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.
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.
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.
The authors are not aware of any research comparing computed tomography (CT) and avascular necrosis (AVN) of the scaphoid bone. The primary aim of our study was to investigate the use of longitudinal CT in predicting AVN of the proximal pole of the scaphoid, and subsequent fracture nonunion following internal fixation. Thirty-two patients operated on by the senior author for scaphoid fracture were included. Preoperative CT scans were independently assessed for deformity, comminution, fracture position, proximal pole sclerosis, and bridging trabeculae. Intra-operative biopsy of the proximal pole was assessed independently by a blinded musculoskeletal histologist. AVN was determined by histology of a proximal pole biopsy, using the criteria described by Ficat. Post-operative CT scan was utilised to determine fracture union. Preoperative CT features which significantly correlated with AVN were, increased radiodensity of the proximal pole, the absence of any bridging trabeculae comminution, dorsal cortical angle, proximal fracture and age less than 20. Features predictive of subsequent nonunion were fractures of the proximal, increased radiodensity of the proximal pole, and AVN. Preoperative CT scan findings are significantly correlated with histologically confirmed AVN and fracture union. Preoperative longitudinal CT scan is of significant prognostic value and should be considered to assist in predicting outcome and assessing treatment options.
The purpose of the study was to describe the normal anatomy of glenoid labrum. 20 dry bone scapulas and 19 cadaveric shoulders were examined. Light microscopy was performed on 12 radial slices through the glenoid. An external capsular circumferential ridge, 7–8mm medial to the glenoid rim marks the attachment of the capsule. A separate internal labral circumferential ridge 4mm central to the glenoid rim marks the interface for the labrum and articular cartilage. A superior-posterior articular facet contains the superior labrum. Two thirds of the long head of biceps arise from the supraglenoid tubercle, the remainder from the labrum. The superior labrum is concave and is loosely attached to the articular cartilage and glenoid rim. In contrast the anterior-inferior labrum is convex, attaches 4mm central to the glenoid rim and has a strong attachment to articular cartilage and bone. The anatomy of the superior and anterior-inferior labrum are fundamentally different.
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.
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.
In our database of 7935 patients referred for investigation of a soft-tissue mass, only 100 were found to have a soft-tissue metastasis (1.3%). Our aim was to define the clinical features of such patients and to identify the site of their primary tumour. The most common presentation was a painful lump, deep to the fascia, ranging between 2 cm and 35 cm (mean 8.3 cm) with 78% of the lumps located deep to the fascia. The mean age of the patients at presentation was 64 years (22 to 84) and there were almost equal numbers of men and women. Of 53 patients with a history of malignancy, 52 had metastases from the same primary (lung in 12, melanoma in ten, kidney in nine, gastrointestinal track in four, breast in five, bladder in four, and others in eight). The other 47 had no history of malignancy and the metastasis was the first presentation. The primary sites in these cases were the lung in 19, gastro-intestinal track in four, kidney in two, melanoma in nine, other in three, and unknown (despite investigations) in ten. There was no correlation between the site of the metastases and the primary tumour. Of the 7935 patients, 516 had a history of malignancy. Of these, only 10% had a soft-tissue metastasis, 29% had a benign diagnosis, 55% a soft-tissue sarcoma and 6% another malignancy. Patients with soft-tissue metastases have similar clinical features to those with soft-tissue sarcomas and should be considered for assessment at appropriate diagnostic centres for patients with suspicious soft-tissue lumps.
The purpose of the study was to describe the normal anatomy of glenoid labrum. Twenty dry bone scapulas and 19 cadaveric shoulders were examined. Light microscopy was performed on 12 radial slices through the glenoid. An external capsular circumferential ridge, 7–8mm medial to the glenoid rim marks the attachment of the capsule. A separate internal labral circumferential ridge 4mm central to the glenoid rim marks the interface for the labrum and articular cartilage. A superior-posterior articular facet contains the superior labrum. Two thirds of the long head of biceps arise from the supraglenoid tubercle, the remainder from the labrum. The superior labrum is concave and is loosely attached to the articular cartilage and glenoid rim. In contrast the anterior-inferior labrum is convex, attaches 4mm central to the glenoid rim and has a strong attachment to articular cartilage and bone. The anatomy of the superior and anteroinferior labrum are fundamentally different. Suture anchor repair of the superior labrum should be 7mm medial to the glenoid rim whereas the anterior-inferior labrum should be repaired to the face of the glenoid. By defining the normal anatomy of the superior labrum, pathological tears can be identified.
