Clear cell sarcoma of soft tissues is a rare, poorly understood tumour with little written about it in peer reviewed literature. The aim of this paper is to present a consecutive series of patients treated at our institution.

All patients were staged using the system of the musculo-skeletal tumour society (MSTS). The aim of surgery was to achieve a wide excision. Adjuvant chemotherapy or radiotherapy was used in some patients depending on the margins, age and general health of the patient. Follow-up comprised clinical examination, magnetic resonance imaging (MRI) of the tumour bed and chest x-rays. Patients were seen 3 monthly for the first 2 years and then 6 monthly.

Between 1997 and 2003 14 patients were included. There were 5 males and 9 females with a mean age of 49 years (21–82). Mean follow-up was 42 months (1–84). Seven tumours occurred in the upper limb and 7 in the lower limb. Four patients were lymph node positive at presentation. The mean maximum diameter of the tumour was 5.6 cm (2–8). Ten patients were referred prior to excision but 4 patients had already undergone inadvertent excision biopsy elsewhere. Four patients developed local recurrence and 3 patients developed metastases. Seven patients remain disease free, 2 have no active disease, 1 is alive with disease and 4 have died of the disease.

The 2 year survival in this series is 71%. Poor prognostic factors include positive lymph nodes at diagnosis, maximum diameter of the tumour greater than 5cm and incomplete initial excision. It is important that these patients are treated early and that wide excision is achieved. We recommend early referral to a recognised musculo-skeletal tumour centre.

Introduction Patients with musculo-skeletal tumours require appropriate staging investigations followed by prompt treatment. Biopsy of these lesions is hazardous and, when poorly performed, may compromise limb salvage surgery and patient survival. We examine the early management of such patients referred to our unit with particular reference to the biopsy.

Methods We conducted a prospective audit of all patients referred to our musculo-skeletal tumour service during 2002. Inclusion criteria were patients with a tumour of the musculo-skeletal system of unknown tissue diagnosis at presentation. Biopsies were performed either by the referring surgeon or the senior author. Patient demographics were recorded as well as details of the histological diagnosis, the site of the tumour and its stage. We recorded who performed the biopsy, the type of biopsy, the choice of biopsy site, whether or not adequate material was obtained and whether or not a poorly performed biopsy compromised the definitive treatment. We saw 162 patients (83 men and 79 women) with a mean age of 41 years (6 to 85). There were 81 primary bone tumours of which 40 were malignant, 58 primary soft tissue tumours of which 29 were malignant, 15 metastases from unknown primary tumours and eight haematological malignancies. One hundred and thirty-nine tumours were in the extremities, 12 pelvic, six trunk and six spinal. We saw patients at a mean of 11 days after receiving the referral letter. Of the 69 primary malignant tumours we saw 60 (87%) within two weeks and 67 (97%) within four weeks. The referring surgeon had performed biopsies in 29 cases.

Results Of the tumours biopsied by other surgeons, adequate diagnostic material was obtained in 75% compared to 99% in those biopsied by us. The biopsy site was suboptimal in 11/29 (38%). Fine needle aspiration (FNA) had been performed in 7/29 with only two (29%) providing diagnostic material. Poorly performed biopsy changed our definitive treatment in 5/29 (17%). Of those four had amputations and one patient received radiotherapy instead of further surgery. Twelve of 29 (41%) patients required re-excision of an incompletely excised tumour.

Conclusions Biopsies taken from sub-optimal sites may contaminate surrounding tissues, are frequently non-diagnostic and may compromise limb salvage surgery. FNA is particularly unreliable. We conclude that these patients are best served by early referral to a specialist centre where the biopsy can be performed quickly, safely and accurately.

Introduction Free, vascularised fibular grafting is well described in limb salvage surgery. The mechanical properties of the fibula make it ideal for replacement of bony defects after tumour resection and it can be sacrificed with minimal morbidity. We review the outcome of a consecutive series of 24 patients.

Methods Between 1993 and 2002 we performed free vascularised fibular grafts in 24 patients as part of a limb salvage procedure following tumour excision. Pre-operatively patients were staged using the Musculoskeletal Tumour Society (MSTS) system. Post-operatively patients were followed-up with radiographs and clinical examination. From the radiographs graft hypertrophy and time to bony union was documented. Functional outcome was assessed using the MSTS scoring system. Complications were recorded. There were 15 women and nine men with a mean age of 26 years (6 to 52). Mean follow-up was 51 months (12 to 106). There were 19 malignant tumours, all stage 2b, and five giant cell tumours. The mean length of graft was 12.5 cm (4.5 to25). Sixteen grafts were used in the upper limb and eight in the lower limb. Arthrodesis was performed in eight cases and intercalary reconstruction in 16 cases. Fixation of grafts was achieved with a plate and screws in 21, a blade plate in two and an IM nail in one. In six cases the resected tumour bone was reinserted as autograft after extracorporeal irradiation.

