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Limb salvage is now customary in the treatment of primary bone tumours. The proximal tibia is a frequent site for these neoplasms but reconstruction, especially in children, is a formidable challenge. We reviewed 20 children with extendible replacements of the proximal tibia, all with a minimum follow-up of five years. Five died from their disease and, of the remaining 15, four had above-knee amputations for complications. Infection occurred in seven patients; in five it was related to the lengthening procedure. Aseptic loosening is inevitable in the younger children and only two have avoided a revision, amputation or other major complication; both were aged 12 years at the time of the initial surgery. Despite this, 11 children are alive with a functioning leg and a mean Musculoskeletal Tumour Society functional score of 83%. The lengthening mechanisms used in our series required extensive open operations. We are now using a simpler, minimally invasive, technique which we hope will decrease the incidence of complications. At present, the use of extendible prostheses of the proximal tibia remains an experimental procedure.

We carried out excision of a solitary bony metastasis from renal-cell carcinoma in 25 patients in the hope that this would produce a prolonged disease-free interval. Two patients had excisions only, five had amputations and 18 had excision and endoprosthetic replacement. The one-, three- and five-year cumulative survival rates were 88%, 54% and 13%, respectively. There were three complications. One patient developed a local recurrence and three had problems related to the endoprosthesis. We recommend radical excision of a solitary bony metastasis from renal-cell carcinoma to achieve local control of the tumour for the remainder of the patient's life.

Method. We performed a two-stage vascularized ipsilateral fibular graft transfer for segmental tibial defect following excision of malignant bone tumours.Results. We report 10 patients who had this procedure with an average follow-up of 116 months.The graft was transposed medialy on its vascular pedicle by two-stage surgery. Full weight bearing was achieved in six patients at 8-43 months postoperatively, but every patient had a significant complication.Discussion. The use of this method in isolation is not recommended for reconstruction of the tibia following tumour excision.

Purpose. Transmission of malignant tumour cells to a bone graft donor site is a rare complication of bone grafting.We report a case of seeding of malignant fibrous histiocytoma from the femur to a pelvic bone graft donor site.Discussion. We review the literature, discuss the possible mechanism of tumour transfer and offer advice aimed at avoiding this complication.

Dedifferentiated chondrosarcoma is a rare, highly malignant variant of chondrosarcoma in which a high-grade spindle-cell sarcoma coexists with a lower-grade chondroid tumour. We have reviewed our experience with this neoplasm in 22 patients, all of whom were treated using modern oncological principles of planned resection and chemotherapy. Despite this the median survival was under nine months and only 18% were alive at five years. Those patients who received chemotherapy, and in whom wide margins of excision were achieved at operation, did best. It is essential to have an accurate preoperative diagnosis in order to plan treatment which may offer a better prospect of cure.

Over a 25-year period we have treated 36 patients with osteosarcoma of the pelvis. Of the tumours, 24 (67%) were primary osteosarcomas and 12 (33%) arose either after irradiation or in association with Paget's disease. Six patients had a hindquarter amputation and 12 were treated by a limb-salvage procedure with intrapelvic excision. The five-year survival rate of all the patients with pelvic osteosarcoma was 18%, while for 17 treated by chemotherapy and surgery it was 41%. The prognosis for patients presenting with metastases or with secondary osteosarcoma was appalling and none survived after 29 months. No patient over the age of 50 years when seen initially survived for a year. Youth and a good response to chemotherapy along with complete surgical excision offer the best chance of cure.

We have performed endoprosthetic replacement after resection of tumours of the proximal tibia on 151 patients over a period of 20 years. During this period limb-salvage surgery was achieved in 88% of patients with tumours of the proximal tibia. Both the implant and the operative technique have been gradually modified in order to reduce complications. An initial rate of infection of 36% has been reduced to 12% by the use of a flap of the medial gastrocnemius, to which the divided patellar tendon is attached. Loosening and breakage of the implant have been further causes of failure. We found that the probability of further surgical procedures being required was 70% at ten years and the risk of amputation, 25%. The development of a new rotating hinge endoprosthesis may lower the incidence of mechanical problems. Limb salvage for tumours of the proximal tibia is fraught with complications, but the good functional outcome in successful cases justifies its continued use.

