Ewing’s sarcoma principally arises in bone but can also present as a soft tissue tumour. Very few studies have assessed the outcomes of extra-skeletal Ewing’s sarcomas. This study compares the oncological outcomes of the two forms of Ewing’s sarcomas to see if there is any difference in prognostic factors. 198 patients with primary, non metastatic Ewing’s sarcoma diagnosed between 1980 and 2005 were identified from our database. There were 118 males and 80 females with a median age of 15 years. The three most common sites of diagnosis were the femur (24%), pelvis (15%) and tibia (13%). There were 169(85%) bony Ewing’s and 29 (15%) extra-skeletal Ewing’s sarcomas. All patients received chemotherapy. 86% of the patients had surgery for local control but 28(14%) patients had radiotherapy. The overall survival at five years was 89% and was related to the age of patient (92% <
16years p=0.005), size (p=0.03) and site of tumour (p=0.004) as well as the response to chemotherapy. There was no difference in the overall survival of patients with bony Ewing’s (90%) and extra-skeletal Ewing’s (85%) (p=0.85). There was a 10% risk of local recurrence at 5 years with site of tumour (p=0.01) and surgical excision (p=0.05) being significant prognostic factors. The risk of local recurrence was also not related to the type of Ewing’s sarcoma. This large series has shown that the oncological outcomes of Ewing’s sarcoma is related to tumour characteristics, patient age and treatment factors and not determined by the tissue component.
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.
We could not show that delay in diagnosis led to a worse prognosis.
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).
Of the 7242 patients with soft tissue lumps, 476 had a past history of malignancy. Of these patients, only 12% actually had a soft tissue metastasis while 28% had a benign diagnosis, 55% a soft tissue sarcoma and 5% other malignancy.
To identify the incidence of a cortical breech on the initial presentation X-rays of patients with distal femoral GCTs, and whether this lead to a higher rate of local recurrence of tumour, a prospective database is kept of all patients seen in the unit. Initial presentation X-rays on 54 patients with distal femroal GCTs were reviewed. The size of the tumour was estimated by measuring the largest dimensions of the tumour (depth, breadth &
height). The volume of the distal femur was estimated using the same X-ray and computer programme. The X-rays were then carefully studied for evidence of a cortical breach. The records were also checked for evidence of subsequent locally recurrent disease and subsequent surgery. X-rays were reviewed on 54 patients (29 male, 25 female), range of 18–72 years. All patients had a biopsy-proven GCT of the distal femur, X-rays (prior to biopsy) were reviewed. 34 (63%) patients with a cortical breech on X-ray. The mean tumour volume: distal femoral volumes (TV:DFV) was statistically greater between those patients with a cortical breach and those without, using ANOVA (p<
0.0001). There were 13 patients with local recurrent disease but no statistical difference in subsequent local recurrence rates between the two patient groups. There was also no statistical differences between the number of operations for those who presented with a cortical breach or without. There was no evidence that more radical surgery was required if a patient presented with a cortical breach. The risk of cortical breech in patients with GCTs of the distal femur is dependant upon the tumour volume to distal femur volume ratio. If the ratio is above 54% then present with a cortical breech on X-ray is likely (95% confidence interval).There is no evidence those patients with a cortical breach have a higher rate of local recurrence, an increased number of operations or more radical surgery.
The use of distal femoral centralising devices has been advocated in order to achieve an even cement mantle. This has been shown to improve femoral component survival but it is recognised that the presence of voids in the mantle has a deleterious effect on the mechanical strength of cement at laboratory testing and in terms of implant survival. The effect of centralising devices on the mantle in relation to the timing of stem insertion has not previously been investigated. The purpose of this study is to assess the quality of the cement mantle in artificial bone using a polished taper stem with centralisation inserted at different stages of cement cure time and using different cements. Three cement types were studied, 45‘saw bone’ models were used. The cementation was carried out in an operating theatre at constant temperature of 23.2Ê°C. The cement was mixed according to the manufacturers instructions and pressurised. Early, intermediate and late stem insertion times were determined for each cement type. The late group included stems with and without centralisers. Video recordings of the stem cement interface were made with a 4 mm endoscope after stem removal. Large cement mantle defects were noted in the ‘with centraliser’ group in 7 out of 15 late insertion times and all had small defects in the mantle. None of the ‘without centraliser’ group had cement mantle defects. Based on our results we advise surgeons to be very aware of the timing of stem insertion when using centralisers.
