Introduction: Following resection of tumours in the distal femur, reconstruction with joint-sparing prostheses have shown good short-term functional outcomes. There is however limited literature on the affect of knee-sparing prostheses on function of the femoral physes in skeletally immature children.

Method: We discuss two patients, a male (11yrs) and female (10yrs) who had joint-sparing distal femoral prostheses inserted for treatment of Ewing’s sarcoma. The knee joint, along with the distal growth plate, was preserved and fixed to the distal end of the prosthesis using unicortical screws positioned distal to the physis. In the female, these screws were removed 6 months post-op due to prominence of the screws under the skin. In both patients, we assessed radiographs from immediately post-surgery and the most recent follow-up (20 and 28 months respectively). In each set, for the operated limb, we measured the height and width of the distal femoral epiphysis, the total length of the femur and the length of the proximal femoral bone segment from the femoral head to the proximal bone-prosthesis interface. In addition, post-operative assessments of leg lengths, bilaterally, were documented.

Results: In both patients, distal femoral epiphyseal height and width in the operated leg showed no significant change following endoprosthetic replacement. In the female, growth did not resume even after removal of the epiphyseal screws. In both patients, lengths of the femur and the proximal bone segment increased significantly following surgery. The patients demonstrated no clinical leg length discrepancy at the most recent follow-up.

Discussion: This study suggests that the function of the distal femoral growth plate ceases following insertion of joint-sparing distal femoral endoprostheses, probably due to trans-physeal fixation. This does not appear to resume following early removal of distal screws. The proximal growth plate, however, continues to function adequately enough to maintain symmetry in overall leg length.

Introduction: Chondrosarcoma is the second most common primary malignant bone tumour. Distinguishing between grades is not necessarily straightforward and may alter the management of the disease. We evaluated the correlation between the pre-operative needle biopsy and excision biopsy histological grading of chondrosarcoma of the femur, tibia and humerus.

Methods: A consecutive retrospective series of 100 patients with a histological diagnosis of chondrosarcoma made at a supra-regional bone tumour unit was reviewed. Twenty-one patients were excluded because 20 had only excision biopsy, due to radiological confidence in the diagnosis, and one had only the pre-operative biopsy on record, thus this series included 79 available cases. The remaining patients underwent a pre-operative needle biopsy.

Results: In 11 instances, there was a discrepancy in histological grade. Therefore, there was an 86% (68 out of 79) accuracy rate for pre-operative histological grading of chondrosarcoma, based on needle biopsy. However, the accuracy of the diagnostic biopsy to distinguish low-grade from high-grade was 90% (71 out of 79).

Discussion: From this series we conclude that accurate image-guided biopsy is a very useful adjunct in determining histological grade of chondrosarcoma and the subsequent treatment plan. At present, a multidisciplinary approach, comprising experienced Orthopaedic Surgeons, Radiologists and Pathologists offers the most reliable means of accurately diagnosing and grading chondrosarcoma of long bones.

Introduction: Alveolar soft part sarcoma (ASPS) is a soft tissue tumour found primarily in adolescents and young adults. It has an incidence of 0.5 – 1.0 % of all soft tissue sarcomas and a predilection for females. The lesion is inherently malignant and may occur throughout the body however, reports suggest they are predominantly detected in the anterior thigh. Symptoms include a painless slow-growing mass but ASPS can commonly present with brain or lung metastases due to the high vascularity of the lesion. Microscopically, cellular groups exist with centralised areas of necrosis, thus resulting in a pseudoalveolar appearance.

Methods: We identified six cases of histologically proven ASPS. The mean age was 28.5 years (21–36). Four patients were male. All patients had a primary presentation of a mass, two of which were painful. All patients, except one, presented within 8 months of the onset of symptoms (mean 3.8 months) with the other seeking medical advice only after 72 months. The location of the mass included the thigh in three patients and the triceps, soleus and retroperitoneal space in the others. Four patients underwent pre-operative diagnostic biopsies. Surgical excision was performed in all patients.

Results: The mean size of tumour was 9.4 x 9.4 x 6.3cm but there was no correlation between lesion size and duration of symptoms. The patient who presented late was found to have multiple lung metastases prior to surgery. Despite appropriate adjuvant therapy, three patients developed post-operative metastases at a mean of 5.2 months (1–24), two of which died along with the patient who presented with metastases at a mean of 2.6 years.

