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, 34% 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 32% at ten years compared with 4% for rotating hinge knees with a hydroxyapatite collar. The overall risk of revision for any reason was halved by use of the rotating hinge.
99 patients with new diagnosed soft tissue sarcomas involving the pelvic region were studied to determine the outcome and prognostic factors for survival and local recurrence. The mean age at diagnosis was 57 years. There were 55 males and 44 females. The mean tumor size was 12cm. The tumor was deep in 79 patients and superficial in 20. Surgical treatment was excision in 93 patients and hindquarter amputation in 6 patients. Histological grade was low grade in 23 and high grade in 75 patients. 7% of the patients had metastases at presentation. The 5 year overall survival was 57% and local recurrence occurred in 22% of the patients. The risk of inadequate surgical margins in patients with tumors within the pelvic brim was 50% compared to 18% for those with tumors located outside the pelvic brim. The significant predictors of local recurrence were inadequate margins and location of the tumor within the pelvic brim. Tumor size, grade and depth did not influence development of local recurrence. Significant predictors of survival included metastases at presentation, tumor grade and depth. The cumulative 5 year survival for patients with deep high grade tumors, deep low grade tumors, superficial high grade and superficial low grade tumors were 45%, 74%, 63% and 100% respectively (p=0.01). The 5-year overall survival was 66% in those patients without local recurrence compared to 37% in those who develop local recurrence (p=0.005). Multivariate analysis revealed that development of local recurrence was the most important determinant of overall and metastases free survival. We conclude that patients with pelvic soft tissue sarcoma who develop local recurrence have an extremely poor prognosis. Patients with high grade and inadequate surgical margins represent a particular group with very high risk of metastases and death even with radiotherapy and perhaps should be considered for other adjuvant treatment.
130 consecutive patients with metastastic tumours of the extremity bones treated with resection with or without major endoprosthetic reconstruction were studied retrospectively to determine the indication for surgery, complications, clinical outcome and oncological results of treatment. The mean age at diagnosis was 61 (22 – 84). The tumours originated from a variety of organs. Lower extremity was involved in 104 and upper extremity in 26. Metastatic disease was solitary in 55 patients and multiple in 75 at the time of surgery. The median follow-up possible from the time of operation to review was 48 months (0-103). The indication for surgery was radical treatment of solitary metastases with curative intent in 33, pathological fracture in 46, impending fracture in 27, failure of prior fixation devices in 17, painful swelling or extremity in 37. Surgical treatment included excision of expendable bones without reconstruction in 20 patients and resection with endoprosthetic reconstruction in 110 patients. 7 patients received adjuvant chemotherapy and the majority received adjuvant radiotherapy. At the time of review, 58 patients had died at a mean time of 23 months (0–90) from surgery (53 from progressive metastatic disease and 5 from other causes). 72 were alive at mean follow-up of 22 months (1–103) from surgery. 36 patients (28%) were alive at 2 years post-surgery and 8 (6%) at 5 years. One patient died intra-operatively. Post-operative complications occurred in 32 patients (25%). 18 patients required further surgical procedures for dislocation, infection haematoma, stiff joint, plastic surgical procedures. All the patients had control of pain and 90% achieved desired mobility. There was no difference in the survival of patients who presented with solitary and multiple metastases, renal and non-renal metastases, and upper or lower limb metastases.
Only 1% of all primary bone tumours are situated in the distal humerus. Destruction of the distal humerus by tumour is rare and reconstruction of the distal humerus is challenging. Because of the amount of bone loss following tumour excision, excision arthroplasty or arthrodesis is impossible and hence some form of reconstruction is usually required. Allograft reconstruction and hemiarthroplasty are uncommon and lead to an unpredictable outcome. Ten patients underwent endoprosthetic replacement of the distal humerus for bone tumours over a thirty one-year period. There were 8 primary and 2 secondary tumours and male to female ratio was 2:3. Average age of the patients was 47.5 years (15–76 years). Mean follow up was 8 years (9 months - 31 years). Four patients required further surgery, three having revision for asceptic loosening and two of these and one other later needing a rebushing. There were no permanent nerve palsies, infections, local recurrences or mechanical failures of the implant. Four patients died of their disease between 12 and 71 months after operation, all with their prosthesis working normally. Average flexion deformity was 15 degrees (0–35) and average flexion of these patients was 115 degrees (110–135). The average TES Score for these patients was 73% (29% to 93%). The activities which the patients found to be no problem (TES score more than 4.5 out of 5) were: brushing hair, drinking from a glass, putting on make up or shaving, picking up small items, turning a key in a lock, doing light household chores and socialising with friends, whilst activities that proved difficult (TES score less than 3 out of 5) were: gardening and lifting a box to an overhead shelf. Pain was not a problem and only 1 of the surviving patients reported ever having to use regular analgesics.
