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Objective: Pre-operative whole-bone MRI is required to assess intra-osseous tumour extent and to identify skip metastases in cases of bone sarcoma. The current study aims to determine the sensitivity, specificity, and diagnostic accuracy of whole-bone MRI for the identification of skip metastases., Materials and Method: Review of 162 patients with long bone osteosarcoma or Ewing sarcoma who had undergone whole-bone MRI to assess intra-osseous tumour length and identify skip metastases. Comparison was made with post-chemotherapy MRI to look for a change in the appearance of suspected skip metastases, and resection specimens were assessed for the presence of skip metastases. The presence of local osseous recurrence was determined at final follow-up., Results: There were 112 males and 50 females (mean age 18.8 years), with 119 osteosarcomas and 43 Ewing sarcomas. Skip metastases were diagnosed on whole-bone MRI in 23 cases (14.2%). In 2 cases, pre-operative needle biopsy diagnosed enchondromata, resulting in false positive diagnoses. Skip metastases were diagnosed in the resection specimens in 3 cases, and based on comparison with post-chemotherapy MRI in 12. There was no evidence of local osseous recurrence in 160 patients, while late recurrence occurred in 2 patients. Sensitivity was calculated as 88.2%, specificity as 97.6%, and diagnostic accuracy as 96.7%., Conclusion: Whole-bone MRI has a high sensitivity, specificity, and diagnostic accuracy for the identification of skip metastases in osteosarcoma and Ewing sarcoma. The possibility of false positive skip lesions and late local osseous recurrence is also highlighted.

Aims: The aim of this study is to assess outcomes of patients ≤12 years who undergo Stanmore noninvasive extendible endoprosthetic replacement of the distal femur (DF NIEPR)., Patients and Methods: A total of 101 children (mean age 9.6 years) were included. All complications which required further surgery were recorded. Clinical and functional outcomes were evaluated with Musculoskeletal Tumor Society (MSTS) scores at a mean follow-up of 64 months (range 6-174)., Results: Thirty-one (30.7%) patients died at a mean of 33 months. Forty had prosthesis removed after a mean of 43 months (range, 7-103). Attaining of the full lengthening potential before skeletal maturity was the most frequent reason for revision surgery, particularly in those with smaller lengthening potential (P  =  0.039). Implant survival rate for other causes was 61.7% at 5 years and 45.0% at 10 years. At final follow-up mean MSTS score was 26 (range, 13-29). Twenty-two (21.5%) patients had a final limb-length discrepancy (LLD)  > 2 cm., Conclusions: DF NIEPR produces a good functional outcome, with the prevention of major LLD at skeletal maturity in the majority of the cases. We suggest patient selection criteria to account for the stage of the disease due to the high cost of the NIEPR, and high percentage requiring revision, and a 60% mortality rate in those patients presenting with distant disease burden., (© 2019 Wiley Periodicals, Inc.)

Purpose: In this study, we asked the question of whether non-invasive (NI) extendible bone tumour implants are as reliable and reduce infection when compared with patients who received a minimally invasive (MI) extendible implant., Methods: Forty-two NI extendible bone tumour implants were investigated at a mean follow-up of 22 months (range, 1-87 months) and 63 MI implants at a mean follow-up of 49 months (range, 1-156 months)., Results: Kaplan-Meier analysis showed that the probability of MI implant survival was 58.8% compared with 78.6% in NI patients. No significant difference between these two patient groups was found. Infection was the main reason for failure in the MI implant group where nine (35%) implants were revised. However, only one (11%) NI implant was revised for infection ( p = 0.042). None of the NI implants failed due to aseptic loosening; however, six (23%) MI implants were revised for aseptic loosening of the intramedullary stem. Four (15%) of the failed MI implants were revised due to full extension and five (56%) of failed NI implants were replaced as the implant had been fully extended where the patient still required growth., Conclusion: Where possible, an NI massive prosthesis should be used in this patient group. Our results suggest that MI prostheses should be infrequently used due to the high incidence of infection. Lengthening of NI prostheses is painless, can be carried out in the clinic and is more cost-effective. However, further work is required to increase the amount of growth potential available in these implants.

