Aim: To identify patient, tumour or treatment factors that influence outcome in patients with radiation induced sarcoma of bone.

Method: A retrospective review of an oncology database supplemented by referral back to original records.

Results: We identified 42 patients who presented to our Unit over a 25 year period with a new sarcoma of bone following previous radiotherapy. The age of the patients at presentation ranged from 10 to 84 years of age (mean: 17 years) and the time interval from previous radiotherapy ranged from 4 to 50 years (median: 14 yrs; mean: 17 years). The median dose of radiotherapy given had been 50 Gy but there was no correlation of radiation dose with time to development of sarcoma. The pelvis was the most common site for development of sarcoma (14 cases) but breast cancer was the most common primary tumour (8 cases). 9 of the patients had metastases at the time of diagnosis of the sarcoma. Osteosacoma was the most common diagnosis (30). Treatment was by surgery and chemotherapy when indicated and 30 of the patients had treatment with curative intent. The survival rate was 41% at 5 years for those treated with curative intent but in those treated palliatively median survival was only 6 months and all had died by one year. The only factor found to be significant for survival was the ability to completely resect the tumour, thus limb sarcomas had a better prognosis (66% survival at 5 years) than central ones (12%)(p=0.009).

Conclusion: Radiation induced sarcoma is a rare complication of radiotherapy. Both surgical and oncological treatment is likely to be compromised by previous treatment the patient has received. Despite this 40% of patients will survive more than 5 years with aggressive modern treatment.

Background: Limb preserving surgery in patients with tumours involving the whole femur present a formidable challenge.

Results: We present our experience of treating such patients with total femur endoprostheses over the last 30 years (1975 to 2005). There were twenty six consecutive patients including 14 males and 12 females. Average age was 40 years (14 – 82 years) at the time of surgery. Eleven patients were still alive of which nine were free of disease at the time of review. The mean follow-up was 57 months (3 to 348). Using Kaplan Meier estimates, the long-term patient survival at 10 years was 37%. The survival of patients with primary localised tumour was 50% at 10 years.

Revision of the prostheses was necessary in two patients at 110 and 274 months after surgery because of recurrent dislocation in one and aseptic loosening of the acetabular cup and tibial stem in the other. Amputation was necessary in two patients, one due to deep infection and the other due to local recurrence. The long-term limb survival being 92% at 10 years. Nine patients who were alive with no evidence of disease were assessed for function of the salvaged limbs using the musculoskeletal tumour society (MSTS) rating system. The mean functional score was 72%.

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.

Purpose of study: Curettage with bone grafting is the accepted method of treating benign tumours of the talus. However, the natural history of ungrafted defects at this site is unknown. We report a series of 8 patients (6 male and 2 female) who underwent curettage of the talus without subsequent bone grafting.

Methods and results: 6 patients had chondroblastoma, one had osteoblastoma and one had an intraosseous ganglion. 4 lesions were located in the talar neck, 3 in the talar dome and one in the talar body. Mean age at presentation was 21.7 years. Mean tumour volume was 16cm3 (range 3.5–48cm3). Post-surgical follow-up was collected at a mean of 35 months (range 5–84 months).

The bone defect consolidated fully, with no talar collapse, in all 8 cases. 5 of the 8 patients had no pain and full range of movement at last follow-up. 4 patients had no evidence of osteoarthritis at last follow-up, 2 patients had OA grade 1, one had OA grade 2, and one had OA grade 3 pre-operatively which then progressed to grade 4. One patient had two episodes of local recurrence which were treated by curettage and bone grafting, then by radioablation.

Conclusion: Our results suggest that curettage alone leads to good consolidation of talar defects without an increase in complications. We conclude that bone grafting is not a necessary adjunct to the curettage of talar lesions.

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.

Conclusion: Improvements in design of distal femoral replacements have significantly decreased the risk of revision surgery. Infection remains a serious problem for these patients.

Aim: To investigate the outcome of prosthetic pelvic replacements., analyzing complications and suggesting ways of avoiding these

Method: We reviewed the records of all 52 patients undergoing custom made hemipelvic replacement of the pelvis at our centre over the past 30 years.

Results: The mean age of the patients was 40 (range 13 to 75) and the most common diagnosis was chondrosarcoma followed by Ewing’s sarcoma. 4 patients had metastases at the time if diagnosis. All tumours involved the acetabulum (P2) and 9 had a significant extension up into the ilium with 28 involving the pubis. There was a very high incidence of complications – local recurrence arose in 40% and infection in 32% at 5rs, there was a 10% risk of amputation (all for local recurrence). The overall survival of the patients was 69% at 5yrs and 59% at 10 yrs and the survival of the prosthesis without a major complication (infection or local recurrence) was only 40% at 5 and 10 yrs. Local recurrence was related to effectiveness of chemotherapy and margins whilst infection was increased by tumours involving the pubic area.