Discussion: This series demonstrates that this tumour may present as a painful mass. Despite early detection and appropriate treatment, ASPS remains a highly malignant neoplasm with a high associated mortality rate.

We have reviewed five adult patients treated with endoprosthetic reconstruction of the proximal radius following resection of non-traumatic lesions. The patients had a mean age of 33.4 years (20 to 60) at the time of surgery and the mean follow-up was 7.6 years (0.8 to 16).

Following surgery, all elbows were clinically stable and there was 100% survivorship of the prosthesis. Evaluation of function was assessed clinically and by the Mayo Elbow Performance Score, achieving a mean of 86% (70 to 100).

Results at medium-term follow-up are encouraging with regards to elbow stability, implant survivorship and functional outcome.

Although bone and soft-tissue sarcomas are rare, early diagnosis and prompt referral to a specialised unit offers the best chance of a successful outcome both in terms of survival and surgical resection. This paper highlights the clinical and radiological features that might suggest the possibility of a bone or soft-tissue sarcoma and suggests a succinct management pathway for establishing whether a suspicious bone or soft-tissue lesion could be malignant.

Disarticulation of the hip in patients with high-grade tumours in the upper thigh results in significant morbidity. In patients with no disease of the proximal soft tissue a femoral stump may be preserved, leaving a fulcrum for movement and weight-bearing. We reviewed nine patients in whom the oncological decision would normally be to disarticulate, but who were treated by implantation of an endoprosthesis in order to create a functioning femoral stump. The surgery was undertaken for chondrosarcoma in four patients, pleomorphic sarcoma in three, osteosarcoma in one and fibrous dysplasia in one. At follow-up at a mean of 80 months (34 to 132), seven patients were alive and free from disease, one had died from lung metastases and another from a myocardial infarction. The mean functional outcome assessment was 50 (musculoskeletal tumor society), 50 and 60 (physical and mental Short-form 36 scores).

Implantation of an endoprosthesis into the stump in carefully selected patients allows fitting of an above-knee prosthesis and improves wellbeing and the functional outcome.

Segmental resection of malignant bone disease in the femoral diaphysis with subsequent limb reconstruction is a major undertaking. This is a retrospective review of 23 patients who had undergone limb salvage by endoprosthetic replacement of the femoral diaphysis for a primary bone tumour between 1989 and 2005.

There were 16 males and seven females, with a mean age of 41.3 years (10 to 68). The mean overall follow-up was for 97 months (3 to 240), and 120 months (42 to 240) for the living patients. The cumulative patient survival was 77% (95% confidence interval 63% to 95%) at ten years. Survival of the implant, with failure of the endoprosthesis as an endpoint, was 85% at five years and 68% (95% confidence interval 42% to 92%) at ten years. The revision rate was 22% and the overall rate of re-operation was 26%. Complications included deep infection (4%), breakage of the prosthesis (8%), periprosthetic fracture (4%), aseptic loosening (4%), local recurrence (4%) and metastases (17%). The 16 patients who retained their diaphyseal endoprosthesis had a mean Musculoskeletal Tumour Society score of 87% (67% to 93%). They were all able to comfortably perform most activities of daily living.

Femoral diaphyseal endoprosthetic replacement is a viable option for reconstruction following segmental resection of malignant bone disease. It allows immediate weight-bearing, is associated with a good long-term functional outcome, has an acceptable complication and revision rate and, most importantly, does not appear to compromise patient survival.

Elastofibroma dorsi is an uncommon, benign, slow-growing soft-tissue tumour of uncertain aetiology. It classically presents as an ill-defined mass at the inferior pole of the scapula with symptoms which include swelling, discomfort, snapping, stiffness and occasionally pain.

We report the symptoms, function and outcome after treatment of 21 elastofibromas in 15 patients. All were diagnosed by MRI and early in the series four also underwent CT-guided biopsy to confirm the diagnosis. In all, 18 tumours were excised and three were observed. After excision, the mean visual analogue score for pain decreased from 4.6 (0 to 10) pre-operatively to 2.4 (0 to 8) post-operatively (p = 0.04). The mean shoulder function, at a mean follow-up of 4.2 years (3 months to 16 years), was 78.1% (30 to 100) using the Stanmore percentage of normal shoulder assessment scoring system. The mean range of forward flexion improved from 135° (70° to 180°) to 166° (100° to 180°) after excision (p = 0.005). In four patients a post-operative haematoma formed; one required evacuation. Three patients developed a post-operative seroma requiring needle aspiration and one developed a superficial infection which was treated with antibiotics.