Background: Aseptic loosening of massive bone tumor implants is a major cause of prosthesis failure. Evidence suggests that an osteointegrated hydroxyapatite (HA)-coated collar would reduce the incidence of aseptic loosening around the cemented intramedullary stem in distal femoral bone tumor prostheses. Because these implants often are used in young patients with a tumor, such treatment might extend the longevity of tumor implants. Questions/purposes We asked whether (1) HA-coated collars were more likely to osteointegrate; (2) HA collars were associated with fewer progressive radiolucent lines around the stem-cement interface; and (3) HA-coated collars were associated with less bone loss at the bone-shoulder implant junction?, Methods: Twenty-two patients were pair-matched to one of two groups--either (1) implants with a HA-coated ingrowth collar (HA Collar Group); or (2) implants without an ingrowth collar (Noncollar Group). Age, sex, and length of followup were similar in both groups. HA-coated collars were developed and used at our institution from 1992 to address the high failure rate attributable to aseptic loosening in patients with massive bone tumor implants. Before this, smooth titanium shafts were used routinely adjacent to bone at the transection site. The minimum followup was 2 years (mean, 7 years; range, 2-12 years). Radiographs obtained throughout the followup period were analyzed and osteointegration at the shaft of the implant quantified. Radiolucent line progression around the cemented stem was semi-quantitatively assessed and cortical bone loss at the bone-shoulder implant junction was measured during the followup period., Results: Comparison of the most recent radiographs showed nine of 11 patients had osteointegrated HA collars, whereas only one patient in the Noncollar Group had osteointegration (p > 0.001). The radiolucent line score quantified around the cemented stem was lower in the HA Collar Group when compared with the Noncollar Group (p = 0.001). Results showed an increase in cortical bone loss at the bone-shoulder implant junction in the Noncollar Group when compared with the HA Collar Group (p < 0.001)., Conclusions: Osteointegration at the implant collar resulted in fewer radiolucent lines adjacent to the intramedullary cemented stem and decreased cortical bone loss immediately adjacent to the transection site. These results suggest that the HA collar may help reduce the risk of aseptic loosening in patients with this type of implant, but longer followup and a larger prospective comparison series are necessary to prove this more definitively.

In patients with a tumour affecting the distal ulna it is difficult to preserve the function of the wrist following extensive local resection. We report the outcome of 12 patients (nine female, three male) who underwent excision of the distal ulna without local soft-tissue reconstruction. In six patients, an aggressive benign tumour was present and six had a malignant tumour. At a mean follow-up of 64 months (15 to 132) the mean Musculoskeletal Tumour score was 64% (40% to 93%) and the mean DASH score was 35 (10 to 80). The radiological appearances were satisfactory in most patients. Local recurrence occurred in one patient with benign disease and two with malignant disease. The functional outcome was thus satisfactory at a mean follow-up in excess of five years, with a relatively low rate of complications. The authors conclude that complex reconstructive soft-tissue procedures may not be needed in these patients., (©2014 The British Editorial Society of Bone & Joint Surgery.)

Background: Several different strategies have been reported for the treatment of chondromyxoid fibromas, all with variable outcomes and high recurrence rates., Methods: We report on 22 consecutive cases of chondromyxoid fibromas treated by intralesional curettage, four of which had adjuvant cementation at our institution between 2003 and 2010. We assessed the functional outcome using the Musculoskeletal Tumour Society (MSTS) scoring system., Results: Nine males and 16 females with a mean age of 36.5 years (range 11 to 73) and a mean follow-up of 60.7 months were included in the study. Local recurrence occurred in two patients (9%) within the first 2 years following the index procedure. This was treated by re-curettage only of the residual defect. Two postoperative complications occurred: a superficial wound infection in one patient and a transient deep peroneal nerve neurapraxia in the other. The mean postoperative MSTS score was 96.7%., Conclusions: Intralesional curettage and cementation is as an effective treatment strategy for chondromyxoid fibromas, providing satisfactory functional results with a low recurrence rate. Careful case selection with stringent clinical and radiographic follow-up is recommended.

The pre-operative differentiation between enchondroma, low-grade chondrosarcoma and high-grade chondrosarcoma remains a diagnostic challenge. We reviewed the accuracy and safety of the radiological grading of cartilaginous tumours through the assessment of, first, pre-operative radiological and post-operative histological agreement, and second the rate of recurrence in lesions confirmed as high-grade on histology. We performed a retrospective review of major long bone cartilaginous tumours managed by curettage as low grade between 2001 and 2012. A total of 53 patients with a mean age of 47.6 years (8 to 71) were included. There were 23 men and 30 women. The tumours involved the femur (n = 20), humerus (n = 18), tibia (n = 9), fibula (n = 3), radius (n = 2) and ulna (n = 1). Pre-operative diagnoses resulted from multidisciplinary consensus following radiological review alone for 35 tumours, or with the addition of pre-operative image guided needle biopsy for 18. The histologically confirmed diagnosis was enchondroma for two (3.7%), low-grade chondrosarcoma for 49 (92.6%) and high-grade chondrosarcoma for two (3.7%). Three patients with a low-grade tumour developed a local recurrence at a mean of 15 months (12 to 17) post-operatively. A single high-grade recurrence (grade II) was treated with tibial diaphyseal replacement. The overall recurrence rate was 7.5% at a mean follow-up of 4.7 years (1.2 to 12.3). Cartilaginous tumours identified as low-grade on pre-operative imaging with or without additional image-guided needle biopsy can safely be managed as low-grade without pre-operative histological diagnosis. A few tumours may demonstrate high-grade features histologically, but the rates of recurrence are not affected., (©2014 The British Editorial Society of Bone & Joint Surgery.)