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 unusual phenomenon of histological grade change in locally recurrent soft tissue sarcomas is examined by retrospective review of a large sarcoma database. Increased histological grade was found to occur in 20% of recurrent tumours. Several possible factors predisposing to grade change were examined, and only the histologic diagnosis of myxoid malignant fibrous histiocytoma was found to be significant. Despite increased histologic grade, these tumours do not appear to have a worse prognosis in terms of developing systemic disease. Soft tissue sarcomas (STS) have a reported local recurrence rate of between five and thirty percent. Recurrent tumours are often similar histologically to the initial tumour, however they are occasionally of higher histological grade than the original lesion. Factors that predispose to this change in grade are not known. We sought to identify the frequency at which locally recurrent STS demonstrate a change in histological grade, and to investigate the possible factors leading to this change. We also investigate whether a change in grade is associated with a poorer prognosis. We identified one hundred and seventy-three patients who developed locally recurrent STS, one hundred and twenty-four of which met inclusion criteria and who will form the basis of this study. Ninety-two patients (74%) had no change in histological grade, twenty-four (19%) demonstrated an increase in histological grade and eight (7%) a decreased histological grade. Univariate analysis of time to local recurrence, histological diagnosis and use of radiotherapy and chemotherapy did not reveal significant differences between the groups who did and did not undergo change in grade. When the diagnosis of myxoid MFH was looked at separately, there was a higher proportion in the group that developed increased histological grade. Development of a change in grade was not associated with a poorer survival rate. Increase in histological grade occurs in approximately 20% of locally recurrent STS, but this phenomenon is not associated with a poorer prognosis than if the grade remains the same. A histological diagnosis of myxoid MFH predicts for an increase in histological grade.
Non-union of long bone fractures can be a challenging problem. There are several methods of treatment and they depend upon various patient factors, biology of non-union, and presence of infection. When faced with failure of treatment with biological reconstructive procedures patients have little choice. At our institute we have treated 10 such patients with radical excision and reconstruction using tumour endoprostheses as a last attempt to save the limb. Median age of the patients was 71 years (25–85). 2 patients were male and 8 were female. Median follow-up was 49 months (8–229). 5 had infected non-union. Resection and massive endoprosthetic reconstruction involved the distal femur in 4 patients, proximal femur 3, distal humerus 2 and total Humerus in 1 patient. Time from diagnosis of non-union to treatment was 0 to 96 months (median 11 months) and patients had had 0 to 6 (median 3) previous operations 5 infected non-unions were operated as 2 stage procedures and received long term antibiotics. 4 out of 5 infected non-unions were salvaged. There were 5 complications, namely periprosthetic fracture, infection, a dislocated shoulder, radial nerve palsy, suture of bosing. All the patients achieved immediate mobility and stability. Extendible prosthesis allowed partial correction of limb shortening.
Pathological fracture occurs in 5–10% of all primary malignant bone tumours. It is thought that they unfavourably influence survival, because the fracture haema-toma may contaminate adjacent tissues. Management is often more aggressive and one is less inclined to consider limb saving surgery. Aim of this study was to determine whether the presence of pathological fracture had an effect on rate of limb salvage surgery, role of adjuvant treatment and survival. A retrospective study was done on all patients with a pathological fracture through localised Ewing’s sarcoma, treated between 1979 and 2001. Of 289 patients with localised Ewing’s sarcoma, 27 had a pathological fracture. Eighteen presented with fracture, in 9 fracture occurred after biopsy. All were treated with chemotherapy according to protocol. Two fractures were already treated by osteosynthesis elsewhere, the rest healed with conservative treatment. After chemotherapy, 20 patients were treated surgically: 19 with limb saving surgery, 1 with amputation. Apart from chemotherapy, treatment was surgery alone in 15, surgery and radiotherapy in 5, and radiotherapy alone in 7 patients. Indications for radiotherapy were close margins, poor chemotherapy response, or pelvic tumours. Surgical margins were wide in 16 patients, marginal in 2, and intralesional in 1 patient. Local recurrence occurred in 2 patients, primarily treated with chemotherapy and radiotherapy alone. Five year survival was 60%, metastasis free survival 59%, both comparable with rates reported in literature.
