The definition of a ‘safe’ tumour margin remains controversial. Enneking’s original definition of intralesional, marginal, wide and radical margins was based on a study of just 40 patients of whom only 12 had limb salvage surgery. Since that time thre have been numerous attempts to try and define tumour margins more clearly based on anatomical structures and distances of the tumour from the cut edge. Whilst all can agree on what is a radical margin (usually an amputation) and what is an intralesional margin (tumour exposed) there remains a lack of clear definition of what comprises a wide margin and what is a marginal margin. As an example three large tumour units were asked to assess their margins for adequacy. While the rated of adequate excision varied from 49% to 70%, all 3 units had near identical local recurrence rates of 17–20%. The rate of local recurrence is also modified by adjuvant therapy. A suggestion for an International study assessing tumour margins is made, investigating both anatomical and biological factors.
Overall cumulative patient survival was 58% at 5 years and 44% at 10 years. Locally recurrent disease occurred in 350 patients (14%), 204 patients (8%) presented with and 720 patients (30%) subsequently developed metastatic disease. Prognostic factors for locally recurrent disease were arm tumours (p=0.003, HR=0.3), hip tumours (p=0.01, HR=0.31), thigh tumours (p=0.002, HR=0.52), intralesional margins (p<
0.0001, HR=3.7), high grade tumours (p=0.03, HR=1.8), tumour size 3–6cm (p=0.04, HR=0.54) and tumour size 6–10cm (p=0.03, HR=0.63). Prognostic factors for patient survival were deep location (p=0.02, HR=1.6), high grade tumours (p<
0.0001, HR=4.7), intermediate grade tumours (p<
0.0001, HR=3.4), surgical margins (p=0.04), age at diagnosis (p<
0.0001, HR=1.02), size of tumour <
3cms (p=0.04, HR=0.29), 3–6cms (p<
0.0001, HR=0.41), 6–10cms (p=0.007, HR=0.63), no locally recurrent disease (p=0.0001, HR=0.59).
those 7 whose LR was inoperable due to size, those 7 patients with LR_5cm who had concomitant metastases and in the 5 patients who already had maximum doses of radiotherapy.
We reviewed the treatment and clinical outcome of 32 consecutive patients with Ewing’s sarcoma who presented with or developed pathological fracture after biopsy between 1984 and 2004. The minimum follow-up was 18 months. The mean age at diagnosis was 20 years (5 – 51). There were 18 males and 14 females. All patients were newly diagnosed and had localized disease at the time of diagnosis. 21 patients presented with pathological fracture while 11 patients developed fracture during the course of chemotherapy. The femur was the most common location in 15 patients. All the patients had chemotherapy according to the protocol current at the time of treatment. 6 patients had radiotherapy alone while 26 patients underwent surgical excision and reconstruction. Of the patients who had surgery, 7 patients had adjuvant radiotherapy. Fracture healing was the norm after pre-operative chemotherapy. Surgical margins were wide in 17 patients, marginal in 4 and intralesional in 3 patients. Local recurrence developed in one patient (3%). Metastases occurred in 12 patients (37%). At the time of review 16 patients were free of disease, 3 were alive with disease and 13 patients had died of disease. The cumulative 5 year metastases free and overall survival in all the patients was 58% and 61 % respectively and similar to patients with Ewing’s sarcoma without fracture treated at our centre. The prognosis of patients who presented with fracture was exactly similar to those who developed fracture in the course of treatment. We conclude that limb preserving surgery is perfectly safe in patients with Ewing’s sarcoma who have associated pathological fracture and survival is not in any way compromised. Survival of patients who present with fracture is similar to those who develop fracture in the course of treatment. The exact role of adjuvant radiotherapy in these patients needs to be clarified.