Discussion: Although a successful hemipelvic replacement can produce a good functional outcome, the incidence of complications is very high. Steps to reduce these risks should be carefully considered including the use of wider margins and the use of silver coated prostheses as well as greater use of local or free flaps in selected cases.

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.

Conclusion: 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.

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.

Conclusion: Resection of established non-union and reconstruction with endoprostheses is a good salvage operation for elderly and low demand patients in whom time consuming biological reconstruction is not desirable.

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.

Conclusion: Chemotherapy allows fractures to consolidate with conservative treatment. Adequate surgical margins can be achieved in the majority of patients with limb saving surgery. Adjuvant radiotherapy does not seem necessary if margins are wide. Survival is not negatively influenced by pathological fracture. The survival rate following limb saving surgery in these patients is similar to that of patients in literature where amputation is done. Limb saving surgery seems a safe option.

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.

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 have investigated the significance of the method of treatment on the oncological and functional outcomes and on the complications in 184 patients with soft-tissue sarcomas of the adductor compartment managed at three international centres. The overall survival at five years was 65% and was related to the grade at diagnosis and the size of the tumour. There was no difference in overall survival between the three centres. There was, however, a significant difference in local control with a rate of 28% in Centre 1 compared with 10% in Centre 2 and 5% in Centre 3.

The overall mean functional score using the Toronto Extremity Salvage Score in 70 patients was 77% but was significantly worse in patients with wound complications or high-grade tumours. The scores were not affected by the timing of radiotherapy or the use of muscle flaps.

This large series of soft-tissue sarcomas of the adductor compartment has shown that factors influencing survival do not vary across the international boundaries studied, but that methods of treatment affect complications, local recurrence and function.

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.

Aim: To identify prognostic for patients who develop local recurrence after initial attempted curative treatment for a soft tissue sarcoma (STS).

Method: All patients who developed a local recurrence (LR) after initial primary treatment of a STS were identified from a prospective database. Their management and outcome were analysed to find prognostic factors.

Results: 178 patients were identified. They had a median age at original diagnosis of 53 and 102 of the patients had high grade tumours, 50 intermediate grade and 23 low grade. The median time to LR was 14 months but extended up to 11.5 years. 47 of the patients developed metastases either before or synchronously with the LR. In these patients the median survival was 20 months with only 4% surviving to 5 years. In the 131 patients who did not have identifiable metastases at the time of diagnosis, 74 subsequently developed metastases at a median time of 12 months following the development of LR.

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.

Conclusion: Local recurrence has a poor prognosis but this is because it frequently arises in patients who have other bad prognostic factors. Whilst obtaining local control is important, overall survival is poor, but not as bad for those patients who develop metastases.

Aims: To establish the frequency and demographics of soft tissue sarcomas (STS) presenting in the lower limb.

Methods: Patients presenting to a tertiary referral orthopaedic oncology unit over a 10-year period were prospectively entered into a computerised database. The site of primary STS and demographic details were also recorded.

Results: 1519 STS in all body regions were treated. 1067 (70.2%) within the lower limb. 57.0% thigh, 13.0% calf, 8.2% foot and ankle, 7.7% buttock, 5.7% knee, 4.6% pelvis and 3.8% in the groin. There was a male predominance (56.2%). M:F ratio was 2.5:1 for the groin and 1.3:1 for the thigh with the other body regions approximately equal.

Conclusion: The majority of STS are found in the lower limb. In this large series there was a male predominance most marked in groin presentations.

Aims: Soft tissue sarcomas (STS) of the foot and ankle are rare tumours. The aims of this study were to examine the presenting features and highlight those associated with a delay in diagnosis.

Methods: Patients presenting during a 10-year period were identified using a computerised database within the Orthopaedic Oncology Unit at the Royal Orthopaedic Hospital, Birmingham, UK. Additional information was obtained from a systematic case note review.

Results: 1519 patients were treated for STS of which 87 (8.2%) had tumours sited in the foot and ankle. Of these, 75 (86.2%) had presented with a discrete lump (42 (56%) of them having an inadvertent “whoops” excision biopsy), 3 (3.4%) with ulceration and the remaining 9 (10.3%) with symptoms more commonly associated with other benign foot and ankle pathology. Within the group of 9 patients they had previously been treated as plantar fasciitis (3), tarsal tunnel syndrome (2), Morton’s neuroma (1) and none specific hind foot pain (3). Median delay from onset of symptoms to diagnosis as STS was 26 months for this group (mean 50; range 6–180 months) compared to 12 months (mean 32; range 3–240) for the “whoops” biopsy group and 10 months (mean 16; range 2–60 months) for the unbiopsied discrete lump group.

Conclusion: Soft tissue sarcoma in the foot and ankle may present insidiously and with symptoms of other benign pathologies. Failure to respond to initial treatment of suspected common benign pathology should be promptly investigated with further imaging e.g. MRI scan or high resolution ultrasound, or with specialist consultation.