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Abstract Background and aims Endoprosthetic replacements of the proximal femur are commonly required to treat destructive metastases with either impending or actual pathological fractures at this site. Modular prostheses provide an off the shelf availability and can be adapted to most reconstructive situations for proximal femoral replacements. The aim of this study was to assess the clinical and functional outcomes following modular tumour prosthesis reconstruction of the proximal femur in 100 consecutive patients with metastatic tumours and to compare them with the published results of patients with modular and custom made endoprosthetic replacements. Methods 100 consecutive patients who underwent modular tumour prosthetic reconstruction of the proximal femur for metastases using the METS system from 2001 to 2007 were studied. The patient, tumour and treatment factors in relation to overall survival, local control, implant survival and complications were analysed. Functional scores were obtained from surviving patients. Results and conclusion There were 45 male and 55 female patients. The mean age was 60.2 years. The indications were metastases. Seventy five patients presented with pathological fracture or with failed fixation and 25 patients were at a high risk of developing a fracture. The mean follow up was 15.9 months [range 0–77]. Three patients died within 2 weeks following surgery. 69 patients have died and 31 are alive. Of the 69 patients who were dead 68 did not need revision surgery indicating that the implant provided single definitive treatment which outlived the patient. There were three dislocations (2/5 with THR and 1/95 with unipolar femoral heads). 6 patients had deep infections. The estimated five year implant survival (Kaplan-Meier analysis) was 83.1% with revision as end point. The mean TESS score was 64% (54%–82%). We conclude that METS modular tumour prosthesis for proximal femur provides versatility; low implant related complications and acceptable function lasting the lifetime of the patients with metastatic tumours of the proximal femur.

Background and purpose The natural pattern of bone healing in large bony defects following curettage alone as treatment of benign bone tumors around the knee is not well reported. We analyzed the outcome in 146 patients. Patients and methods 146 patients with over 18 months of follow-up who underwent curettage without bone substitute filling or bone grafting for a benign tumor in the distal femur or upper tibia were included. The mean diameter of the defects following curettage was 5.7 (1.3-11) cm and the estimated average volume was 63 (1-240) cm3. The plain radiographs before and following curettage were reviewed to establish the size of the initial defect and the rate of reconstitution and infilling of the bone. The time to full weight bearing and any complications were recorded. Results There was a variable rate of infilling; some defects completely reconstituted to a normal appearance while some never filled in. In 88% of the cases, no further intervention after curettage was required and the mean time to full weight bearing was 6 weeks. The risk of subsequent fracture or the late development of osteoarthritis was strongly related to the size of the cyst at diagnosis, with cysts of > 60 cm3 (about 5 cm in diameter) having a much higher incidence of complications. Interpretation This study demonstrates the natural healing ability of bone without any adjuvant filling. It could be used as a baseline for future studies using any sort of filling with autograft, allograft, or bone substitute. [ABSTRACT FROM AUTHOR]

Purpose: Periosteal osteosarcomas are rare cartilage-rich bone tumours characterized by a juxtacortical eccentric position and are normally regarded oncologically as of intermediate to high grade. Their low incidence is mirrored by a small number of reported cases in the world literature. While there is general agreement that wide surgical excision is required, there is a paucity of evidence regarding adjuvant therapy. Previous reports have not indicated any consistent approach to this to allow appraisal. Patients and methods: We report 17 cases treated at our centre over 16 years. Our policy was to use chemotherapy when the tumour showed any features of high grade. Results: To date, no deaths have resulted from recurrence or metastasis of the tumour although there have been two deaths from other causes. Discussion: Comparison of survival with existing studies is made to draw conclusions regarding future treatment of this condition in terms of surgical and adjuvant approaches. [ABSTRACT FROM AUTHOR]

Purpose Limb salvage surgery of soft tissue sarcomas is associated with both a risk of local recurrence and wound complications. Although the lower limb appears to be at greater risk of wound-related morbidity, few studies separate anatomical compartments. We believe that the adductor compartment of the thigh has a particularly high rate of complications and so performed a retrospective analysis of all soft tissue sarcomas arising in this region undergoing limb salvage. Patients Patients with intermediate and high grade adductor compartment tumours were identified from our database and the case notes were reviewed for patient, tumour, surgical and wound variables, identifying those with wound complications both before and after discharge. Results Of 49 patients who underwent limb salvage surgery, 22 (42.9%) developed complications. Twelve patients (24.5%) required further surgery prior to wound healing and 10 patients had delays in post-operative radiotherapy. There were significant differences in the rates of preceding surgery, open biopsy performed at other centres and previous radiotherapy to this region between the complicated and uncomplicated groups. Discussion The management of these difficult tumours carries a high rate of wound complications and requires careful planning prior to tissue biopsy. Open biopsies should be performed by the tumour surgeon to allow easy inclusion of this site in the definitive procedure. In previously irradiated or operated limbs, alternative strategies for wound management may need to be considered. [ABSTRACT FROM AUTHOR]

