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We report a retrospective study over a 15-year period, between 2005 and 2020, evaluating clinical and functional outcomes in patients who underwent reconstruction of the distal radius with an endoprosthetic replacement following excision of both malignant and aggressive benign bone tumours. Data was collected retrospectively from a prospectively maintained electronic database, and prospectively via telephone patient consultation. Musculoskeletal Tumour Society and patient-rated wrist evaluation scores were assessed at a minimum of 1 year postoperatively. Of nine implants, five were arthrodeses and four were arthroplasties. One patient required amputation within 6 months for proximal metastatic disease. At last follow-up, eight patients subjectively reported good function. Five patients returned to high functionally demanding jobs. Mean Musculoskeletal Tumour Society and patient-rated wrist evaluation scores were 72% and 50/100, respectively. We conclude that distal radius endoprosthetic replacements offer acceptable functional outcomes and remain a viable option when biological reconstruction is not possible. Level of evidence: IV [ABSTRACT FROM AUTHOR]

Purpose: Describe and compare the functional and oncological outcomes and complications between paediatric and adult patients after intra-lesional treatment of benign tumours of the proximal femur, stabilised with an autologous non-vascularised fibular strut graft (NVFSG). Methods: Retrospective review including 54 patients with a benign histopathological diagnosis treated between 1987 and 2018. The mean age at operation was 17 years (range, 3 to 37 years) with a median follow-up of 39.5 months (IQR 46.7 months). Patients were grouped according to their age at diagnosis (< 16 years versus ≥ 16 years). Data collection included weight-bearing status, Musculoskeletal Tumour Society (MSTS) score, local recurrence, revision surgery and complications. Local recurrence-free survival (LRFS) and revision-free survival (RFS) were calculated and compared between groups. Results: The median MSTS score for all patients was 98.3% (IQR 6.7%) without a statistically significant difference (p = 0.146) between both groups. The median time to full weight-bearing was 12 weeks (IQR 0 weeks). Local recurrence occurred in five (9%) patients. LRFS for all patients was 96% at 2 years and 88% at 5 years. Although local recurrence was more frequent in the paediatric group, LRFS did not significantly differ (p = 0.155, 95% CI 223.9 to 312.3) between both groups. Reoperation rate was 13% and was indicated for local recurrence, post-operative fracture, graft resorption and avascular necrosis. RFS for all patients was 90% at 2 years and 85% at 5 years. There was no statistically significant difference (p = 0.760, 95% CI 214.1 to 304.6) regarding RFS between both groups. Conclusion: The use of an autogenous NVFSG after intra-lesional curettage of benign proximal femoral lesions allows for a biological, structural stabilisation without additional osteosynthesis, hastening weight-bearing and avoiding metalwork-related complications with minimal post-operative morbidity and complications and excellent functional and oncological outcome for both children and adults. [ABSTRACT FROM AUTHOR]

Surgery is the main treatment option for patients with aneurysmal bone cyst (ABC). We report our experience of using denosumab as an alternative treatment in a child with a multiply recurrent and unresectable tibial ABC. The efficacy and safety of denosumab in the paediatric population, and in the treatment of ABC, are still to be fully evaluated. We describe a 13-year-old boy with an extensive and aggressive ABC involving the proximal tibia, which had recurred following multiple previous surgeries. The patient had ongoing severe pain, was unable to weight-bear, and was at significant risk of pathological fracture. En bloc resection and embolization were not deemed viable, and a decision to use denosumab was made. He received 17 doses of subcutaneous denosumab (70 mg/m2) over a 27-month period, at increasing dose intervals. His symptoms significantly improved, and bony consolidation was observed within six months of treatment. He was able to walk without protection and fully weight-bear without any pain by 18 months. With an increase to a six-month dosing interval, the patient presented with a severe, symptomatic rebound hypercalcaemia requiring bisphosphonate therapy. This reoccurred on two further occasions. This case adds to the evidence that denosumab is effective in the treatment of ABC in paediatric patients, but there is a risk of rebound hypercalcaemia. Therefore, patient awareness and biochemical monitoring for rebound hypercalcaemia are essential. [ABSTRACT FROM AUTHOR]