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 undertook a cemental unipolar proximal femoral endoprosthetic replacement in 131 patients with a mean age of 50 years (2 to 84). Primary malignant tumours were present in 54 patients and 67 had metastatic disease. In addition, eight patients had either lymphoma or myeloma and two had non-oncological disorders. The mean follow-up was 27 months (0 to 180). An acetabular revision was required later in 14 patients, 12 of whom had been under the age of 21 years at the time of insertion of their original prosthesis. The risk of acetabular revision in patients over 21 years of age was 8% at five years compared with 36% in those aged under 21 years. All the unipolar hips in this younger age group required revision within 11 years of the initial operation. We conclude that unipolar replacement should not be used in younger patients and should be avoided in patients with a life expectancy of more than five years.
Endoprosthetic replacement of the proximal femur may be required to treat primary bone tumours or destructive metastases either with impending or established pathological fracture. Modular prostheses are available off the shelf and can be adapted to most reconstructive situations for this purpose. We have assessed the clinical and functional outcome of using the METS (Stanmore Implants Worldwide) modular tumour prosthesis to reconstruct the proximal femur in 100 consecutive patients between 2001 and 2006. We compared the results with the published series for patients managed with modular and custom-made endoprosthetic replacements for the same conditions. There were 52 males and 48 females with a mean age of 56.3 years (16 to 84) and a mean follow-up of 24.6 months (0 to 60). In 65 patients the procedure was undertaken for metastases, in 25 for a primary bone tumour, and in ten for other malignant conditions. A total of 46 patients presented with a pathological fracture, and 19 presented with failed fixation of a previous pathological fracture. The overall patient survival was 63.6% at one year and 23.1% at five years, and was significantly better for patients with a primary bone tumour than for those with metastatic tumour (82.3% vs 53.3%, respectively at one year (p = 0.003)). There were six early dislocations of which five could be treated by closed reduction. No patient needed revision surgery for dislocation. Revision surgery was required by six (6%) patients, five for pain caused by acetabular wear and one for tumour progression. Amputation was needed in four patients for local recurrence or infection. The estimated five-year implant survival with revision as the endpoint was 90.7%. The mean Toronto Extremity Salvage score was 61% (51% to 95%). The implant survival and complications resulting from the use of the modular system were comparable to the published series of both custom-made and other modular proximal femoral implants. We conclude that at intermediate follow-up the modular tumour prosthesis for proximal femur replacement provides versatility, a low incidence of implant-related complications and acceptable function for patients with metastatic tumours, pathological fractures and failed fixation of the proximal femur. It also functions as well as a custom-made endoprosthetic replacement.
We treated 98 patients with peri-acetabular tumours by resection and reconstruction with a custom-made pelvic endoprosthesis. The overall survival of the patients was 67% at five years, 54% at ten years and 51% at 30 years. One or more complications occurred in 58.1% of patients (54), of which infection was the most common, affecting 30% (28 patients). The rate of local recurrence was 31% (29 patients) after a mean follow-up of 71 months (11 to 147). Dislocation occurred in 20% of patients (19). Before 1996 the rate was 40.5% (17 patients) but this was reduced to 3.9% (two patients) with the introduction of a larger femoral head. There were six cases of palsy of the femoral nerve with recovery in only two. Revision or excision arthroplasty was performed in 23.7% of patients (22), principally for uncontrolled infection or aseptic loosening. Higher rates of death, infection and revision occurred in men. This method of treatment is still associated with high morbidity. Patients should be carefully selected and informed of this pre-operatively.
Analysis of the outcome of neurovascular island flap developed to reconstruct volar-oblique fingertip amputations. A comparison of results with the initial study. Patients were contact telephonically and recalled for review. A subjective questionnaire was filled in and objective clinical measurements taken. The parameters of the original study were reproduced in order to compare results. We have 12 cases since 2004. 5 Cases were lost to follow up with only clinic notes available. 7 Cases were reviewed. Mean age 20y (4–65y). Good subjective results with regard to cold intolerance, hypersensitivity, numbness, pain and stiffness. Good cosmesis and patient satisfaction. Objective measurements of IPJ stiffness were insignificantly different from the contra lateral side and 2 point discrimination <
=5mm. A single flap failure due to sepsis. It was concluded that this was a safe and reliable method of reconstruction, with a number of advantages over previous methods.
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.
Conclusion: We conclude that total femur endoprosthetic replacement offers an excellent method of limb reconstruction following excision of the whole femur either for primary or metastatic tumours. However, patients survival after such operation is poor due to disease related factors.