Background: The objective of this study was to examine the degree of osteointegration into a hydroxyapatite-coated collar and relate this finding to aseptic loosening in patients with a distal femoral replacement used to treat primary bone cancer. Our hypothesis was that the implant collar would increase osteointegration and reduce the rate of aseptic implant loosening., Methods: Sixty-one patients treated with a primary cemented distal femoral prosthesis between 1992 and 2001 were included in this study. The mean duration of follow-up was 8.5 years (range, two to eighteen years). Extracortical bone growth into the grooved hydroxyapatite-coated collar was quantified radiographically. Histological sections through four hydroxyapatite-coated collars and four implants with no collar, retrieved following amputation due to local recurrence or at autopsy at a mean of 3.5 years (range, 1.4 to 6.1 years) after implantation, were evaluated as well., Results: Five (8%) of the implants were revised because of aseptic loosening, 3% of the implants fractured, and 3% were revised because of infection. Six limbs (10%) required amputation because of local tumor recurrence. On radiographs, osteointegration into the collar was seen to have occurred in 70% of the patients and did not correlate with sex, age, diagnosis, or length of time postoperatively. Histological analysis showed mature lamellar bone within the grooves of the hydroxyapatite-coated collar, and bone was observed in direct contact with the hydroxyapatite coating. Extracortical bone failed to make direct contact with the surface of the implants manufactured without a collar., Conclusions: The use of cemented distal femoral massive bone tumor prostheses with a hydroxyapatite-coated collar located at the shoulder of the implant was followed by a low (8%) rate of revision due to aseptic loosening. The use of hydroxyapatite grooved collars may lead to osteointegration of the implant shoulder (collar) and may reduce the rate of aseptic loosening.

Local recurrence along the biopsy track is a known complication of percutaneous needle biopsy of malignant musculoskeletal tumours. In order to completely excise the track with the tumour its identification is essential, but this becomes increasingly difficult over time. In an initial prospective study, 22 of 45 patients (48.8%) identified over a three-month period, treated by resection of a musculoskeletal tumour, had an unidentifiable biopsy site at operation, with identification statistically more difficult after 50 days. We therefore introduced the practice of marking the biopsy site with India ink. In all 55 patients undergoing this procedure, the biopsy track was identified pre-operatively (100%); this difference was statistically significant. We recommend this technique as a safe, easy and accurate means of ensuring adequate excision of the biopsy track.

Purpose: The optimal reconstructive method after resection of malignant bone tumours of the proximal ulna is unknown.We report the outcome of endoprosthetic replacement in a young patient population., Methods: This was a retrospective review of four patients[three males and one female; mean age 17.5 (range 11–31)years] who underwent limb salvage with a proximal ulnar endoprosthetic replacement following excision of malignant bone tumour. Mean follow-up was 85 (range 14–194) months., Results: All patients were alive at final follow-up and reported an improvement in pain. One patient required transhumeral amputation for intralesional excision complicating a local recurrence at one month. Two patients developed fixed flexion deformities of the elbow, one of whom required radial-head excision. Mean Musculoskeletal Tumour Society (MSTS)score and Toronto Extremity Salvage Score (TESS) were 27(range 25–28) and 81 (73–88), respectively., Conclusions: Custom-made proximal ulna endoprosthetic replacement following resection of malignant bone tumours in young patients provides a stable reconstruction option with satisfactory function and without apparent compromise in patient survival.

In skeletally immature patients, resection of bone tumours and reconstruction of the lower limb often results in leg-length discrepancy. The Stanmore non-invasive extendible endoprosthesis, which uses electromagnetic induction, allows post-operative lengthening without anaesthesia. Between 2002 and 2009, 55 children with a mean age of 11.4 years (5 to 16) underwent reconstruction with this prosthesis; ten patients (18.2%) died of disseminated disease and one child underwent amputation due to infection. We reviewed 44 patients after a mean follow-up of 41.2 months (22 to 104). The mean Musculoskeletal Tumor Society score was 24.7 (8 to 30) and the Toronto Extremity Salvage score was 92.3% (55.2% to 99.0%). There was no local recurrence of tumour. Complications developed in 16 patients (29.1%) and ten (18.2%) underwent revision. The mean length gained per patient was 38.6 mm (3.5 to 161.5), requiring a mean of 11.3 extensions (1 to 40), and ten component exchanges were performed in nine patients (16.4%) after attaining the maximum lengthening capacity of the implant. There were 11 patients (20%) who were skeletally mature at follow-up, ten of whom had equal leg lengths and nine had a full range of movement of the hip and knee. This is the largest reported series using non-invasive extendible endoprostheses after excision of primary bone tumours in skeletally immature patients. The technique produces a good functional outcome, with prevention of limb-length discrepancy at skeletal maturity.