The median survival for patients without metastases at the time of LR was 3 years with a 31% survival at 10 years. The most important prognostic factor in this group was grade with low grade tumours having a much better outlook (70% survival at 10 years) than intermediate or high grade tumours (24% at 10 years). Complete control of the first local recurrence could not be shown to be a prognostic factor.
The accuracy was 96.9%. CT overestimated metastases in 4%.
Limb preserving surgery following segmental resection of the distal end of the radius and its articular surface presents a major challenge. We have studied 11 consecutive patients with aggressive tumours located in the distal radius that required segmental resection of the distal radius and its articular surface to evaluate the clinical and functional outcome of reconstruction of such defects. The mean age at the time of diagnosis was 33 years (7–60). Follow up ranged from 12 to 306 months (median 56). Histological diagnosis was osteosarcoma in 4 patients, chondrosarcoma in 2, giant cell tumour in 5 and meta-static carcinoma in 1 patient. Four patients received chemotherapy. The length of excised bone ranged from 6 to 14cm. Reconstruction was performed with non-vascularised proximal fibula strut graft in 6 patients, ulna transposition in 3 and custom made endoprosthesis in 2 patients. The wrist joint was arthrodesed in 5 patients. At the time of review 2 patients had died of disease, one was alive with disease and 8 were alive and free of disease. Non-union of the graft occurred in one patient, reflex sympathetic dystrophy in 2 and prosthetic dislocation in one. One patient had local recurrence. Four patients required further surgery including one patient who needed an amputation for severe reflex sympathetic dystrophy, one graft revision for non-union, one secondary wrist arthrodesis and one closed reduction of dislocated endoprosthesis. Patients without arthrodesis often had clinical and radiological signs of wrist instability. The majority of the patients achieved satisfactory function with little or no discomfort and ability to perform activities of daily living. We conclude that limb salvage surgery is worthwhile in patients with resectable tumours of the distal radius.
Myxoid liposarcoma (MLS) is an unusual type of soft tissue sarcoma as it tends to metastasize frequently to sites other than the lungs. This study was aimed to investigate the natural history of patients with MLS to try and identify prognostic factors which could help predict outcome and aid earlier detection of metastases. Data was prospectively collected from patient notes and analysed retrospectively. Prognostic factors and metastatic pattern were examined using Kaplan-Meier curves. There were 124 patients with MLS, aged between 28 and 93, the median size of the tumours was 12cm and the most common site was the thigh. Following treatment with excision and radiotherapy the 5yr survival was 65%. Survival was related to younger age (p=0.010) and proximal site (p=0.003) and was also related to the % round cell component of the tumour but was not related to either size or depth of the tumour. Site and margins of excision were significant prognostic factors for local recurrence of disease. 32% of patients developed metastases, of whom 18 cases (46.2%) developed pulmonary metastases and 21 (53.8%) developed extra pulmonary metastases. The sites of these varied hugely and was not significantly related to the site or size of the primary tumour. There was no difference in time to develop metastases or in overall survival between the two groups. Median survival following metastases was 24 months. Although MLS has an unusual pattern of metastases the site of metastases does not predict a better or worse outcome. Intensive follow up for extraskeletal metastases is probably not justified until they become symptomatic.