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.
We report our experience of treating 17 patients with benign lesions of the proximal femur with non-vascularised, autologous fibular strut grafts, without osteosynthesis. The mean age of the patients at presentation was 16.5 years (5 to 33) and they were followed up for a mean of 2.9 years (0.4 to 19.5). Histological diagnoses included simple bone cyst, fibrous dysplasia, aneurysmal bone cysts and giant cell tumour. Local recurrence occurred in two patients (11.7%) and superficial wound infection, chronic hip pain and deep venous thrombosis occurred in three. Pathological fracture did not occur in any patient following the procedure. We conclude that non-vascularised fibular strut grafts are a safe and satisfactory method of treating benign lesions of the proximal femur.
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.
We investigated whether our policy of routine re-excision of the tumour bed after an unplanned excision of a soft-tissue sarcoma was justified. Between April 1982 and December 2005, 2201 patients were referred to our hospital with the diagnosis of soft-tissue sarcoma, of whom 402 (18%) had undergone an unplanned excision elsewhere. A total of 363 (16.5%) were included in this study. Each patient was routinely restaged and the original histology was reviewed. Re-excision was undertaken in 316 (87%). We analysed the patient, tumour and treatment factors in relation to local control, metastasis and overall survival. Residual tumour was found in 188 patients (59%). There was thus no residual disease in 128 patients of whom 10% (13) went on to develop a local recurrence. In 149 patients (47%), the re-excision specimen contained residual tumour, but it had been widely excised. Local recurrence occurred in 30 of these patients (20%). In 39 patients (12%), residual tumour was present in a marginal resection specimen. Of these, 46% (18) developed a local recurrence. A final positive margin in a high-grade tumour had a 60% risk of local recurrence even with post-operative radiotherapy. Metastases developed in 24% (86). The overall survival was 77% at five years. Survival was related to the grade of the tumour and the finding of residual tumour at the time of re-excision. We concluded that our policy of routine re-excision after unplanned excision of soft-tissue sarcoma was justified in view of the high risk of finding residual tumour.
Pathological fractures due to metastasis with destruction of the acetabulum and central dislocation of the hip present a difficult surgical challenge. We describe a series using a single technique in which a stable and long-lasting reconstruction was obtained using standard primary hip replacement implants augmented by strong, fully-threaded steel rods with cement and steel mesh, where required. Between 1997 and 2006, 19 patients with a mean age of 66 years (48 to 83) were treated using a modified Harrington technique. Acetabular destruction was graded as Harrington class II in six cases and class III in 13. Reconstruction was achieved using three 6.5 mm rods inserted through a separate incision in the iliac crest followed by augmentation with cement and a conventional cemented Charnley or Exeter primary hip replacement. There were no peri-operative deaths. At the final follow-up (mean 25 months (5 to 110)) one rod had fractured and one construct required revision. Of the 18 patients who did not require revision, 13 had died. The mean time to death was 16 months (5 to 55). The mean follow-up of the five survivors was 31 months (18 to 47). There were no cases of dislocation, deep infection or injury to a nerve, the blood vessels or the bladder.
We have investigated whether improvements in design have altered the outcome for patients undergoing endoprosthetic replacement of the proximal tibia following resection of a tumour. Survival of the implant and ‘servicing’ procedures have been documented using a prospective database. A total of 194 patients underwent a proximal tibial replacement, with 95 having a fixed-hinge design and 99 a rotating-hinge with a hydroxyapatite collar; their median age was 21.5 years (10 to 74). At a mean follow-up of 14.7 years (5 to 29), 115 patients remain alive. The risk of revision for any reason in the fixed-hinge group was 32% at five years, 61% at ten years and 75% at 15 and 20 years, and in the rotating-hinge group 12% at five years, 25% at ten years and 30% at 15 years. Aseptic loosening was the most common reason for revision in the fixed-hinge knees, fracture of the implant in the early design of rotating hinges and infection in the current version. The risk of revision for aseptic loosening in the fixed-hinge knees was 46% at ten years. This was reduced to 3% in the rotating-hinge knee with a hydroxyapatite collar. The cemented, rotating hinge design currently offers the best chance of long-term survival of the prosthesis.
