Introduction Myxofibrosarcoma is described by WHO as comprising of a spectrum of malignant fibroblastic lesions with variably myxoid stoma, pleomorphism and with a distinctively curvilinear vascular pattern. They are reported to be one of the commonest sarcomas of elderly patients. It has previously been reported to have a high rate of locally recurrent disease (50-60%). The aim of the study was to investigate the ROH series of tumours to determine prognostic factors for survival and local recurrence. Methods Patients were identified from the ROH database who had been treated with a new presentation of myxofibrosarcoma. The size, grade and depth of the tumour at presentation was noted. Those patients who had suffered local or distal disease relapse or died were highlighted. Analysis was undertaken by Kaplan Meier survival curves for univariate and cox regression for multivariate analysis. Results 90 patients were indentified who had undergone surgery for myxofibrosarcoma. The mean age was 65 years (range 23-93 years) with an equal male to female ratio. They presented deep to fascia in 49 cases (54%) and were most commonly located in the lower extremity in 53 cases (59%) with 32 cases being in the thigh. The maximum dimension at presentation was 9cm (range 0.3 to 42cm). The grade at presentation was high in 41 cases, intermediate in 35 cases and low in 14 cases. The risk of local recurrence was 25% at 5 years and was independent of margin or size. Overall survival was 59% at 5 years with high grade tumours being the only independent prognostic factor for survival at 5 years (HR=10). Conclusion Myxofibrosarcoma is a tumour that often presents superficially with a high rate of local recurrence. Other studies (Gronchi 2010) have suggested that size and surgical margins were prognostic but this was not seen in our study.

All patients referred to our unit with previously untreated metastatic renal cancer were included in this review. We investigated likely prognostic factors including age, sex, site, synchronous or metachronous metastasis, stage of the disease and the type of treatment received. From 1976 until 2004, a total of 198 patients were treated by our unit for renal metastases. 15 patients were excluded because they were referred after failure of previous treatment or only had advice. 96 patients were already known to have renal metastasis with their diagnosis having been made between 0.2 and 17 years from the diagnosis of primary cancer (mean 4 years). 33 patients presented to us with a pathological fracture and were found to have renal cancer. A total of 54 patients had multiple metastases and 129 had a solitary metastasis. The cumulative survival from the time of diagnosis of the bone metastasis is 70 percent at 1 year, 40% at 3 years and 18% at 5 years. In patients with a solitary metastasis, the overall survival was 74% at 1 year and 45% at 3 years, whereas in patients with multiple metastases it was 55% at 1 year and 22% at 3 years. (p=0.02) In patients with a solitary metastasis treated by excision of the metastasis, the survival at 1 year was 86% as compared to 38% for those that were treated with just a local procedure. Cox multivariate analysis shows that survival was better in those with solitary metachronus metastasis who underwent a radical procedure. Conclusion We recommend a radical procedure for patients who present with a solitary renal metastasis, particularly those with a disease-free interval of more than one year.

Introduction: Limb salvage reconstruction evolved from the treatment of primary bone tumours. Endoprosthetic replacements (EPR) were originally designed for this purpose, but the versatility of these implants has resulted in an extension in the indications for their use. Severe bone loss, failed revision surgery and persistent deep infection present similar challenges and when a salvage procedure is required, EPR are occasionally used. The aim of our study was to assess the medium term survival and functional outcome of EPR. Materials and Methods: 38 patients (23 females and 15 males), who underwent EPR for non-neoplastic conditions were identified from a prospectively kept database of all patient seen at the Royal Orthopaedic Hospital Oncology Service. The indications for replacement included failed joint replacement, fracture non-union, failed internal fixation and periprosthetic fractures. 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. Results: Patients had a mean age of 60 years (range 15–85 years) at surgery and had between 0 and 4 previous operations prior to EPR. Seven out of 38 patients had recorded deep infection prior to surgery (18%). The Kaplan-Meier implant survival was 91.3% at 5 years, 68.5% at 10 years and 45.7% at 20 years. The limb salvage survival for all reconstructions was 75% at 10 years. 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. Conclusion: EPR appears to be effective and the medium term survival is encouraging. The aim of a pain free functional limb is achievable with this technique. The complication rates are acceptable considering the salvage nature of these patients. We recommend referral of complex cases to a tertiary centre with expertise in this type of surgery.

Abstract

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.

Metastatic bone disease resulting in acetabular destruction can provide the orthopaedic surgeon with the difficult challenge of achieving a stable reconstruction of the hip to provide pain relief and restoration of mobility. We review of twenty patients with metastatic disease requiring major acetabular reconstruction presenting to our orthopaedic oncology unit over a five year period was undertaken. This yielded 15 female and 5 male patients with mean age 59 years. The primary lesion was breast (8 cases), renal (3) prostate (2), myeloma (2) and others (5) with a solitary acetabular metastasis in 75% of cases. Eight patients had received radiotherapy to the region pre-operatively. In all cases, diseased bone was macroscopically cleared from the pelvis and reconstruction performed by means of a Harrington procedure with threaded pins passed antegrade from the iliac crest 915 cases) or mesh and screws (5 cases), all reinforced with cement around which a total hip arthroplasty was performed. Mean follow-up was 16 months. Complications were broken pin (1 case), dislocation of femoral prosthesis (1) and deep venous thrombosis (1). Three patients died of their disease at a mean of 12 months from surgery. The remaining 17 patients continue to function at a satisfactory level with no patients having required revision surgery for loosening or deep infection. We believe that surgical reconstruction of the acetabulum is worthwhile and can provide these deserving patients with improvement in quality of life.

Introduction: A ‘whoops’ procedure is when a lump, which subsequently turns out to be a soft tissue sarcoma (STS), is shelled out by a surgeon who is not aware of the diagnosis. In many cases residual tumour will be left behind necessitating further surgery. The significance of a whoops procedure in terms of survival and local control remains uncertain. This study has used case matched controls to compare outcome between two groups. Method: 794 patients of soft tissue sarcoma with minimum follow up of 5 years were found on our prospectively collected database. 113 were whoops cases, 96 had restaging and reexcision. An observer blinded to the outcome of patients matched the whoops cases with virgins by known prognostic factors i.e. grade, depth, patient age, site, size and diagnosis of the tumour. We have investigated outcome in terms of local control, metastatic disease and survival by known prognostic factors and by their status at presentation. Results: 96 patients with a whoops procedure were compared with 96 referred directly to our unit. Despite attempts to match patients with as many variables as possible there was a tendency for the patients with whoops to have smaller tumours that were subcutaneous, they were however well matched for grade and stage at diagnosis. 64% of whoops patients had adequate final margin whereas only 44% of virgins had adequate margins. Overall 1.43 additional operations were needed to achieve final margins for whoops cases as against 0.21% for virgin cases. Overall 27% patients had amputation 20% for whoops and 34% for virgin cases nearly 60% were ray amputations of foot or hand. Overall 50% had radiotherapy and 25% had chemotherapy. There was no statistical difference in local recurrence or survival of patients between whoops and virgins at 5 years follow up. Inadequate margins and residual tumour were significant risk factors for local recurrence and high grade, size more than 5 cm, and age more than 50 years were significant prognostic factors for overall survival of the patients. Conclusion: Inadvertent surgical excision of a STS is not desirable but does not seem to lead to an adverse outcome in this series in which wide re-excision of the area involved has been carried out.

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.