Our findings support previous reports suggesting that a pre-operative tissue diagnosis is not necessary in most cases since the lesion can be confidently diagnosed by MRI, when interpreted in the light of appropriate clinical findings. Surgical excision in symptomatic patients, is helpful.

It has been suggested that elastofibroma is caused by a local tissue reaction and is not a true neoplastic process. A strong association has been noted between elastofibroma and repetitive use of the shoulder, which is supported by our findings.

Smoking is known to have an adverse effect on wound healing and musculoskeletal conditions. This case-controlled study looked at whether smoking has a deleterious effect in the outcome of autologous chondrocyte implantation for the treatment of full thickness chondral defects of the knee.

The mean Modified Cincinatti Knee score was statistically significantly lower in smokers (n = 48) than in non-smokers (n = 66) both before and after surgery (p < 0.05). Smokers experienced significantly less improvement in the knee score two years after surgery (p < 0.05). Graft failures were only seen in smokers (p = 0.016). There was a strong negative correlation between the number of cigarettes smoked and the outcome following surgery (Pearson’s correlation coefficient −0.65, p = 0.004).

These results suggest that patients who smoke have worse pre-operative function and obtain less benefit from this procedure than non-smokers. The counselling of patients undergoing autologous chondrocyte implantation should include smoking, not only as a general cardiopulmonary risk but also because poorer results can be expected in smokers following this procedure.

We undertook a retrospective review of 33 patients who underwent total femoral endoprosthetic replacement as limb salvage following excision of a malignant bone tumour. In 22 patients this was performed as a primary procedure following total femoral resection for malignant disease. Revision to a total femoral replacement was required in 11 patients following failed segmental endoprosthetic or allograft reconstruction. There were 33 patients with primary malignant tumours, and three had metastatic lesions. The mean age of the patients was 31 years (5 to 68). The mean follow-up was 4.2 years (9 months to 16.4 years). At five years the survival of the implants was 100%, with removal as the endpoint and 56% where the endpoint was another surgical intervention. At five years the patient survival was 32%. Complications included dislocation of the hip in six patients (18%), local recurrence in three (9%), peri-prosthetic fracture in two and infection in one. One patient subsequently developed pulmonary metastases. There were no cases of aseptic loosening or amputation. Four patients required a change of bushings. The mean Musculoskeletal Tumour Society functional outcome score was 67%, the mean Harris Hip Score was 70, and the mean Oxford Knee Score was 34.

Total femoral endoprosthetic replacement can provide good functional outcome without compromising patient survival, and in selected cases provides an effective alternative to amputation.

Endoprosthetic replacement of the distal tibia and ankle joint for a primary bone tumour is a rarely attempted and technically challenging procedure. We report the outcome of six patients treated between 1981 and 2007. There were four males and two females, with a mean age of 43.5 years (15 to 75), and a mean follow-up of 9.6 years (1 to 27). No patient developed a local recurrence or metastasis. Two of the six went on to have a below-knee amputation for persistent infection after a mean 16 months (1 to 31). The four patients who retained their endoprosthesis had a mean musculoskeletal tumour society score of 70% and a mean Toronto extremity salvage score of 71%. All were pain free and able to perform most activities of daily living in comfort.

A custom-made endoprosthetic replacement of the distal tibia and ankle joint is a viable treatment option for carefully selected patients with a primary bone tumour. Patients should, however, be informed of the risk of infection and the potential need for amputation if this cannot be controlled.

