and pelvis in 11 patients. Overall survival was 66% at 5 years and 48% at 10 years. The 5 and 10 year survival for the 23 patients who had metastases at the time of diagnosis was 13% and 0% respectively compared to 75% and 54% for those without metastases at diagnosis. Local recurrence occurred in 18 patients (13%). The significant prognostic factors for survival included presence of metastases at diagnosis and development of local recurrence. Tumour size and depth, age of patients and use of chemotherapy did not significantly influence survival. We conclude that the clinical factors which influence survival of patients with synovial sarcoma are different from those of soft tissue sarcomas in general. Biological factors may better predict prognostic survival than the usual clinical factors.
Four patients had obvious infection confirmed by histology and/or microbiology prior to surgery. Endoprosthetic Reconstruction was performed as a 1 stage procedure in 13 and as a 2 stage in 4. Complications occurred in 5 patients. These included recurrence of infection in 1, persistent pain in 1, aseptic loosening in 1, periprosthetic fracture in 1 and a non ST myocardial infarction in 1. At the last follow-up, (mean 5years, range 1–18years) majority of patients achieved good range of motion and good mobility.