Endoprosthetic Replacements are one of the most commonly used types of limb salvage following surgical excision of bone tumours. The advantages of Endoprosthetic Replacements are their initial reliability and the rapid restoration of function along with their ready availability. The problems with Endoprosthetic Replacements are the long term problems of wear, loosening, infection and mechanical failure. Increasing and insolvable problems may lead to the necessity for amputation. This paper assesses the risk of amputation following Endoprosthetic Replacement. A total of 1262 patients have undergone Endoprosthetic Replacement surgery at our centre in the past 34 years. They have a total of 6507 patient years of follow up. A total of 112 patients have had subsequent amputation (8. 9%). The reasons for amputation were local recurrence in 71(64. 4%), infection in 38(33. 9%), mechanical failure in 2(1. 8%) and continued pain in 1 case (0. 8%). The risk of amputation was greatest in the proximal tibia 15. 5% (n=38/246), followed by pelvis 10. 2%(5/49), and femur 7. 4% (n=58/784), whilst the risk of amputation was least in the humerus at 6. 4% (n= 1l/182). The time to amputation varied from 2 days to 16. 3 7 years, with a mean of 31 months. The risk of amputation decreased with time although 10% of the amputations took place more than 5 years after implantation. The greatest risk of amputation is in the first 5 years and is due to local recurrence, whilst infection poses the next greatest threat. The risk decreases with time. Attempts to control both local recurrence and infection will decrease the need for amputation. Late failure of the endoprosthetic replacements, even in young patients does not seen to be a major cause of amputation thus far.
To investigate the fears of female patients of child bearing age, who required surgical stabilisation for pelvic fractures, and to assess the outcomes of subsequent pregnancy. Between 1990 &
2002 from a prospectively kept database in our institution, patients sustaining pelvic fractures requiring surgery who were women under 35 years of age at the time of injury were identified and included in this study. Patient’s medical records and radiographs (birth canal status) were reviewed and data collected. All the patients were attempted to be contacted by telephone and a questionnaire completed recording the type of pelvic injury, previous obstetric history, fears regarding future pregnancy, pregnancy outcomes, Euroqol pain scores pre &
post fracture and painkiller usage. Those who were unable to be contacted by telephone, were followed up by a postal questionnaire. The mean time of follow up from injury was 4.2 years (range 1 to 12 years). Out of 554 patients, 197 (36%) were women and of these, 54 (27%) patients were less than 35 years old at the time of injury. A telephone questionnaire was completed on 31 patients [57 % (study group)], results from the postal questionnaire are being collated. The mean age of the study group at injury was 23.3 years (range 6 to 34 years). There were 14 (45 %) isolated ace-tabular fractures and 17 (55 %) pelvic ring fractures. 11 (36 %) had previously had children prior to the injury, and 22 (71 %) had planned to have children in the future, prior to fracture. 23 (74 %) had expressed fears related to their future ability to have children. Out of 8 (26 %) patients who had subsequent pregnancies, only 1 (12.5%) had a normal vaginal delivery. Out of the rest, 3 (37.5%) patients had investigations for pelvic disproportion; 2 (20%) went on to elective caesarean section for disproportion; 1 patient requested an elective section after concerns regarding delivery; 1 patient had a ventouse assisted delivery for delayed second stage; 1 patient had an ectopic pregnancy; 1 patient had a miscarriage at 18 weeks gestation and 1 patient had infertility problems. 4 out of 31 (13%) patients were advised against future pregnancy and one patient underwent a tubal ligation following this advice. Pelvic fractures represent a serious group of injuries; after initial recovery, many female patients have serious concerns regarding future pregnancy. A number will go on to have further pregnancies, and many will suffer the risk of complications following their pelvic injury.