This paper describes the preliminary results of a proximal tibial endoprosthesis which spares the knee joint and enables retention of the natural articulation by replacing part of the tibial metaphysis and diaphysis. In eight patients who had a primary malignant bone tumour of the proximal tibia, the distal stem, which had a hydroxyapatite-coated collar to improve fixation, was cemented into the medullary canal. The proximal end had hydroxyapatite-coated extracortical plates which were secured to the remaining proximal tibial metaphysis using cortical screws. The mean age of the patients at operation was 28.9 years (8 to 43) and the mean follow-up was for 35 months (4 to 48). The mean Musculoskeletal Tumour Society score was 79% (57% to 90%), the mean Oxford Knee score was 40 points of 48 (36 to 46) and the mean knee flexion was 112° (100° to 120°). In one patient, revision to a below-knee amputation through the prosthesis was required because of recurrence of the tumour. Another patient sustained a periprosthetic fracture which healed with a painful malunion. This was revised to a further endoprosthesis which replaced the knee.

In the remaining six patients the prosthesis allowed preservation of the knee joint with good function and no early evidence of loosening. Further follow-up is required to assess the longevity of these prostheses.

We describe the application of a non-invasive extendible endoprosthetic replacement in skeletally-mature patients undergoing revision for failed joint replacement with resultant limb-length inequality after malignant or non-malignant disease. This prosthesis was developed for tumour surgery in skeletally-immature patients but has now been adapted for use in revision procedures to reconstruct the joint or facilitate an arthrodesis, replace bony defects and allow limb length to be restored gradually in the post-operative period.

We record the short-term results in nine patients who have had this procedure after multiple previous reconstructive operations. In six, the initial reconstruction had been performed with either allograft or endoprosthetic replacement for neoplastic disease and in three for non-neoplastic disease. The essential components of the prosthesis are a magnetic disc, a gearbox and a drive screw which allows painless lengthening of the prosthesis using the principle of electromagnetic induction. The mean age of the patients was 37 years (18 to 68) with a mean follow-up of 34 months (12 to 62). They had previously undergone a mean of six (2 to 14) open procedures on the affected limb before revision with the non-invasive extendible endoprosthesis.

The mean length gained was 56 mm (19 to 107) requiring a mean of nine (3 to 20) lengthening episodes performed in the outpatient department. There was one case of recurrent infection after revision of a previously infected implant and one fracture of the prosthesis after a fall. No amputations were performed. Planned exchange of the prosthesis was required in three patients after attainment of the maximum lengthening capacity of the implant. There was no failure of the lengthening mechanism. The Mean Musculoskeletal Tumour Society rating score was 22 of 30 available points (18 to 28).

The use of a non-invasive extendible endoprosthesis in this manner provided patients with good functional results and restoration of leg-length equality, without the need for multiple open lengthening procedures.

Chondral damage to the knee is common and, if left untreated, can proceed to degenerative osteoarthritis. In symptomatic patients established methods of management rely on the formation of fibrocartilage which has poor resistance to shear forces. The formation of hyaline or hyaline-like cartilage may be induced by implanting autologous, cultured chondrocytes into the chondral or osteochondral defect.

Autologous chondrocyte implantation may be used for full-thickness chondral or osteochondral injuries which are painful and debilitating with the aim of replacing damaged cartilage with hyaline or hyaline-like cartilage, leading to improved function. The intermediate and long-term functional and clinical results are promising.

We provide a review of autologous chondrocyte implantation and describe our experience with the technique at our institution with a mean follow-up of 32 months (1 to 9 years).

The procedure is shown to offer statistically significant improvement with advantages over other methods of management of chondral defects.

We report a case which highlights the progression of osteofibrous dysplasia to adamantinoma and questions whether intralesional curettage is the appropriate treatment. The role of a joint-sparing massive endoprosthesis using cortical fixation is demonstrated and we describe a unique biomedical design which resulted in the manufacture of an end cap to allow amputation through a custom-made proximal tibial replacement, rather than an above-knee amputation following recurrence.

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.

Aims: he purpose of this study was to determine whether autologous chondrocyte implantation (ACI) in patients with articular cartilage defects of the knee resulted in patients returning to pre-injury levels of work and physical activities.

Methods: 133 consecutive patients from January 2001 to December 2002 underwent ACI at our institution. A telephone and postal questionnaire was conducted to ascertain a detailed occupational and leisure activity profile in this cohort of patients. For each job held for at least 2 months, we asked whether an average working day had involved any of the ten specified physical activities. Similarly, for each sport that had been played more than 5 times a year, we asked the age the sporting activities had began and whether they were able to return to these sports after surgery. Occupation for each patient was given a 3 digit code according to the Standard Occupational Classification System 2000 and hence determined whether the work performed was manual or non-manual.