We carried out extensible endoprosthetic replacement of the proximal or total humerus in 18 children aged between six and 12 years, after resection of primary bone tumours mainly for osteosarcoma and Ewing's sarcoma. In 11 patients we performed 44 lengthening procedures, with an average of two per child annually and a mean total extension of 29.9 mm per patient. We were able to achieve lengthening of the operated limb with few complications and a mean functional rating of 79.3% according to the Enneking system. Progressive lengthening of these prostheses does not adversely affect the overall function of the arm, and superior subluxation of the head of the prosthesis has not been a problem.

Biopsy tissues of 52 patients with Ewing's sarcoma of bone treated between 1983 and 1993 were examined immuno-histochemically to determine the significance of p53 protein in diagnosis and prognosis of Ewing's sarcoma. Mean age at diagnosis was 17 years (range 6-36) and minimum follow-up was 30 months. The tumours were located in the extremities and central bones in 35 and 17 patients respectively. Metastases were present in seven patients at diagnosis. Treatment consisted of chemotherapy, surgery and/or radiotherapy in all the patients. Overexpression of p53 protein was demonstrated in seven patients (14%). There was no relationship between expression of p53 and site of tumours. Patients who overexpressed p53 protein appeared to have more advanced diseases at diagnosis and poorer response to chemotherapy than those without p53 overexpression. The 5-year relapse-free survival and overall survival in patients without metastases at the time of diagnosis were 66% and 71%, respectively, in p53 protein-negative patients compared with 20% relapse-free and overall survival in those with p53 protein overexpression (P= 0.01). The poorer prognosis in p53 protein-positive patients was independent of site, local treatment or necrosis of the tumours (P < 0.05). Over-expression of p53 protein is an independent poor prognostic factor in Ewing's sarcoma of bone.

We studied the CT and MR scans, and the histology of 50 patients with primary Ewing's sarcoma of bone to determine the association between the change in tumour volume and necrosis after chemotherapy, and to ascertain their influence on prognosis. The mean age of the patients was 17 years. The limbs were involved in 40 and the axial bones in ten. The volume of the tumour at diagnosis varied from 31 to 1790 ml. There was a significant relationship between necrosis and the measured change in volume of the tumour after chemotherapy. Progression of the tumour despite chemotherapy was seen only in patients with necrosis of grades 4 to 6. Necrosis significantly influenced survival (p < 0.05), but the effect of change in volume was less significant. Change in volume of the tumour is a good predictor of necrosis induced by chemotherapy. Necrosis is a strong prognostic factor in Ewing's sarcoma.

Below knee amputation remains the treatment of choice for most patients with aggressive tumours of the distal tibia. We report the clinical and functional outcome of limb preserving surgery and endoprosthetic reconstruction of the distal tibia and ankle joint in five patients who declined amputation. The mean age was 32 years. Two had osteosarcoma, one Ewing's sarcoma, leiomyosarcoma and Giant cell tumour. Three patients developed significant complications including local recurrence, wound dehiscence and infection, and fibula impingement. Despite these complications the patients declined amputation even in the presence of significant discomfort. Early function was excellent in all patients but deteriorated with time. The patients still maintained an Enneking Score of more than 50%. Some patients are unwilling to undergo amputation for aggressive tumours of the distal tibia. For these, excision and reconstruction with endoprosthesis allow early functional recovery but there is significant medium term morbidity and functional deterioration.

Purpose. Clear cell chondrosarcoma is a rare variant of chondrosarcoma. Six cases are herein reported.Subjects. We have treated six patients with clear cell chondrosarcoma in the past 28 years, representing 1.6% of all chondrosarcomas seen in this time period.Results and Discussion. Half the patients had been initially underdiagnosed and inappropriately treated.Conclusions. Our results and our review of the literature highlight the fact that inadequate initial treatment leads to a high rate of both local recurrence and metastasis whilst wide initial excision is usually curative.

Fifty-four patients had endoprosthetic replacement of the proximal femur after resection of primary malignant or aggressive bone tumors. The mean age of the patients was 41 years. The average duration of followup was 9 years (range, 5-24 years). At the time of this review 35 patients are surviving. There were six revisions of the acetabulum and seven revisions of the femoral component. The survivorship of the prostheses without revision was 77% at 10 years falling to 57% at 20 years. There was a 13% risk of amputation and a 1.8% incidence of infection. The mean of the functional scores of the 35 living patients was 83% (range, 60%-97%). This study showed that successful long term results can be obtained with cemented massive endoprostheses despite the incidence of recognized complications including polyethylene wear and aseptic loosening.