Focuses on the developments in surgery. Risk of amputation following endoprosthetic replacement; Types of reconstruction used in total scapular resection; Recommendation of extensile axillary exposure in the management of axillary tumors.

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Abstract 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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Abstract: The prognostic value of alkaline phosphatase (AP) measured before and after chemotherapy, but before surgery was established in a retrospective survey of patients. The patients were 18 years or older, with non-metastatic high-grade osteosarcoma. Pre-chemotherapy AP was available in 89 cases, post-chemotherapy AP in 86 patients, and both in 71 cases. AP was classified as Normal (<100% upper limit), High (100%⩽AP<200%) or Very High (AP⩾200%). Osteosarcoma subtype was predominantly conventional. No correlation was found between subtype and chemotherapy response, local recurrence or survival. Pre-chemotherapy AP was raised more in the osteoblastic subtype. Post-chemotherapy AP and normalisation were the same among different subtypes. AP was not correlated with local recurrence. Normal or High pre-chemotherapy AP correlated with better survival at 10 years (64% and 70%) than Very High pre-chemotherapy AP (37%, P =0.005). Post-chemotherapy AP correlated with survival (68%, 39% and 25% in the Normal, High and Very High group, P =0.0007) and response to chemotherapy (P =0.049). A pre-chemotherapy AP above twice Normal correlated with worse survival. If AP decreased after chemotherapy, but was still raised, survival was better, but still worse than if AP normalised. A raised post-chemotherapy AP predicts poor chemotherapy response. [Copyright &y& Elsevier]

Histological examination of locally recurrent soft tissue sarcomas usually reveals an appearance similar to that of the original tumour. Occasionally, however, recurrent sarcomas appear more or less malignant histologically than the initial lesion. The goals of this paper were to identify the frequency with which this phenomenon occurs, factors that predict for a change in grade and to determine if this change is associated with a different prognosis from other patients with local recurrence. From a large sarcoma database, 124 patients with local recurrence were identified. These patients were distributed into groups who had no change in histological grade, increased histological grade or decreased histological grade on local recurrence. Increased grade occurred approximately 20% of the time, whereas decreased grade occurred in 7% of cases. A histological diagnosis of myxofibrosarcoma was predictive of an increase in histological grade on local recurrence. An increase in histological grade with local recurrence was not associated with a poorer survival. [Copyright &y& Elsevier]

Background and Aims: Endoprosthetic replacements of the proximal femur are commonly required to treat destructive metastases with either impending or actual pathological fractures at this site. Modular prostheses provide an off the shelf availability and can be adapted to most reconstructive situations for proximal femoral replacements. The aim of this study was to assess the clinical and functional outcomes following modular tumour prosthesis reconstruction of the proximal femur in 100 consecutive patients with metastatic tumours and to compare them with the published results of patients with modular and custom made endoprosthetic replacements., Methods: 100 consecutive patients who underwent modular tumour prosthetic reconstruction of the proximal femur for metastases using the METS system from 2001 to 2007 were studied. The patient, tumour and treatment factors in relation to overall survival, local control, implant survival and complications were analysed. Functional scores were obtained from surviving patients., Results and Conclusion: There were 45 male and 55 female patients. The mean age was 60.2 years. The indications were metastases. Seventy five patients presented with pathological fracture or with failed fixation and 25 patients were at a high risk of developing a fracture. The mean follow up was 15.9 months [range 0-77]. Three patients died within 2 weeks following surgery. 69 patients have died and 31 are alive. Of the 69 patients who were dead 68 did not need revision surgery indicating that the implant provided single definitive treatment which outlived the patient. There were three dislocations (2/5 with THR and 1/95 with unipolar femoral heads). 6 patients had deep infections. The estimated five year implant survival (Kaplan-Meier analysis) was 83.1% with revision as end point. The mean TESS score was 64% (54%-82%). We conclude that METS modular tumour prosthesis for proximal femur provides versatility; low implant related complications and acceptable function lasting the lifetime of the patients with metastatic tumours of the proximal femur.