Results: 97 patients responded to the questionnaire. There were 53 females and 44 males and the mean age at the time of operation was 34.5 (range 14 to 49). Category 6 (Personal Services Occupations) was the most common occupation pre-operatively, whereas category 4 (Administrative and Secretarial Occupations) was the most common post-operatively. 7% of patients’ work involved kneeling or squatting and this figure rose to 12%, 4 years following surgery. 42% of patients had to make some form of modification to their work (usually less physical or more office based). 47% of patients were able to return to at least one of the sports they played pre-injury.

Conclusion: This is the first study to demonstrate that patients are able to return to work and resume sporting activity following autologous chondrocyte implantation.

Aims: The main purpose of this study was to evaluate hemiplevis replacements in patients with periacetabular tumours in terms of the functional results and implant survivorship of the prosthesis. The second aim was to evaluate the complications and how they might be prevented in the future.

Methods: Case notes, hospital databases and radiographs were reviewed in 51 patients treated in a 16 year period with a custom made hemipelvis replacement (Stanmore Implants Worldwide). There were 13 deaths and 4 were lost to follow up. Of the remaining 34 patients there were 18 males and 16 females with a mean age of 48.6 (range 14 – 71).

Results: At a mean follow-up of 78 months (range 7 – 204), 70% of patients were still alive and the most common diagnosis was chondrosarcoma (17 cases). There were 2 cases of prosthesis failure and 22 of the 34 patients had one or more complications (12 cases of infection (8 deep and 4 superficial), 4 nerve palsies, 2 dislocations and 7 others). 13 patients had to have a second operation. The mean MSTS score was 63.8% and the TESS score was 59.8%

Conclusion: Infection was the most common complication and was the most significant reason for reoperation. This method of reconstruction is associated with a high morbidity rate and should be performed only at specialist centres, but the functional and oncological outcomes are satisfactory.

Background: Advances in adjuvant and neoadjuvant therapies have rendered many tumours that previously necessitated amputation amenable to limb salvage procedures. However, a significant proportion of tumours are still treated by hindquarter amputation in an attempt to cure the patient, or to reduce the tumour load. This tends to be lengthy, mutilating and is associated with high morbidity and poor survivorship.

Aims: To review the survivorship, quality of life and functional assessment following hindquarter amputations performed in this centre in the last 10 years.

Methods: This was a retrospective study of 51 consecutive patients who had hindquarter amputations for tumours between 1996 to 2006. Available patients were evaluated using contemporary functional outcome assessments (Musculoskeletal Society Tumour Score, Toronto Extremity Salvage Score, SF36).

Results: Fifty-one patients (31 males, 20 females) had palliative(8) or curative hindquarter amputations(43) for Chondrosarcoma(18), Malignant Fibrous Histiocytoma(6), Osteosarcoma(4) and other sarcoma sub-types(23). The mean age was 50.7 years (range 24–78). The mean duration of symptoms until referral was 5.2 months, the mean time from referral to tissue diagnosis was 16.2 days (range 2–80) and the time from confirmed histological diagnosis to surgery was 39.2 days (range 2–190) on average. Significant complications included phantom limb pain(15), wound problems(24), urinary problems(6), cardiopulmonary events(5) and erectile dysfunction(3). 33 of the 51 patients have passed away, with a mean survival postoperatively of 10.7 months (range 2–43), with carcinomatosis the main cause of death. The mean cumulative survival following hindquarter amputation in this hospital is 17.3 months.

Conclusions: Patients with no metastasis and clear margins at amputation had a better cumulative survival rate. Therefore the decision to proceed for hindquarter amputation to achieve a curative resection is justified but has to be weighed up against the associated significant complications, morbidity and functional deterioration.

Dedifferentiated chordoma is a rare and aggressive variant of the conventional tumour in which an area undergoes transformation to a high-grade lesion, typically fibrous histiocytoma, fibrosarcoma, and rarely, osteosarcoma or rhabdomyosarcoma. The dedifferentiated component dictates overall survival, with smaller areas of dedifferentiation carrying a more favourable prognosis. Although it is more commonly diagnosed in recurrences and following radiotherapy, there have been a few reports of spontaneous development. We describe four such cases, which were diagnosed de novo following primary excision, and discuss the associated clinical and radiological features.