Unlabelled: Purpose Paget' s osteosarcoma has a fearful reputation with a quoted survival of at best 5% at 5 years.We therefore reviewed our experience of 26 patients treated over the last 25 years using modern staging and limb salvage techniques to see if there had been any improvement in survival., Subjects: We identified 26 patients on the Royal Orthopaedic Hospital Oncological database with a diagnosis of sarcoma secondary to Paget's disease., Results: The survival rate was 53% at 1 year, 25% at 2 years and no patient survived for 5 years.The median survival was 21 months for those treated with curative intent and 7 months for those treated palliatively. Four of the five patients treated with limb-sparing surgery developed local recurrence between 5 and 12 months, the fifth died at 14 months.There was no difference in survival between amputation and limb salvage., Discussion: The development of sarcomatous change in Paget's disease is well recognised. It represents an important segment of primary bone tumours in patients over 40 years of age.The prognosis is appalling. Indeed only 15 of 368 cases (4%) from a number of historical series have survived more than 5 years. Our results are similarly disappointing with no survivors at 5 years despite modern methods of management of bone tumours.While there is no difference in local recurrence rates or survival between limb reconstruction and limb ablation the poor prognosis for both means that neither can be recommended at present. Sarcomatous change in Pagetoid bone should therefore be regarded as a different disease to primary osteosarcoma. It remains an incurable disease with a poor prognosis.

Purpose. The purpose of this study was to analyse the height at diagnosis and growth in 72 skeletally immature children who had been treated for osteosarcoma in the area of the knee.Subjects. Of the patients, the average age at diagnosis was 10 years in girls and 12 years in boys. All children received neo-adjuvant chemotherapy, and had limb salvage by endoprosthetic replacement.Results and conclusion. The results of this study indicate that there is no evidence that children with osteosarcoma are taller at diagnosis than their normal counterparts. However, there was a marked retardation in growth in the year following the administration of cytotoxic chemotherapy. There were 19 children who reached skeletal maturity. The final height in these children was not significantly different from the normal population.

Patient. We describe a case of chondroblastoma of the os calcis which metastasized to the tibia, soft tissues and lung. A complete response of the lung lesions was noted with chemotherapy.Discussion. Review of the published literature shows that metastatic chondroblastoma only arises following local recurrence of the tumour.

We report our results in 24 children with malignant primary bone tumours of the distal femur treated with a Stanmore extendible endoprosthesis (SEER). This consists of a femoral component that can be lengthened, a constrained knee and an uncemented sliding tibial component which crosses the proximal tibial physeal plate perpendicularly. The average age of the patients at diagnosis was ten years and the mean follow-up was 4.7 years (2.5 to 7.9). The mean growth of the affected tibia was 76% (18 to 136) and of the fibula 83% (15 to 750) of the growth of the unaffected limb. Measurement of growth arrest lines showed that the mean growth of the proximal tibial physis on the affected side was 69% (43 to 100) of that of the normal side. The great variability in the growth of the physis cannot yet be explained.

We reviewed 20 patients after forequarter amputation performed for high-grade malignant tumours of the shoulder girdle (Enneking grades IIB to III). The operations were classified as palliative or curative according to the resection margins and the presence of disseminated disease at the time of the surgery. There were five palliative and 15 curative procedures. Two patients died from unrelated causes, septicaemia and suicide. Eight died in the first two years, four of whom had had a palliative operation. Four died between two and five years after surgery, one after a palliative operation. Five patients are alive, at a mean of 89.4 months after surgery, four of whom are free from disease. The median survival after a palliative amputation was 20.6 months. Our overall five-year survival (palliative and curative cases) was 21.2%, for curative cases it was 30.2%. None of the patients use an artificial prosthesis. Despite the disfigurement which results from this operation, it still has a useful role to play in the management of high-grade malignant tumours of the upper limb.