We have retrospectively analysed the experience of a musculoskeletal oncological unit in the management of adult head and neck soft tissue sarcomas from 1990 to 2005. Thirty-six patients were seen, of whom 24 were treated at this unit, the remainder only receiving advice. The median age of the patients was 46 years. Most of the sarcomas were deep and of high or intermediate grade with a median size of 5.5 cm. Eleven different histological subtypes were identified. Wide excision was possible only in 21% of the cases. 42% of the patients developed local recurrence and 42% developed metastatic disease usually in the lungs. Overall survival was 49% at 5 years. Tumour size was the most important prognostic factor. Adult head and neck soft tissue sarcomas have a high mortality rate with a high risk of local recurrence and metastatic disease. The rarity of the disease would suggest that centralisation of care could lead to increased expertise and better outcomes.

Ewing's tumors are rare pediatric neoplasms that are characterized by specific chromosomal translocations and gene rearrangements. All of the fusion genes reported to date in Ewing's tumors juxtapose the EWS gene at 22q12 to an ETS-related gene, the most common of which are FLI1 at 11q24 and ERG at 21q22. We present here four cases of Ewing's tumor, which showed no evidence of a EWS gene rearrangement, but instead contained translocations involving 16p11 and 21q22. A rearrangement involving the same chromosome bands, t(16;21)(p11;q22), is found in rare cases of acute myeloid leukemia and fuses the FUS gene at 16p11 to the ERG gene at 21q22. In two of our Ewing's tumor cases, we were able to show at the sequence level that the translocation between chromosomes 16 and 21 similarly results in a FUS/ERG fusion. In one case, exons 1-5 and most of exon 6 of FUS were fused in-frame to exon 9 of ERG; in the other case, FUS exons 1-7 were fused in-frame to ERG exons 8-9. The functional fusion transcript is expected to be expressed from the der(21)t(16;21) derivative. In the two other t(16;21)-positive Ewing's cases, we performed bacterial artificial chromosome fluorescence in situ hybridization analysis on metaphases and interphase nuclei to demonstrate colocalization of bacterial artificial chromosomes containing FUS and ERG genes, also highly suggestive of fusion gene formation. These represent the first four cases where FUS, rather than EWS, is rearranged with an ETS-family transcription factor in Ewing's tumors. Our data provide additional evidence that the transactivation domains of the TET family of RNA-binding proteins (such as EWS and FUS) are interchangeable, and suggests a novel mechanism of oncogenesis in Ewing's tumors.

Between 1983 and 1996, nine children were treated with extensible endoprosthetic replacements of the proximal femur after resection of primary bone tumors. Four patients died of their disease. The remaining five patients were observed for an average follow-up period of 7.6 years. In these five patients, the authors performed an average of 10.2 operations per patient, including five lengthenings and a mean total extension of 69.7 mm per patient. Acetabular loosening and hip dislocations were the most frequent complications. Only two patients have not had a revision or a major complication. Despite this, four children are alive with a functioning lower limb and a mean Musculoskeletal Tumor Society functional score of 77.6%. Extensible endoprosthesis of the proximal femur is a viable alternative to hip disarticulation and in selected children offers an opportunity for near-normal development of the lower limb by allowing equalization of limb length and the ability to walk without the use of mobility aids.

Thirty-four patients with infected massive endoprostheses had two-stage revision procedures to cure the infection and retain a useful functioning limb. An antibiotic impregnated cement spacer constructed with two cement gun liners and a Kuntscher nail provided temporary stability and also allowed a high dose of local antibiotic concentration. In three patients the infection never was controlled, and one of these patients had a successful second revision surgery. Six other patients had reinfection varying from 14 to 92 months after surgery. Risk factors for reinfection were previous radiotherapy and any additional operative intervention. The overall success rate for controlling infection was 91% at 1 year and 74% at 5 years. Six patients required amputation. The mean functional outcome for the retained limbs using the Musculoskeletal Tumor Society scoring system was 77%. Two-stage revisions seem almost as effective at controlling infection in massive endoprostheses as in conventional joint replacements, particularly if additional surgical interventions can be avoided.