We describe 22 cases of bizarre parosteal osteochondromatous proliferation, or Nora's lesion. These are surface-based osteocartilaginous lesions typically affecting the hands and feet. All patients were identified from the records of a regional bone tumour unit and were treated between 1985 and 2009. Nine lesions involved the metacarpals, seven the metatarsals, one originated from a sesamoid bone of the foot and five from long bones (radius, ulna, tibia, and femur in two). The mean age of the patients was 31.8 years (6 to 66), with 14 men and eight women. Diagnosis was based on the radiological and histological features. The initial surgical treatment was excision in 21 cases and amputation of a toe in one. The mean follow-up was for 32 months (12 to 162). Recurrence occurred in six patients (27.3%), with a mean time to recurrence of 49 months (10 to 120). Two of the eight patients with complete resection margins developed a recurrence (25.0%), compared with four of 14 with a marginal or incomplete resection (28.6%). Given the potential surgical morbidity inherent in resection, our data suggest that there may be a role for a relatively tissue-conserving approach to the excision of these lesions.

The best method of reconstruction after resection of malignant tumours of the tibial diaphysis is unknown. In the absence of any long-term studies analysing the results of intercalary endoprosthetic replacement, we present a retrospective review of 18 patients who underwent limb salvage using a tibial diaphyseal endoprosthetic replacement following excision of a malignant bone tumour. There were ten men and eight women with a mean age of 42.5 years (16 to 76). Mean follow-up was 58.5 months (20 to 141) for all patients and 69.3 months (20 to 141) for the 12 patients still alive. Cumulative patient survival was 59% (95% confidence interval (CI) 32 to 84) at five years. Implant survival was 63% (95% CI 35 to 90) at ten years. Four patients required revision to a proximal tibial replacement at a mean follow-up of 29 months (10 to 54). Complications included metastases in five patients, aseptic loosening in four, peri-prosthetic fracture in two, infection in one and local recurrence in one. The mean Musculoskeletal Tumor Society score and the mean Toronto Extremity Salvage Score were 23 (17 to 28) and 74% (53 to 91), respectively. Although rates of complication and revision were high, custom-made tibial diaphyseal replacement following resection of malignant bone tumours enables early return to function and provides an attractive alternative to other surgical options, without apparent compromise of patient survival.

The optimal reconstructive method following segmental resection of malignant tumours in the humeral diaphysis is unknown as there are no prospective long-term studies comparing biologic with endoprosthetic reconstruction. This is a retrospective review of 13 patients who, between 1995 and 2010, had undergone limb salvage at our institution using a custom-made humeral diaphyseal endoprosthetic replacement following excision of malignant bone disease. There were 9 males and 4 females with a mean age of 35 years at the time of surgery (range: 10 to 78). Mean follow-up was 56.8 months (range: 5 to 148). Cumulative patient survival was 75% at 10 years. Implant survival, with removal of the endoprosthesis or part of it for any reason as an end point, was 47% at 10 years. Seven patients required revision (54%). Complications included metastases in four, aseptic loosening in four, peri-prosthetic fracture in two and local recurrence in two. Mean MSTS and TESS scores were 23 (18 to 27) and 67% (52-80) respectively. Custom-made humeral diaphyseal replacement following resection of malignant bone tumours provided functional results superior to amputation, without an obvious compromise in patient survival. There was a relatively high revision rate for aseptic loosening and peri-prosthetic fracture and patients should be counselled about this preoperatively.

Between 1997 and 2007, 68 consecutive patients underwent replacement of the proximal humerus for tumour using a fixed-fulcrum massive endoprosthesis. Their mean age was 46 years (7 to 87). Ten patients were lost to follow-up and 16 patients died. The 42 surviving patients were assessed using the Musculoskeletal Tumor Society (MSTS) Score and the Toronto Extremity Salvage Score (TESS) at a mean follow-up of five years and 11 months (one year to ten years and nine months). The mean MSTS score was 72.3% (53.3% to 100%) and the mean TESS was 77.2% (58.6% to 100%). Four of 42 patients received a new constrained humeral liner to reduce the risk of dislocation. This subgroup had a mean MSTS score of 77.7% and a mean TESS of 80.0%. The dislocation rate for the original prosthesis was 25.9; none of the patients with the new liner had a dislocation at a mean of 14.5 months (12 to 18). Endoprosthetic replacement for tumours of the proximal humerus using this prosthesis is a reliable operation yielding good results without the documented problems of unconstrained prostheses. The performance of this prosthesis is expected to improve further with a new constrained humeral liner, which reduces the risk of dislocation.

Chondrosarcoma is most frequently present in the pelvis and long bones and rarely seen in the bones of the hand. Traditionally the treatment of choice for involvment of the hand is ray amputation, however this causes significant functional deficit if there is thumb involvement. There are limited cases in literature of resection of thumb chondrosarcoma with restoration of function. We present a case of high grade chondrosarcoma of the first metacarpal treated with resection and free fibular graft reconstruction. We report excellent functional outcome with this procedure.

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.