A postal questionnaire was sent to 225 GPs and 225 Orthopaedic Surgeons (Consultant and Specialist Registrars) in 20 hospitals in North West England. They were asked to give their routine clinical practice with regard to investigation of underlying osteoporosis in 3 clinical scenarios :
55 year old lady with a low trauma Colles fracture 60 year old lady with a vertebral wedge fracture 70 year old lady with a low trauma femoral neck fracture. The participants were asked whether patients over 50 years old with low trauma fractures required investigation for osteoporosis, and whether an osteoporosis Nurse Specialist would provide a beneficial service. The response rate was 52% (n=l17) from Orthopaedic Surgeons and 49% (n=l11) from GPs. Both groups agreed that patients over 50 years old with low trauma fractures required investigation for osteoporosis (81 % surgeons and 96% GPs), and that Osteoporosis Nurse Specialists may provide a beneficial Service (81% Surgeons and 94% of GPs). A majority of surgeons (56%) replied that they would routinely discharge the Colles fracture patient without requesting or initiating investigation for underlying osteoporosis. However, a majority of GPs (67%) would not investigate a similar patient for osteoporosis, unless prompted by the surgeon or patient. A greater proportion of both surgeons (71%) and GPs (64%) would routinely initiate investigations or treatment for osteoporosis in the Vertebral Wedge fracture patient. 65% of surgeons would simply discharge a patient with a femoral neck fracture after orthopaedic treatment and 40% of GPs will simply file the hospital discharge letter. Most Orthopaedic Surgeons and GPs are aware that low trauma fractures in patients over 50 years old require investigation for Osteoporosis, however, a large population of patients with Colles and Femoral Neck fractures are not being given the advantages of secondary prevention of Osteoporosis. This may lead to greater workload for Orthopaedic Surgeons in the future.
To assess the efficacy of the current surveillance programme for patients with sarcoma we undertook a prospective analysis of all patients with sarcoma treated between 1990 and 1995. The patients routinely enter a surveillance programme which consists of regular clinical evaluation, CXR and radiological imaging. We reviewed 643 cases of sarcoma with mean follow up 8. 4 years (range 6. 2–11. 3). Local recurrence occurred in 14% of cases and 34% developed metastases. The cumulative survival at 10 years was 59%. 46% of the deaths were directly attributable to metastases. For the soft tissue sarcomas 15% of the local recurrences were picked up at surveillance appointment and 70% were picked up early by the patient. For the sarcomas of bony origin 36% were picked up at surveillance and 57% were picked up early by the patient. Pulmonary metastasis was by far the common metastasis with 82% developing these. 78% were identified by surveillance CXR of which 83% were asymptomatic and 34% went on to thoracotomy and metastectomy. Of the other metastases a third were picked up during surveillance and all were symptomatic. Surveillance programmes have a role in the management of patients with sarcoma, allowing the earlier identification of local recurrence and metastasis. Clinical evaluation and CXR were found to be, in particular, valuable tools, but patient education and open access to clinics is also important.
Endoprosthetic replacement (EPR) following Bone Tumour excision is common. A major complication of EPRs is infection, which can have disastrous consequences. This paper investigates the cause of infection, management and sequelae. Over 10, 000 patients have been treated in our unit over 34 years. Information collected prospectively on a database includes demographic data, diagnosis, treatment (including adjuvant), complications, and outcomes. Data was analysed to identify any infection in EPRs, its management and outcome. Factors such as operating time, blood loss, adjuvant therapy, type of prosthesis (extendable or standard) were investigated. Outcomes of treatment options were evaluated. Data was analysed on 1265 patients undergoing EPR over 34 years, giving a total follow up time of over 6500 patient years. 137 (10.8%) patients have been diagnosed with deep infection (defined by a positive culture [n=128] or a clinically infected prosthesis with pus in the EPR cavity [n=9]). Of these 49 (34%) required amputations for uncontrollable infection. The commonest organisms were Coagulase Negative Staphylococcus, Staphylococcus aureus and Group D Streptococci. The only satisfactory limb salvaging operation was 2 stage revision, which had 71% success in curing infection. Systemic antibiotics, antibiotic cement or beads and surgical debridement had little chance of curing infection. Infection rates were highest in the Tibial (23.1%) &
Pelvic (22.9%) EPRs (p<
0.0001). Patients who had pre or post-operative radiotherapy had significantly higher rates of infection (p<
0.0001), as did patients with extendable EPRs (p=0.007). Patients who had subsequently undergone patella resurfacing and rebushing also had a higher rate of infection (p=0.019 and p=0.052). Infection is a serious complication of EPRs. Treatment is difficult and prolonged. 2 stage revision is the only reliable method for limb salvage following deep infection. Prevention must be the key to reducing the incidence of this serious complication.