Large benign lytic lesions of the proximal femur present a significant risk of pathological fractures. We report our experience of treating 9 consecutive patients with such defects treated with curettage and fibula strut grafting without supplementary osteosynthesis to evaluate the outcome of this type of reconstruction.. The mean age at the time of diagnosis was 13 years (8–21). Follow up ranged from 2 to 215 months (median 15). Histological diagnosis was fibrous dysplasia in 10 patients and unicameral cyst in 2. All the patients were at risk of pathological fracture. None of the patients developed pathological fracture after surgery and the lesions consolidated fully within one year. Local recurrence occurred in one patient (8%). Minor donor site complications occurred four patients. All the patients were able to fully weight usually within 3 months of surgery. At the time of review all but one patient were completely asymptomatic and fully weight bearing. The only symptomatic patient was the patient with local recurrence which has recently been treated. We conclude that fibula strut graft is a good method of reconstruction of cystic defects in the proximal femut. It prevents pathological fracture, allows mechanical reinforcement of the lesion and delivers biological tissue allowing early consolidation of the defect.
We reviewed our experience with diaphyseal endoprostheses to determine the survival of this type of reconstruction and factors affecting that survival.
The weights of evidence (WE) are logs of the likelihood ratios and can be added and a probability then calculated. e.g. a 36 yr old with a 10cm, deep, painless lump that is increasing in size scores −0.39 + 0.4 + 0.4 – 0.11 + 0.58 = 0.88. This equates to a risk of the lump being malignant of 70%.
The generally unfavourable prognosis and perceived risks have led surgeons to palliate, stabilise in situ or amputate for distal femoral metastases despite recognised morbidity and life style restrictions. We conclude that DF EPR should be considered as a limb salvage option in patients with distal femoral mets.
The purpose of this paper is to present the rare occurrence of clear cell chondrosarcoma in a patient suffering from Niemann-Pick disease (NPD). NPD is a rare autosomal recessive inborn error of metabolism. It is a lysosomal storage disorder affecting children and adolescents often causing death in early childhood although in milder form patient may survive up to adulthood. Although chondrosarcoma is a relatively common malignant bone lesion, these lesions rarely affect patients suffering from Niemann-Pick disease. This article presents the case of a 50-year-old man with a chondrosarcoma in the olecranon of the left ulna who also suffered from milder form of Niemann-Pick disease. Radiography and MRI showed cortical disruption and an expansile lesion. Histologically, the tumour was classified as grade 2 clear cell chondrosarcoma with classical differentiation according to the American Joint Committee of Cancer pathologic staging system. Amputation of an above elbow type was performed to obtain clear surgical margins. A case report and discussion of this rare association of a patient suffering from milder form of NPD who developed a clear cell chondrosarcoma is presented here. NPD and clear cell chondrosarcoma are rare diseases and together their occurrence was not found reported in the literature. NPD patients rarely live to the age of 50 years. This raises a possibility that if Niemann-Pick patients survived long enough they might develop chondrosarcoma. Alternatively this could just be coincidental. More research is needed to clarify this possibility.
130 consecutive patients with metastatic tumours of the extremity bones treated with resection with or without major endoprosthetic reconstruction were studied retrospectively to determine the indication for surgery, complications, clinical outcome and oncological results of treatment. The mean age at diagnosis was 61 (22 – 84). The tumours originated from a variety of organs. Lower extremity was involved in 104 and upper extremity in 26. Metastatic disease was solitary in 55 patients and multiple in 75 at the time of surgery. The median follow-up possible from the time of operation to review was 18 months (0–103) The indication for surgery was radical treatment of solitary metastases with curative intent in 33, pathological fracture in 46, impending fracture in 27, failure of prior fixation devices in 17, painful swelling or extremity in 37. Surgical treatment included excision of expendable bones without reconstruction in 20 patients and resection with endoprosthetic reconstruction in 110 patients. 7 patients received adjuvant chemotherapy and the majority received adjuvant radiotherapy. At the time of review, 58 patients had died at a mean time of 23 months (0–90) from surgery (53 from progressive metastatic disease and 5 from other causes). 72 were alive at mean follow-up of 22 months (1–103) from surgery. 36 patients (28%) were alive at 2 years post-surgery and 8 (6%) at 5 years. One patient died intra-operatively. Post-operative complications occurred in 32 patients (25%). 18 patients required further surgical procedures for dislocation, infection haematoma, stiff joint, plastic surgical procedures. All the patients had control of pain and 90% achieved desired mobility. There was no difference in the survival of patients who presented with solitary and multiple metastases, renal and non-renal metastases, and upper or lower limb metastases. We conclude that selected patients with bone metastases can benefit from resection and major bone reconstruction with acceptable morbidity. We have not identified predictable prognostic factors in these selected patients.