Surgery is the main treatment option for patients with aneurysmal bone cyst (ABC). We report our experience of using denosumab as an alternative treatment in a child with a multiply recurrent and unresectable tibial ABC. The efficacy and safety of denosumab in the paediatric population, and in the treatment of ABC, are still to be fully evaluated. We describe a 13-year-old boy with an extensive and aggressive ABC involving the proximal tibia, which had recurred following multiple previous surgeries. The patient had ongoing severe pain, was unable to weight-bear, and was at significant risk of pathological fracture. En bloc resection and embolization were not deemed viable, and a decision to use denosumab was made. He received 17 doses of subcutaneous denosumab (70 mg/m2) over a 27-month period, at increasing dose intervals. His symptoms significantly improved, and bony consolidation was observed within six months of treatment. He was able to walk without protection and fully weight-bear without any pain by 18 months. With an increase to a six-month dosing interval, the patient presented with a severe, symptomatic rebound hypercalcaemia requiring bisphosphonate therapy. This reoccurred on two further occasions. This case adds to the evidence that denosumab is effective in the treatment of ABC in paediatric patients, but there is a risk of rebound hypercalcaemia. Therefore, patient awareness and biochemical monitoring for rebound hypercalcaemia are essential. [ABSTRACT FROM AUTHOR]

Purpose Chondrosarcomas typically present in adults during the fifth to seventh decades and are rare in young patients. The biological behaviour and oncological outcomes may be different in children and adolescents. Methods We retrospectively evaluated the outcomes of all patients with chondrosarcoma of bone who were younger than 18 years of age at the time of diagnosis and were treated at our centre between 1995 and 2018. Results The 15 consecutive patients studied included nine male and six female cases, with a mean age at diagnosis of 13 years (7 to 17). The median follow-up was 117 months (30 to 277). The tumours were primary and secondary in ten and five patients, respectively. The tumours were central in 13 and surface in two patients. The tumour locations were the humerus in five, digits in five, femur in three, radius in one and pelvis in one patient. The histological grades were grade I in seven, grade II in seven and grade III in one patient. The surgical treatments were limb salvage in ten patients and ray amputation in five patients. The surgical margins were wide in eight, marginal in two and intralesional in five patients. All the patients were alive and continuously free of disease at the time of the last follow-up. No patient developed metastases or local recurrence. Conclusion Chondrosarcoma of bone in children and adolescent patients has a very good prognosis and is less aggressive compared with published outcomes in older patients. [ABSTRACT FROM AUTHOR]

Background: Extendable endoprostheses are used to reconstruct segmental defects following resection of bone sarcomas in skeletally immature patients. However, there remains a paucity of studies with regard to long-term outcomes.Methods: We retrospectively reviewed 124 skeletally immature children who underwent an extendable endoprosthetic replacement and survived more than 10 years after the surgical procedures. Anatomical sites included the distal part of the femur (n = 66), the proximal part of the femur (n = 13), the proximal part of the tibia (n = 29), and the proximal part of the humerus (n = 16). Complications and implant survival were classified according to the modified Henderson criteria.Results: The mean follow-up was 24 years (range, 10 to 36 years). The mean age at the time of the extendable endoprosthetic replacement was 9 years (range, 2 to 16 years). All patients had reached skeletal maturity at the last follow-up. The 10-year endoprosthetic failure-free survival rate was 28%. A total of 243 complications occurred in 90% of patients; these complications were most frequently related to soft-tissue problems (27% of complications). The incidence of and cumulative survival with respect to each failure mode varied between anatomical sites. Soft-tissue failures occurred most frequently in the proximal part of the femur (77%; p = 0.003), and the distal part of the femur was the most frequent site of aseptic loosening (52%; p = 0.014) and structural failure (55%; p = 0.001). Excluding lengthening procedures, 105 patients (85%) underwent an additional surgical procedure, with a mean of 2.7 surgical procedures per patient (range, 0 to 7 surgical procedures per patient). The mean limb-length discrepancy at the final follow-up was 1 cm (range, 0 to 9 cm). Limb salvage was achieved in 113 patients (91%). The mean Musculoskeletal Tumor Society functional score (the percentage of a total score of 30 points) was 82% (range, 40% to 100%) in 115 patients with available data at the last follow-up.Conclusions: Extendable endoprostheses are associated with a high complication rate and a need for additional surgical procedures over time. Despite this, successful limb salvage with reasonable function and small limb-length discrepancy is achievable in the long term. Our study provides benchmark data for individual anatomical sites for further improvements of outcomes.Level Of Evidence: Therapeutic Level IV. See Instructions for Authors for a complete description of levels of evidence. [ABSTRACT FROM AUTHOR]