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 identified 42 patients who presented to our unit over a 27-year period with a secondary radiation-induced sarcoma of bone. We reviewed patient, tumour and treatment factors to identify those that affected outcome. The mean age of the patients at presentation was 45.6 years (10 to 84) and the mean latent interval between radiotherapy and diagnosis of the sarcoma was 17 years (4 to 50). The median dose of radiotherapy given was estimated at 50 Gy (mean 49; 20 to 66). There was no correlation between radiation dose and the time to development of a sarcoma. The pelvis was the most commonly affected site (14 patients (33%)). Breast cancer was the most common primary tumour (eight patients; 19%). Metastases were present at diagnosis of the sarcoma in nine patients (21.4%). Osteosarcoma was the most common diagnosis and occurred in 30 cases (71.4%). Treatment was by surgery and chemotherapy when indicated: 30 patients (71.4%) were treated with the intention to cure. The survival rate was 41% at five years for those treated with the intention to cure but in those treated palliatively the mean survival was only 8.8 months (2 to 22), and all had died by two years. The only factor found to be significant for survival was the ability to completely resect the tumour. Limb sarcomas had a better prognosis (66% survival at five years) than central ones (12% survival at five years) (p = 0.009). Radiation-induced sarcoma is a rare complication of radiotherapy. Both surgical and oncological treatment is likely to be compromised by the treatment received previously by the patient.
We investigated whether improvements in design have altered the outcome for patients undergoing endoprosthetic replacement of the distal femur after resection of a tumour. Survival of the implant and ‘servicing’ procedures have been documented using a prospective database, review of the design of the implant and case records. In total, 335 patients underwent a distal femoral replacement, 162 having a fixed-hinge design and 173 a rotating-hinge. The median age of the patients was 24 years (interquartile range 17 to 48). A total of 192 patients remained alive with a mean follow-up of 12 years (5 to 30). The risk of revision for any reason was 17% at five years, 33% at ten years and 58% at 20 years. Aseptic loosening was the main reason for revision of the fixed-hinge knees while infection and fracture of the stem were the most common for the rotating-hinge implant. The risk of revision for aseptic loosening was 35% at ten years with the fixed-hinge knee, which has, however, been replaced by the rotating-hinge knee with a hydroxyapatite collar. The overall risk of revision for any reason fell by 52% when the rotating-hinge implant was used. Improvements in the design of distal femoral endoprostheses have significantly decreased the need for revision operations, but infection remains a serious problem. We believe that a cemented, rotating-hinge prosthesis with a hydroxyapatite collar offers the best chance of long-term survival of the prosthesis.
Peri- and sub-prosthetic fractures, or pathological fractures below an existing well-fixed femoral component, with or without an ipsilateral knee replacement, present a difficult surgical challenge. We describe a simple solution, in which a custom-made prosthesis with a cylindrical design is cemented proximally to the stem of an existing, well-fixed femoral component. This effectively treats the fracture without sacrificing the good hip. We describe five patients with a mean age of 73 years (60 to 81) and a mean follow-up of 47 months (6 to 108). The mean overlap of the prosthesis over the femoral component was 7.5 cm (5.5 to 10). There have been no mechanical failures, no new infections and no re-operations. We suggest that in highly selected cases, in which conventional fixation is not feasible, this technique offers a durable option and avoids the morbidity of a total femoral replacement.
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 undertook this retrospective study to determine the rate of recurrence and functional outcome after intralesional curettage for chondroblastoma of bone. The factors associated with aggressive behaviour of the tumour were also analysed. We reviewed 53 patients with histologically-proven chondroblastoma who were treated by intralesional curettage in our unit between 1974 and 2000. They were followed up for at least two years to a maximum of 27 years. Seven (13.2%) had a histologically-proven local recurrence. Three underwent a second intralesional curettage and had no further recurrence. Two had endoprosthetic replacement of the proximal humerus and two underwent below-knee amputation after aggressive local recurrence. One patient had the rare malignant metastatic chondroblastoma and eventually died. The mean Musculoskeletal Tumour Society functional score of the survivors was 94.2%. We conclude that meticulous intralesional curettage alone can achieve low rates of local recurrence and excellent long-term function.
We reviewed our experience with diaphyseal endoprostheses to determine the survival of this type of reconstruction and factors affecting that survival.
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.
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 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%.
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.