Aim: The purpose of this retrospective study was to analyse the risk factors, causes, bacteriology of deep infection following extensible endoprosthetic replacement for bone tumours in children and to review our experience in the treatment of 20 patients with infected prostheses. Materials and methods: 123 patients with extensible endoprostheses were treated between 1983 and 1998. Three types of prostheses, which differed in the lengthening mechanism used, were implanted. 20 of these were diagnosed to have deep infection. Patients were divided into 3 groups: group I- 5 patients were treated with a single stage revision, group II- 13 patients were treated with a two stage revision procedure, group III- 2 patients had a primary amputation. Control of infection was assessed clinically and with inflammatory markers. Function was assessed using the MSTS score. Results: The overall incidence of infection was 16%. The incidence of infection at the proximal tibia and distal femur was 27% and 14% respectively. Staphylococcus epidermi-dis was the most common organism. The most common clinical features were pain and swelling around the pros-theses. Infection in most cases was immediately preceded by an operative procedure or by distant a focus of infection. The number of operative procedures and the site of the prosthesis were significant risk factors. The success rate was 20% in Group I and 84.6% Group II. Amputation was the salvage procedure of choice for failed revision procedures. The mean MSTS functional score was 83% in patients in whom the infection was controlled. Conclusion: The incidence of deep infection is high following extensible endoprostheses. The site of the pros-thesis and the number of operative procedures are significant risk factors.
About one third of patients who require one knee replacement have significant bilateral symptoms and will require surgery on both knees before achieving their full functional potential. The options for these patients are either to have one-stage bilateral knee replacements or two-stage knee replacements. Our aim was to compare the relative local and systematic morbidity of patients who had one-stage bilateral knee arthroplasty with those of patients who had unilateral total knee arthroplasty in a retrospective, consecutive cohort of patients to evaluate the safety of one-stage bilateral total knee arthroplasty. Seventy-two patients treated with one-stage bilateral knee replacements were matched for age, gender and year of surgery with 144 patients who underwent unilateral knee arthroplasty. We found one-stage bilateral arthroplasty was associated with significantly increased risks of wound infection, deep infection, cardiac complications and respiratory complications compared to unilateral knee arthroplasty. No increased risk of thromboembolic complications or mortality was found. We conclude that one-stage bilateral total knee arthroplasty is associated with increased risk of both systematic and local complications compared with unilateral knee replacement and therefore should be performed on only selective cases.
Our centre has used a specially designed custom-made endoprostheses with curved stems to reconstruct femoral defects in patients with residual short proximal femur after excision of primary bone sarcoma over the last 18 years. Two designs of endoprostheses with curved intramedullary stems were used: the rhinohorn stem type and the bifid stem type. We report the safety, survival and functional outcome of this form of reconstruction. Twenty six patients who had these special endoprosthesis reconstruction were studied. The median age was 16 years (range 7 to 60 years). Prostheses with rhino horn stems were used in 15 patients and bifid-stem in 1 1 patients. Twenty patients had the prostheses inserted as a primary procedure after excision of primary bone sarcoma, and in six patients the prostheses were inserted after revision surgery of failed distal femur endoprostheses. Seventeen patients (65%) were alive and free of disease at a median follow-up of 98 months (12 to 203 months) and nine patients had died of metastatic disease. Local recurrence developed in two patients (1 0%) out of the 20 patients. Surgical complications occurred in five patients (191/o). Deep infections occurred in two patients (8%) requiring revision surgery in one patient. Prosthetic failure, occurred in nine patients (35%). The cumulative survival of prostheses was 69% at five years and 43% at 10 years. Musculoskeletal Tumour Society mean functional score was 83% (53% to 97%). In conclusion, preservation of a short segment of the proximal femur and the use of endoprostheses with curved stems for reconstruction of the femur is technically possible. There is an increased risk of fracture of the prostheses decreasing the survival rate. Functional outcome of patients with this form of reconstruction is not significantly different from the functional outcome of patients who have proximal femur or total femur endoprosthetic reconstruction. This operation is particularly desirable in skeletally immature patients and allows normal development of the acetabulum.