We report the first use of an endoprosthetic diaphyseal replacement after the excision of a midshaft radial tumor. We present a rare case of a solitary midshaft radial metastasis in a 72-year-old gentleman who was treated 8 years earlier for primary renal cell carcinoma by radical nephrectomy. Follow-up for this patient was 25 months after implant insertion and at the latest review was pain-free. Functional scores for the elbow (using the Mayo Elbow Performance Score) and the wrist (using the Mayo Wrist Score) were 80 and 60, respectively, both out of 100. Use of a midshaft radial endoprosthesis provides a good functional outcome and symptom relief after extensive tumor resection, with regard to functional outcome and symptom relief.

Background: Different treatment strategies for low-grade chondrosarcomas are reported in the literature with variable outcomes. The aim of this study was to assess the oncological and functional outcomes associated with intralesional curettage and cementation of the lesion as a treatment strategy., Patients and Methods: We performed a retrospective review of 39 consecutive patients with intramedullary low-grade chondrosarcoma of long bones treated by intralesional curettage and cementation at our institution between 1999 and 2005., Results: There were 10 males and 29 females with a mean age of 55.5 years (32-82), and a mean follow-up of 5.1 years (3-8.7). Local recurrence occurred in two patients (5%) within the first two years following index surgery. Both were treated by re-curettage and cementation of the resultant defects. A second local recurrence developed a year later in one of these two patients, for which a further curettage followed by local liquid nitrogen treatment was performed. Overall, there were no cases of post-operative complications or metastases. The patients were assessed using the Musculoskeletal Tumour Society scoring system (MSTS) to determine limb function. The average score achieved was 94% (79-100%)., Conclusion: Intralesional curettage is an effective treatment strategy for low-grade intramedullary chondrosarcoma of long bones, with excellent oncological and functional results. Careful case selection with stringent clinical and radiographic follow-up is recommended.

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.

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 degrees (100 degrees to 120 degrees). 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.

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.

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.

The recurrence of osteosarcoma is rare. Local recurrence occurs in 4-10% of patients following effective treatment. So far, recurrences as late as 15 years have been reported in the literature. We report a unique case of local recurrence of intramedullary osteosarcoma 17 years from initial diagnosis and treatment. Regular long-term follow-up of patients with this diagnosis is crucial to ensure early detection and treatment of potential recurrences.

Radiographs of 33 patients who had an expandable prosthesis inserted after tumor resection in the distal femur were evaluated. We hypothesized that, when a sleeve was present, there was greater stem migration and cortical reaction. The thickness of medial and lateral cortices of the tibia was measured at 6 months and on the last follow-up radiograph. The distance from the edge of the prosthesis to the cortical edge was also compared. When a sleeve was present, there was greater stem migration (P < .001) and a greater increase in lateral cortical thickness (P < .001). There was a higher revision rate when a sleeve was used, but this was not statistically significant. There was no difference in function according to the Toronto Extremity Salvage Score (TESS) between the 2 groups. This is the first study to demonstrate that the presence of a polyethylene sleeve is associated with a tendency for the tibial prosthesis to migrate laterally and cause a greater sclerotic reaction.

We report two rare cases of very late local recurrence of Ewing's sarcoma, occurring 16 years and 19 years after treatment of the primary tumour. In both cases, disease remission had been achieved. Both patients originally underwent non-surgical combined modality therapy following initial diagnosis and both were rendered disease-free. After a long latent phase, both started experiencing unexplained local symptoms. These were investigated at their local hospital but tumour recurrence was not initially considered as a cause of these symptoms. The two patients were eventually referred back to our institution after the diagnosis of recurrent Ewing's sarcoma was established. Both have successfully undergone complete excision of the recurrences and are receiving postoperative adjuvant therapy. These cases highlight the need to remain vigilant for local recurrence, however long after the initial diagnosis.

Unlabelled: Surgical resection margins are reportedly the most important predictor of survival and local recurrence with sacral chordomas. We examined the relevance of invasion of the surrounding posterior pelvic musculature (piriformis and gluteus maximus) at initial diagnosis to local recurrence after sacrectomy. We retrospectively reviewed 18 patients with histologically verified sacral chordoma seen at our institution between 1998 and 2005. There were 14 men and four women with a mean age of 65.1 years (range, 31-78 years). The average overall followup was 4.4 years (range, 0.5-10 years), 5.4 years for the living patients (range, 3-10 years), and 2.8 years for the deceased (range, 0.5-5.4 years). Local recurrence occurred in 12 patients (66%) 29 months postoperatively (range, 2-84 months). Six of these patients had wide excisions at initial surgery, five had marginal excisions, and one had an intralesional excision. Ten patients had wide surgical margins, six of whom (60%) had local recurrences. Tumor invasion of adjacent muscles at presentation was present in 14 patients, 12 of whom (85%) had local recurrences. Sacroiliac joint involvement was seen in 10 patients, nine of whom (90%) had local recurrences. The findings suggest obtaining wide surgical margins posteriorly, by excising parts of the piriformis, gluteus maximus, and sacroiliac joints, may result in better local disease control in patients with sacral chordoma., Level of Evidence: Level IV, prognostic study. See the Guidelines for Authors for a complete description of levels of evidence.