We have investigated whether improvements in design have altered outcome for patients undergoing endoprosthetic replacement of the distal femur following tumour resection. Survival of the implant and ‘servicing’ procedures has been documented using a prospective database and review of the implant design records and case records. A total of 335 patients underwent a distal femoral replacement with 162 having a fixed hinge design and 173 a rotating hinge. The median age of the patients was 24 years (range 13–82yrs). With a minimum follow up of 5 years and a maximum of 30 years, 192 patients remain alive with a median follow up of 11 years. The risk of revision for any reason was 17% at 5 years, 33% at 10 years and 58% at 20 years. Aseptic loosening was the most common reason for revision in the fixed hinge knees whilst infection and stem fracture were the most common reason in the rotating hinges. The risk of revision for aseptic loosening in the fixed hinges was 35% at ten years but appears to have been abolished by the use of a the rotating hinge knee with a hydroxyapatite collar. The overall risk of revision for any reason was halved by use of the rotating hinge. Improvements in design of distal femoral replacements have significantly decreased the risk of revision surgery but infection remains a serious problem for all patients. We believe that a cemented, rotating hinge prosthesis with a hydroxyapatite collar offers the best chance of long term prosthesis survival.
We reviewed the treatment and clinical outcome of 32 consecutive patients with Ewing’s sarcoma who presented with or developed pathological fracture after biopsy between 1984 and 2004. The minimum follow-up was 18 months. The mean age at diagnosis was 20 years (5 – 51). There were 18 males and 14 females. All patients were newly diagnosed and had localized disease at the time of diagnosis. 21 patients presented with pathological fracture while 11 patients developed fracture during the course of chemotherapy. The femur was the most common location in 15 patients. All the patients had chemotherapy according to the protocol current at the time of treatment. 7 patients had radiotherapy alone while 25 patients underwent surgical excision and reconstruction. Of the patients who had surgery, 7 patients had adjuvant radiotherapy. Fracture healing was the norm after pre-operative chemotherapy. Surgical margins were wide in 17 patients, marginal in 4 and intralesional in 3 patients. Local recurrence developed in one patient (3%). Metastases occurred in 12 patients (37%). At the time of review 16 patients were free of disease, 3 were alive with disease and 13 patients had died of disease. The cumulative 5 year metastases free and overall survival in all the patients was 58% and 61 % respectively and similar to patients with Ewing’s sarcoma without fracture treated at our centre. The prognosis of patients who presented with fracture was exactly similar to those who developed fracture in the course of treatment. We conclude that limb preserving surgery is perfectly safe in patients with Ewing’s sarcoma who have associated pathological fracture and survival is not in any way compromised. Survival of patients who present with fracture is similar to those who develop fracture in the course of treatment. The exact role of adjuvant radiotherapy in these patients needs to be clarified.
Synovial sarcoma is a morphologically well-defined neoplasm that most commonly occurs in soft tissue accounting for 5% to 10 % of all soft tissue sarcomas. We reviewed 156 patients with synovial sarcoma of soft tissues treated at a supra-regional centre to determine survival and prognostic factors. There were 77men and 79 women with mean age at presentation of 38 years (3 to 84). Follow-up periods ranged from 3 to 494 months (median 43 months). Tumor was located in lower extremities in 111patients, upper extremities in 34 patients, and trunk and pelvis in 11 patients. Overall survival was 66% at 5 years and 48% at 10 years. The 5 and 10 year survival for the 23 patients who had metastases at the time of diagnosis was 13% and 0% respectively compared to 75% and 54% for those without metastases at diagnosis. Local recurrence occurred in 18 patients (13%). The significant prognostic factors for survival included presence of metastases at diagnosis and development of local recurrence. Tumour size and depth, age of patients and use of chemotherapy did not significantly influence survival. We conclude that the clinical factors which influence survival of patients with synovial sarcoma are different from those of soft tissue sarcomas in general. Biological factors may better predict prognostic survival than the usual clinical factors.
Four patients had obvious infection confirmed by histology and/or microbiology prior to surgery. Endoprosthetic Reconstruction was performed as a 1 stage procedure in 13 and as a 2 stage in 4. Complications occurred in 5 patients. These included recurrence of infection in 1, persistent pain in 1, aseptic loosening in 1, periprosthetic fracture in 1 and a non ST myocardial infarction in 1. At the last follow-up, (mean 5years, range 1–18years) majority of patients achieved good range of motion and good mobility.
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.
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.