The purpose of this retrospective study was to analyze the risk factors, causes, bacteriology of deep infection following extensible endoprosthetic replacement for bone tumours in children and to review our experience in the treatment of 20 patients with infected prostheses. 123 patients with extensible endoprostheses were treated between 1983 and 1998. Three types of prostheses, which differed in the lengthening mechanism used, were implanted. 20 of these were diagnosed to have deep infection. Patients were divided into 3 groups: group I 5 patients were treated with a single stage revision, group 11– 13 patients were treated with a two stage revision procedure, group Ill- 2 patients had a primary amputation. Control of infection was assessed clinically and with inflammatory markers. Function was assessed using the MSTS score. The overall incidence of infection was 16%. The incidence of infection at the proximal tibia and distal femur was 27% and 14% respectively. Staphylococcus epidermidis was the most common organism. The most common clinical features were pain and swelling around the prostheses. Infection in most cases was immediately preceded by an operative procedure or by distant a focus of infection. The number of operative procedures and the site of the prosthesis were significant risk factors. The success rate was 20% in Group 1 and 84% in Group II. Amputation was the salvage procedure of choice for failed revision procedures. The mean MSTS functional score was 83% in patients in whom the infection was controlled. The incidence of deep infection is high following extensible endoprostheses. The site of the prosthesis and the number of operative procedures are significant risk factors. The type of prosthesis used is not a risk factor. Two-stage revision is successful in controlling infection in a majority of these cases.
This is a retrospective study of 70 patients with chondroblastoma treated between 1973 to 2000. Of these 70 patients, 53 had their primary procedure performed at our unit in the form of an intralesional curettage. The purpose of this study was to determine the rates of recurrence and the functional outcomes following this technique. Factors associated with aggressive tumour behaviour were also analysed. The patients were followed up for at least 22 months, up to a maximum of 27 years. 6 out of these 53 cases (11. 3%) had a histologically proven local recurrence. Three patients underwent a second intralesional curettage procedure and had no further recurrences. Two patients had endoprosthetic replacement of the proximal humerus and one patient underwent a below knee amputation following aggressive local recurrences. One patient had the rare malignant metastatic chondroblastoma and died eventually. The mean MSTS score was 94. 1%. We conclude that meticulous primary intralesional curettage without any additional procedure can achieve low rates of local recurrence and excellent long-term functional results.
Malignant peripheral nerve sheath tumours (MPNSTs) constitute 10% of soft tissue sarcomas. A significant proportion arise in neurofibromatosis type 1 (NF1). Several publications have compared MPNST survival in sporadic and NF1 patients, without consensus on whether NF1 is an independent factor for poor prognosis. Clinical and histological data from 135 proven MPNSTs were analysed from 2 national centres for soft tissue tumour surgery diagnosed from 1979 to 2000. 129 patients had follow-up data from 6 months to 21 years. 35 were from patients with NF1. Local treatment involved surgery in surgery in 95%, radiotherapy in 44% and chemotherapy in 21%. NF1 patients were younger than those with sporadic tumours (median age 26 years vs 53 years, p<
0. 001). Overall MPNST survival was almost identical to that in soft tissue sarcomas as a whole, but was worse in NF1 than in sporadic tumours (33% vs 72% at 30 months [p<
0. 01], 17% vs 39% at 60 months, 6% vs 21% at 120 months). A trend towards shorter time to local recurrence was seen in NF1, but not time to metastasis. Superficial tumours gave improved prognosis. Tumour volume over 100ml was associated with worse survival (46% vs 91% at 30 months, p<
0. 02), as was histological grade (80% high grade vs 25% low grade at 60 months, p<
0. 01). In terms of location, a non-significant over-representation of NF1 MPNSTs in the sciatic and brachial plexii was identified. NF1 and sporadic MPNSTs exhibited no difference in depth or tumour volume profile, although NF1 tended towards higher grade. Analysis of survival in only high grade tumours, however, still resulted in a significant survival disadvantage in NF1 (33% vs 70% at 30 months, p<
0. 01). Removal of brachial and sciatic plexus tumours from analysis did not affect survivorship profiles in NF1 and sporadic MPNSTs. Grade, volume and tumour depth correlate with survival; only 7 of 45 patients with deep high grade tumours over 100ml volume were observed to survive beyond 2 years. MPNST survival is worse in NF1 than sporadic tumours. Grade, depth, site and volume differences could not explain this disadvantage.