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.

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.

Purpose: To report the use of a composite ceramic bone graft substitute containing calcium sulphate and hydroxyapatite (HA) in the treatment of large expansive osteolytic benign bone tumours., Methods: 4 women and 9 men aged 8 to 49 (mean, 22) years with aneurysmal bone cysts (n=6) or giant cell tumours (n=7) in the epi- or meta-physeal areas of the lower limbs underwent curettage, phenolisation, and filling with bone graft substitute containing calcium sulphate and HA. The mean tumour size was 38.5 (range, 18-65) ml. The patients were followed up for a mean of 41 (range, 33-52) months. Range of movement, Musculoskeletal Tumor Society Rating Score (MTSRS), and haematological and blood biochemical parameters were measured., Results: Two patients had recurrence at 7 and 9 months, both progressed to grade-III giant cell tumours. One underwent revision with an iliac crest autograft, whereas the other underwent en bloc excision and prosthetic replacement. The 11 other lesions displayed clinical and radiological consolidation at a mean of 4.6 (range, 3-7) months. No restriction of range of movement was observed, except in the patient undergoing prosthetic replacement. The mean MTSRS was 96% (range, 83-100%) of that expected for normal function. During the follow-up period, haematological and blood biochemical parameters stayed within normal limits., Conclusion: Composite bioceramic osteoconductive grafts, which combine porous HA with calcium sulphate, provide a framework for human osteogenesis and avoid donor-site morbidity (autologous bone graft harvesting). Tumour recurrence remains a major concern especially in young patients, as revision invariably requires removal of additional bone, potentially compromising joint integrity.

We treated three patients with aneurysmal bone cysts and seven with giant cell tumours who presented with a large osteolytic lesion in peri-articular areas of the knee. The patients age ranged from 8 to 49 years (mean 25 years). The average tumour volume was measured at 39.8 cm(3) (range 18 to 65 cm(3)). The tumour cavities were treated with curettage, phenolisation and application of a composite bone graft substitute containing 35% calcium-sulphate hemihydrate and 65% hydroxyapatite granules. All patients were followed up for 4.0 to 5.2 years (mean 4.5 years). Tumour recurrence was noted in two cases. In the remaining patients consolidation of the lesion was considered complete at a mean of 4.5 months (3-6.5 months). During follow-up no deformities developed and no radiological signs of joint degeneration were noted. All patients regained close to normal function, with a mean Musculoskeletal Tumour Society Rating Score of 95.1%. Due to their good osteoconductive abilities, composite synthetic bone graft substitute combining porous hydroxyapatite with calcium-sulphate appears to be an effective alternative to autologous cancellous bone graft in the treatment of large osteolytic lesions in peri-articular areas around the knee joint. They bear major advantages through ubiquitous availability and the avoidance of morbidity associated with iliac crest harvest. Concerns remain as the radio-opaque appearance of the bone graft substitute may potentially delay the detection of tumour recurrence.

Between 1988 and 2006, 18 patients had a custom-made endoprosthetic replacement of the distal humerus for bone tumours at our institution. There were 11 primary malignant neoplasms, six secondary deposits, and one benign aggressive tumour. The mean follow-up was for 4.4 years (1 to 18.2). Complications occurred in nine patients and included aseptic loosening in three (16.6%), local recurrence in two (11%), infection in two (11%), neuropraxia of the radial nerve in one (5.5%) and a peri-prosthetic fracture in one (5.5%). Excision was inadequate in four patients (22%), all of which developed local recurrence and/or metastases. There were seven deaths from the primary disease after a mean of 2.3 years (1 to 5), one of whom had an above-elbow amputation for local recurrence seven months before death. The remaining six had satisfactory elbow function at their last follow-up. The 11 living patients were evaluated using the Musculoskeletal Tumour Society and Toronto Extremity Salvage scoring systems. The mean scores achieved were 76% (67% to 87%) and 73% (59% to 79%), respectively. Overall, 17 of 18 patients had significant improvement in the degree of their pain following operation. Custom-made endoprosthetic reconstruction of the elbow for bone tumours is a viable treatment in carefully selected patients. It maintains satisfactory function and provides good pain relief.

Primary giant cell tumours involving digital bones of the hand are rare lesions which are generally diagnosed at an advanced stage. Accurate diagnosis requires clinical evaluation, imaging studies and histopathological assessment. Conservative treatment by digit-sparing surgery is associated with high recurrence rates. In a ten year retrospective review, this study identified only four cases. Three cases involved a phalanx and were treated by distal amputation of the involved digit. None recurred. One involved the metacarpal and recurred twice following repeated curettage and bone grafting. No further recurrence has been detected after resection and replacement with a non-vascularised fibular graft and Silastic implant replacement of the metacarpophalangeal joint. Our small series of cases supports a policy of aggressive primary surgery, including amputation or en bloc resection and reconstruction.