Results In all but one patient the tumour margins were clear. Primary bony union was achieved in 22 patients (92%) at a mean of 35 weeks (12 to 78). Graft hypertrophy was seen in 7/29 cases (24%). Complications included two wound breakdowns, three stress fractures, one muscle contracture, one malunion and one painful plate. Overall eight patients (33%) required second operation. Two patients died of recurrent disease and one has metastases. The mean MSTS functional score was 87% (80 to 93).

Conclusions Free vascularised fibula grafts offer a reliable method of reconstruction after excision of bone tumours. The complication rate appears high and some patients require a revision procedure. However, the problems are relatively easy to correct, bony union is achieved in the majority and functional outcome is good.

Introduction: The aim of this study is to compare the efficacy of the AO Universal Spine System (AO USS) with Harrington-Luque instrumentation for the treatment of King type II idiopathic scoliosis.

Methods/Results: A retrospective analysis was performed on two groups of patients with King II adolescent idiopathic scoliosis. The first group consisted of 40 consecutive patients treated with Harrington-Luque instrumentation between 1990 and 1993. The second group consisted of 25 consecutive patients treated with AO USS instrumentation between 1994 and 1996. The groups were well matched with respect to age, sex and curve severity. Inclusion criteria were patients over the age of 12 years with a King II curve pattern and a Cobb angle of greater than 40°. Half of the patients in each group underwent anterior release prior to posterior fusion. All patients were followed up six monthly for 18 months. The thoracic curve, lumbar curve, kyphosis and lordosis were measured using the Cobb method.

The mean pre-operative thoracic and lumbar curves were 62° and 43.9° respectively in the Harrington group and 57.5° and 35.9° in the AO USS group. On average 11.4 levels were fused in the Harrington group compared to 10.9 levels in the AO USS group. The mean post-operative correction of the thoracic curve in the AO USS group of 64% was significantly greater than the 51% achieved in the Harrington group (p< 0.005). At 18 months there was a 7% loss of correction in the Harrington group and 9% in the AO USS group. The correction of lumbar curve of 41% in the Harrington group and 46% in the AO USS group at 18 months was not significantly different. In the sagittal plane the AO USS group had significantly better preservation of the lumbar lordosis but there was no difference in kyphosis correction. Blood loss was similar in both groups. Mean operative time of 132 minutes in the AO USS group was shorter than the mean time of 153 minutes in the Harrington group (p< 0.05). Two hooks in the Harrington group became dislodged and two in the AO group. There were no neurological complications in either group. All the patients in both groups achieved a solid fusion.

Conclusion: AO USS is a safe and effective instrumentation system for the treatment of King type II adolescent idiopathic scoliosis. Correction of the thoracic curve is superior to that achieved with Harrington-Luque instrumentation and operative time is shorter. AO USS enables better preservation of the lumbar lordosis than Harrington-Luque. There is no difference in blood loss, complication rate and fusion rates between the two techniques. It has become our instrumentation system of choice for this group of patients.

A prospective study on total knee replacement in patients with Juvenile Idiopathic Arthritis was carried out. There were 31 knee replacements in 17 patients; 12 were female. Bilateral procedures were performed in 11 patients; 10 staged during a single admission and 1 performed in a single procedure. The mean age was 19 years (range, 14–26), and the mean follow-up was 23 months (range, 6–44). The surgery was complicated in 9 cases by a previous distal femoral osteotomy. The results were assessed using the Hospital for Special Surgery (HSS) scoring system. A cruciate sacrificing implant was used in all cases, and the patella was resurfaced in 25/31.

The pre-operative mean arc of motion was 62° (range 10°–90° ) and this averaged 89° (range, 20° –115°) at latest follow-up. The pre-operative mean fixed flexion 17° (range, 0° –15° ). The mean pre-operative HSS score was 36 (range, 8–59), and the mean at latest follow-up was 81 (range, 67–95). There has been no sign of aseptic loosening on serial radiographs over the follow-up period. One patient developed a deep infection at 20 months requiring a 2-stage revision and one patient developed a perforated duodenal ulcer postoperatively.

The clinical results are very satisfactory, allowing patients to regain their mobility and independence. The early radiographic results are very encouraging despite the poor bone quality and severe deformities that require correction.

The aim of this study is to evaluate the early results of gleno-humeral reconstruction after tumour excision with a new design of endoprosthesis.

The prosthesis is a fixed fulcrum gleno-humeral replacement consisting of a hydroxyapatite (HA) coated glenoid component with a polyethylene liner and a cemented stem with HA coated collar. Between 1997 and 2000 we inserted the prosthesis into 15 patients with primary bone tumours of the proximal humerus. There were nine males and six females with a mean age of 38 years (range: 8–71 years). Twelve stems were cemented and three uncemented. Two skeletally immature patients had an extendible stem inserted, one subsequently having a successful lengthening procedure. The mean follow-up was 28 months (range: 12–41 years). Functional outcome was assessed using the Musculoskeletal Tumour Society (MTS) scoring system.

There were two early dislocations and one superficial wound infection. Three patients died of their disease and one underwent forequarter amputation for local recurrence. The remaining eleven had satisfactory functional outcomes with a mean MTS score of 81%. Radiologically there has been no evidence of early loosening. Microscopic analysis of the components in the amputated arm showed excellent osseointegration around the HA coated components.