Few studies of wound complications following limb salvage surgery for soft tissue sarcomas separate anatomical compartments. Forty-nine patients with adductor compartment sarcomas underwent limb salvage surgery, 43% developing significant wound complications, 25% requiring further surgery and 20% had delays in adjuvant radiotherapy as a result. Prior surgery by non tumour surgeons and previous radiotherapy led to an increased risk of wound healing problems. In this particular group of patients, special attention should be made to prevent wound healing complications, possibly involving plastic surgeons at an earlier stage of management.
Between 1982 and 1997, twenty-six children between the age of 2 and 15 (mean age 10. 6 years) underwent proximal femoral replacement. Twenty have survived and all but three have reached skeletal maturity. Sequential radiographs have been reviewed with particular reference to acetabular development and fixation of the prostheses. Initially a cemented acetabular component was inserted, but recently uncemented implants and unipolar femoral heads that exactly fit the acetabulum have been used. In older children the acetabulum develops normally and the components remain well fixed. One of nine children over thirteen years with a cemented acetabulum needed revision for loosening and one suffered recurrent dislocations. In younger children the acetabulum continues to develop at the triradiate cartilage, so a cemented acetabulum grows away from the ischiopubic bar. As the component is fixed proximally, it becomes increasingly vertical and will almost inevitably loosen. In our study six of eight children under 13 years of age with a cemented acetabulum needed revision for loosening. Unipolar replacements in younger children tend to erode the superior acetabular margin. Femoral head cover is difficult to maintain, and of four unipolar implants in children under thirteen, two required acetabular augmentation. Cemented cups may be unsuitable for children under thirteen years but our results are not statistically significant. In this age group, unipolar implants may be more appropriate but they have serious potential complications. In children over thirteen, cemented implants survive longer. The number of uncemented implants in our study is too small to comment on long-term survival.
Reconstruction of the shoulder joint following resection of the proximal humerus for bone tumours remains controversial. We report the long term functional results of the simplest form of reconstruction – an endoprosthesis. One hundred patients underwent endoprosthetic replacement of the proximal humerus between 1976 and 1998. Thirty eight had osteosarcoma, 17 had chondrosarcoma, 16 had metastases and 9 had Ewing’s sarcoma. Mean age was 36 years (range 10 to 80 yrs). Survivorship of patients and prostheses were calculated. Function was assessed using the Musculoskeletal tumour society (MSTS) and Toronto extremity salvage (TESS) scoring systems. Thirty patients could come to the clinics for MSTS scoring and 38 out of 49 alive patients replied to the TESS questionnaires sent out to them. The overall survival of the patients was 42% at 10 years. Local recurrence (LR) arose in 16 patients, being most common in chondrosarcoma (26%) and osteosarcoma (22%) and arose in 50% of patients with these tumours who had marginal excisions. Of these 16 patients, 8 had forequarter amputations whilst the remainder had excisions and radiotherapy. Mean time to LR was 12 months and all but two of these 16 patients subsequently died within a mean of 18 months. The prostheses proved reliable and dependable. Only 9 required further surgery of any sort, 2 needing minor surgery to correct subluxation and 7 needing revisions, one for infection after radiotherapy and six for loosening – three after trauma. The survivorship of the prosthesis without any further surgery was 86. 5% at 20years. The survivorship of the limb without amputation was 93% at 20 years. The functional outcome was very predictable. Most patients had only 45 degrees of abduction although three patients had normal movements. The mean MSTS functional score was 79% and the mean TESS score was also 79%. There was a high level of patient satisfaction but difficulty was encountered especially in lifting and in all activities above shoulder height. Endoprosthetic replacement of the proximal humerus is a predictable procedure providing reasonable function of the arm below shoulder height. The endoprostheses have proved highly dependable with a low re-operation rate. There is a high risk of local recurrence after inadequate surgery which should be avoided if possible.