Purpose. The aim of this paper was to assess the growth in the unaffected lower limb of children who had received chemotherapy for a malignant primary bone tumour around the knee.Subjects/methods. Following diagnosis, all children (45, of which 32 were boys and 13 were girls) were staged. If limb-salvage surgery was thought appropriate, measured radiographs of both legs was performed, the bone age was estimated and the expected growth in the femur and tibia was calculated according to Tupman. These procedures were repeated at follow-up and the data plotted. Regression and correlation coefficients were also calculated.Results. The observed regression line in boys was almost identical to Tupman's curve. However, the observed growth in girls was larger than the expected growth.Discussion. It is recommended that the regression lines presented here are used in the calculation of the expected growth in the lower limb of children who have received chemotherapy for a malignant primary bone tumour, especially in girls.

Twenty years of experience with the use of growing endoprostheses for limb salvage in skeletally immature patients is reported. Alternatives for the management of primary malignant bone tumors in this group of patients are amputation, rotationplasty, and allograft or autograft reconstruction. The development of the currently used implant is charted, with commentary on the complications and difficulties which gave rise to design changes. Over this period, 123 expanding endoprostheses have been inserted in 108 patients in this institution, and the results of a series of 54 consecutive distal femoral replacements are reported. The mean functional score was 72%, and the local recurrence rate was 11%. The technique was successful in maintaining limb-length equality. We conclude that in specialized centers of orthopedic oncology that utilize an endoprosthesis with proven reliability of the expansion mechanism, the short- and long-term results of this technique justify its continued use.

We reviewed the surgical treatment and oncological results of 40 patients with pathological fractures from localised osteosarcoma of the long bones to determine the outcome of limb salvage in their management. All had had adjuvant chemotherapy. There were 26 males and 14 females with a median age at diagnosis of 18 years (2 to 46) and a median follow-up of 55 months (8 to 175). We performed limb salvage in 27 patients and amputation in 13. The margins of resection were radical in five patients, wide in 26, marginal in six, wide but contaminated in two and intralesional in one. Local recurrence developed in 19% of those treated by limb salvage and in none of those who had an amputation. The cumulative five-year survival of all the patients was 57% and in those treated by limb salvage or amputation it was 64% and 47%, respectively (p > 0.05). Limb-sparing surgery with adequate margins of excision can be achieved in many patients with pathological fractures from primary osteosarcoma without compromising survival, but the risk of local recurrence is significant.

A case of donor-site fracture following a radial forearm flap was treated conservatively with plaster but failed to unite. Successful union was achieved with bone grafting, external fixation, and electrical stimulation. It is suggested that in similar cases of delayed union, or where a large amount of bone has been resected from the radius and there is a significant risk of fracture or where fracture has occurred intraoperatively, the possibility of primary bone grafting should be considered.

22 patients who sustained high energy wrist injuries are reported. These complex injuries resulted in articular disruption of the distal radius. Associated injuries included scapho-lunate dissociation (18%), central die-punch injuries (14%), ulnar fractures (41%) and diastasis at the distal radioulnar joint (23%). Nine fractures (41%) were open and almost a third of patients had other skeletal injuries. All patients were treated by external fixation and reviewed after a mean follow-up of 2 1/2 years. There were no excellent results and only ten good ones (45%). The mean functional impairment was 32%. The external fixator was effective in maintaining extra-articular alignment, but not in ensuring accurate reduction of the articular surface. Residual incongruity of the joint surface was an adverse prognostic feature. All five patients (22%) with an articular step of more than 2 mm. developed symptomatic arthritis. Failure to restore the joint line did not account for all the unsatisfactory results; persistent scapho-lunate dissociation and problems at the distal radioulnar joint were also important.

The Mitchell's osteotomy is an established and successful operation for hallux valgus. We report a previously undescribed procedure comprising a step or 'Z' osteotomy that is a simplified version of the Mitchell's osteotomy which involves less extensive soft tissue dissection and avoids opening the first metatarsophalangeal (MTP) joint. In total, 51 operations in 40 patients were reviewed with a mean follow-up of 4.7 years. The results were compared with those reported by Mitchell in his paper of 1958, using the same criteria. The mean age was 24 years and 82% of patients were women. In 88% of patients the results were rated excellent or good and there were no serious complications. These results suggest that this procedure is as successful as the Mitchell osteotomy in young patients, and it may have long-term benefits.