Chordomas are rare malignant bone tumours considered to arise from notochordal remnants that persist in the axial skeleton. Although their morphology can resemble that of a carcinoma, chondrosarcoma or malignant melanoma, the axial location and their well-defined immunophenotype, including expression of cytokeratins (CK7/20/8/18/19) and S100, generally allow the diagnosis to be made with confidence once the possibility is considered. In contrast, making a robust diagnosis of an extra-axial chordoma has been difficult in the absence of specific markers for chordomas. We have recently shown in gene expression microarray and immunohistochemistry studies that brachyury, a transcription factor crucial for notochordal development, is a specific and sensitive maker for chordomas. We now present a case of an intracortical tibial tumour, with detailed report of the imaging, and morphological features consistent with a chordoma, where notochordal differentiation was demonstrated with an antibody to brachyury. The tumour cells were also positive for cytokeratins, including CK19, and S100, CEA, EMA and HMBE1, findings which support the diagnosis of chordoma. Brachyury can be employed as a marker of notochordal differentiation and help identify confidently, for the first time, extra-axial bone and soft tissue chordomas, and tumours which may show focal notochordal differentiation.

We developed an in vivo model of the attachment of a patellar tendon to a metal implant to simulate the reconstruction of an extensor mechanism after replacement of the proximal tibia. In 24 ewes, the patellar tendon was attached to a hydroxyapatite (HA)-coated titanium prosthesis. In 12, the interface was augmented with autograft containing cancellous bone and marrow. In the remaining ewes, the interface was not grafted. Kinematic gait analysis showed nearly normal function of the joint by 12 weeks. Force-plate assessment showed a significant increase in functional weight-bearing in the grafted animals (p = 0.043). The tendon-implant interface showed that without graft, encapsulation of fibrous tissue occurred. With autograft, a developing tendon-bone-HA-implant interface was observed at six weeks and by 12 weeks a layered tendon-fibrocartilage-bone interface was seen which was similar to a direct-type enthesis. With stable mechanical fixation, an appropriate bioactive surface and biological augmentation the development of a functional tendon-implant interface can be achieved.

Aim: To describe the use of MRI to identify and biopsy areas of dedifferentiation in patients with a suspected diagnosis of dedifferentiated chondrosarcoma., Materials and Methods: Low-grade chondrosarcoma is characterized at magnetic resonance imaging (MRI) as having a lobulate, hyperintense appearance on T2-weighted spin-echo sequences. T2-weighted MR images were assessed in 15 patients with a final pathological diagnosis of dedifferentiated chondrosarcoma for regions of atypical reduced signal intensity. Information regarding the site of ultrasound or computed tomography (CT)-guided biopsy was available in 10 cases., Results: Nine patients were male and six female with a mean age of 60 years (range 25-77 years). The sites involved were the distal femur (n+4) pelvis (n=3) proximal femur (n=4) femoral diaphysis (n=1) proximal humerus (n=2) and proximal tibia (n=1). The dedifferentiated component consisted of osteosarcoma (n=5) malignant fibrous histiocytoma (n=6) spindle cell sarcoma (n=1) leiomyosarcoma (n=1) and pleomorphic sarcoma (n=1). In 14 of the 15 cases, areas of lower signal intensity lacking in lobulation were identified. In nine of the 10 cases, biopsy site included such areas and yielded high-grade sarcoma., Conclusions: Dedifferentiation within chondrosarcoma may be identified on T2-weighted MRI as areas of reduced signal intensity. These areas should be the preferred site of biopsy.

Aim: High grade periacetabular osteosarcomas extending to the sacro-iliac region present a difficult management problem. We describe our experience of the swing procedure as a method of limb salvage., Method: The procedure was performed on three patients with stage II B osteosarcoma., Results: Functional outcome was poor in all three with a mean Musculoskeletal Tumor Society score of 29% (27-30) at last review. All three developed local recurrence and one developed pulmonary metastases. The mean disease free interval post-operatively was 4.7 months (4-6) and they died of their disease at a mean of 10 months (5-16)., Conclusions: This form of limb salvage is technically demanding and carries a high morbidity. Functional outcome is poor and the prognosis for this group of patients is not improved by surgery. Alternative treatment options are discussed.

The distal radius and its articulations clearly have a vital role in the functional abilities of the hand and wrist. The treatment of tumours of the radius, therefore, needs to achieve satisfactory clearance of the lesions while also preserving as much wrist function as possible. We have employed osteoarticular autologous fibula grafts to reconstitute the distal radius after tumour excision. Since 1992, 13 patients have been treated by this technique for tumours of the distal radius. The group comprised patients with primary and recurrent giant cell tumours of bone, osteosarcoma, chondrosarcoma and Ewing's tumour. This technique achieves a satisfactory functional result without compromise of the prognosis for the tumour., (Copyright 2002 The British Society for Surgery of the Hand.)