Two collagen type IX gene polymorphisms that introduce a tryptophan residue into the protein’s triple-helical domain have been linked to an increased risk of lumbar disc disease. To determine whether a particular subset of symptomatic lumbar disease is specifically associated with these polymorphisms, we performed a prospective case-control study of 107 patients who underwent surgery of the lumbar spine. Patients were assigned to one of five clinical categories (fracture, disc degeneration, disc herniation, spinal stenosis without spondylolisthesis and spinal stenosis with spondylolisthesis) based on history, imaging results, and findings during surgery. Of the 11 tryptophan-positive patients, eight had spinal stenosis with spondylolisthesis and three had disc herniation. The presence of the tryptophan allele was significantly associated with African-American or Asian designation for race (odds ratio 4.61, 95% CI 0.63 to 25.35) and with the diagnosis of spinal stenosis with spondylolisthesis (odds ratio 6.81, 95% CI 1.47 to 41.95). Our findings indicate that tryptophan polymorphisms predispose carriers to the development of symptomatic spinal stenosis associated with spondylolisthesis which requires surgery.
A case of pencil-gore granuloma occurring 45 years after the initial injury is described. The clinical and radiological presentation was suggestive of a soft-tissue sarcoma. The diagnosis was confirmed by biopsy, which also revealed foreign body fragments which, on spectometric analysis, had high concentrations of carbon and aluminium.
We studied 100 patients who had undergone endoprosthetic replacement of the proximal humerus between 1976 and 1998. The outcome was assessed with regard to the survivorship of the patients, the salvaged limbs and the prostheses. Function was determined in the 47 surviving patients, of whom 30 were assessed using the Musculo-Skeletal Tumour Society (MSTS) rating scale and 38 completed the Toronto Extremity Salvage Score (TESS) questionnaire. The median age of the patients was 34 years (10 to 80). The mean follow-up period for surviving patients was nine years (2 to 20). The mean MSTS score at follow-up was 79% and the mean TESS score was 72%. The length of bone which was resected influenced the functional outcome. Abduction of the shoulder was to 45° in most patients. The overall survival was 42% at ten years and that of the limb without amputation 93%. The survival of the prostheses using mechanical failure as the endpoint was 86.5% at 20 years. Endoprosthetic replacement of the proximal humerus is a predictable procedure providing reasonable function of the arm with a low rate of complications at long-term follow-up.
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.
Ten patients underwent endoprosthetic replacement of the distal humerus for bone tumours over a period of 30 years. There were eight primary and two secondary tumours in four men and six women with a mean age of 47.5 years (15 to 76). The mean follow-up was eight years (9 months to 31 years). Four patients required further surgery, three having revision for aseptic loosening; two of these and one other later needing a rebushing. There were no cases of postoperative nerve palsy, infection, local recurrence or mechanical failure of the implant. Four patients died from their disease, all with the prosthesis functioning satisfactorily. At follow-up the mean flexion deformity of the elbow was 15° (0 to 35) and the mean range of flexion was 115° (110 to 135). The functional results showed a mean Toronto extremity salvage score of 73% of normal. Endoprosthetic replacement of the distal humerus and elbow is a satisfactory method of treating these rare tumours.
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.
This retrospective clinical study describes our experience of the use of growing endoprostheses in children with primary malignant tumours of the proximal femur and analyses the results. Between 1983 and 1996 we treated nine children with primary bone tumors of the proximal femur by resection and proximal femoral extensible replacements. Outcomes measured were function of the limb using Musculoskeletal Tumor Society score, oncologic outcome, complications and equalization of limb length. Results: Four patients died as a result of pulmonary metastases. The remaining five patients were observed for an average follow-up period of 7. 6 years (range 11–12. 7 years). One patient had a hindquarter amputation for uncontrolled infection. In these five patients we performed an average of 10. 2 operative procedures per patient (range of 3–17 procedures) including 5 lengthening procedures (range of 1–8 procedures) and a mean total extension of 69. 7 mm per patient. Acetabular loosening and hip dislocations were the most frequent complications. Only two patients have not had a revision or a major complication. Despite this, 4 children are alive with a functioning lower limb and a mean Musculoskeletal Tumour Society functional score of 77. 6%. The limb length discrepancy was less than 1 0 mm in three of these patients. The remaining patient has a discrepancy of 50 mm and is awaiting further limb equalization procedures. Extendible endoprostheses of the proximal femur in selected children is a viable reconstructive procedure. It allows for equalization of limb length and the ability to walk without the use of mobility aids.
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.
34 two-stage revision procedures were carried out between 1989 and 1998 for controlling deep infection following resection of bone tumours and reconstruction with endoprostheses. In 4 cases the procedure failed with early recurrent infection. In six others infection reappeared after further operative procedures. Six of these ten patients required amputation. The success of the procedure in controlling infection was 75% at 5 years. Two stage revision procedures have proved effective in controlling infection in massive endoprostheses but the risk of re-infection appears to be much greater in patients requiring further surgery for any cause.