We performed a functional analysis of massive knee replacements after extra-articular resection of primary bone tumors. Patients did not score as highly on the Musculoskeletal Tumour Society Score and Knee Society Score (P < or = .01) as patients who had undergone intra-articular resection. Lower scores were achieved for emotional acceptance (P < or = .01), functional restriction (P < or = .05), walking distance (P < or = .05), and managing stairs (P < or = .05). These patients had a significantly greater extension lag (P < or = .01) and greater fixed flexion (P < or = .05). This functional deficit is primarily due to a compromised extensor mechanism inherent in the type of surgical resection necessary to achieve adequate removal of the tumor.

To combat the high incidence of aseptic loosening for young patients and for patients with failed implants after resection for bone tumors, intramedullary cementless fixation of massive tumor implants was investigated. These implants consist of a hydroxyapatite coated titanium stem. To date, 47 of these prostheses have been inserted for the treatment of primary bone tumors. Radiographs indicate that the stems are osseointegrated. Radiolucent lines have not been seen between the implant and the bone. Bone remodeling changes have been observed. In several cases in which the implant was not seated properly on the transaction site, bone grew to the shoulder of the implant. Bone remodeling was particularly evident in stems that were coated over their entire surface. In these cases, the implant induced local bone resorption so that the bone around the midstream region became thinner, with resorption of cortical bone on the periosteal surface and maintenance of bone on the endosteal surface adjacent to the stem. This effect was attributed to stress shielding, and a three-dimensional finite element model using loading data obtained from a telemetry study indicated that, where the stem was bonded to the bone over the entire surface, stresses in the outer cortex became reduced. In the finite element model, reducing the region of hydroxyapatite coating to approximately 1/3 of the stem length reduced the extent of the low-stress area in the outer cortex. Subsequently, prostheses have been coated with hydroxyapatite over only approximately 1/3 of their stem. This method of fixing the massive endoprosthesis to the bone is thought to be successful in the short-term and offers an alternative to cemented fixation.

In this case report we demonstrate the usefulness of targeted radiotherapy in the form of rhenium-186 HEDP as a method for dose escalation in the treatment of osteosarcoma.

Although rarely required, extendable reconstruction devices for replacing the entire femur offer children with malignant bone tumors the opportunity of a nearly normal development by overcoming an expected leg length discrepancy. Femoral integrity can be restored, allowing most patients to walk without the use of aids. There are no data available to provide evidence regarding long term results and morbidity in such patients. Six patients (range 2-12 years of age), three with osteogenic osteosarcoma and three with Ewing's sarcoma, were treated between 1988 and 1996 with custom made Stanmore extendable prosthetic total femoral replacements. One patient died 12 months after surgery because of complications relating to pulmonary metastasis. The remaining five patients were observed between 2.7 and 8.9 years (average, 5 years). No tumor recurrence has been recorded and no amputation has been performed. All surviving patients underwent an average of 9.4 operative procedures (range, 4-16 procedures) including 6.4 extension procedures (range, 3-10 procedures), and one prosthetic revision (range, 0-3 procedures). Five revisions in two patients were necessary because of infection, loosening of the prosthesis, mismatch between femoral head and acetabulum, or full extension of the extending mechanism. The functional results were measured in accordance with the Musculoskeletal Tumor Society rating score, with an average result of 77.3%. Total femoral replacement in a growing individual achieves good functional results yet has various risks for an uncertain outcome. Careful selection of the patient and realistic appraisal of the long term prospects are essential for successful treatment.

The use of extendible distal femoral replacements is a relatively new treatment alternative for malignant bone tumours in growing individuals. Although their appearance was widely appreciated, questions about functional practicality and longevity remain unclear. With longer follow-up, advantages of immediate functional restoration and beneficial psychological aspects seem to be overshadowed by an increase in complications such as aseptic loosening, infection or prosthetic failure. We have reviewed 18 children with such tumours who were treated between 1983 and 1990 by custom-made Stanmore extendible distal femoral replacements. Four died from metastatic disease within 2.5 years of operation and two required amputation for local recurrence or chronic infection. The remaining 12 patients were followed for a mean of 8.7 years (6 to 13.2). A mean total lengthening of 5.2 cm was achieved, requiring, on average, 4.3 operations. Using the Musculoskeletal Tumor Society rating score the functional result at review was, on average, 77% of the expected normal function, with seven patients achieving > or = 80%. Revision of the prosthesis was required in ten patients, in six for aseptic loosening, at a mean of 6.2 years after the initial procedure.

Mixed lytic and sclerotic myeloma are uncommon and occur at an earlier age than multiple myeloma in areas of persistent haemopoiesis. Their characteristic symptoms are pain, swelling and pathological fractures and they are usually detected at an early stage. We report a case of a mixed sclerotic/lytic myeloma of the humerus presenting at an unusually advanced stage and associated with a bilateral progressive demyelinating peripheral sensory and motor polyneuropathy resembling polyradiculitis. The neurological changes partly disappeared after the tumour had been resected which suggests that nerve reconstitution may occur due to reversal of the demyelination.