Background: The proximal tibia is one of the most challenging anatomic sites for extremity reconstructions after bone tumor resection. Because bone tumors are rare and large case series of reconstructions of the proximal tibia are lacking, we undertook this study to compare two major reconstructive approaches at two large sarcoma centers.Questions/purposes: The purpose of this study was to compare groups of patients treated with endoprosthetic replacement or osteoarticular allograft reconstruction for proximal tibia bone tumors in terms of (1) limb salvage reconstruction failures and risk of amputation of the limb; (2) causes of failure; and (3) functional results.Methods: Between 1990 and 2012, two oncologic centers treated 385 patients with proximal tibial resections and reconstruction. During that time, the general indications for those types of reconstruction were proximal tibia malignant tumors or bone destruction with articular surface damage or collapse. Patients who matched the inclusion criteria (age between 15 and 60 years old, diagnosis of a primary bone tumor of the proximal tibia treated with limb salvage surgery and reconstructed with endoprosthetic replacement or osteoarticular allograft) were included for analysis (n = 149). In those groups (endoprosthetic or allograft), of the patients not known to have reached an endpoint (death, reconstructive failure, or limb loss) before 2 years, 85% (88 of 104) and 100% (45 of 45) were available for followup at a minimum of 2 years. A total of 88 patients were included in the endoprosthetic group and 45 patients in the osteoarticular allograft group. Followup was at a mean of 9.5 (SD 6.72) years (range, 2-24 years) for patients with endoprosthetic reconstructions, and 7.4 (SD 5.94) years for patients treated with allografts (range, 2-21 years). The following variables were compared: limb salvage reconstruction failure rates, risk of limb amputation, type of failures according to the Henderson et al. classification, and functional results assessed by the Musculoskeletal Tumor Society system.Results: With the numbers available, after competitive risk analysis, the probability of failure for endoprosthetic replacement of the proximal tibia was 18% (95% confidence interval [CI], 10.75-27.46) at 5 years and 44% (95% CI, 31.67-55.62) at 10 years and for osteoarticular allograft reconstruction was 27% (95% CI, 14.73-40.16) at 5 years and 32% (95% CI, 18.65-46.18) at 10 years. There were no differences in terms of risk of failures at 5 years (p = 0.26) or 10 years (p = 0.20) between the two groups. Fifty-one of 88 patients (58%) with proximal tibia endoprostheses developed a reconstruction failure with mechanical causes being the most prevalent (32 of 51 patients [63%]). A total of 19 of 45 osteoarticular allograft reconstructions failed (42%) and nine of 19 (47%) of them were caused by early infection. Ten-year risk of amputation after failure for endoprosthetic reconstruction was 10% (95% CI, 5.13-18.12) and 11% (95% CI, 4.01-22.28) for osteoarticular allograft with no difference between the groups (p = 0.91). With the numbers available, there were no differences between the groups in terms of the mean Musculoskeletal Tumor Society score (26.58, SD 2.99, range, 19-30 versus 27.52, SD 1.91, range, 22-30; p = 0.13; 95% CI, -2,3 to 0.32). Mean extension lag was more severe in the endoprosthetic group than the osteoarticular allograft group: 13.56° (SD 18.73; range, 0°-80°) versus 2.41° (SD 5.76; range, 0°-30°; p < 0.001; 95% CI, 5.8-16.4).Conclusions: Reconstruction of the proximal tibia with either endoprosthetic replacement or osteoarticular allograft appears to offer similar reconstruction failures rates. The primary cause of failure for allograft was infection and for endoprosthesis was mechanical complications. We believe that the treating surgeon should have both options available for treatment of patients with malignant or aggressive tumors of the proximal tibia. (S)he might consider an allograft in a younger patient to achieve better extensor mechanism function, whereas in an older patient or one with a poorer prognosis where return to function and ambulation quickly is desired, an endoprosthesis may be advantageous.Level Of Evidence: Level III, therapeutic study. [ABSTRACT FROM AUTHOR]

Purpose: The aim of the study was to report overall survival, local recurrence and development of metastasis in a group of patients with femur chondrosarcoma that presented with or without a pathological fracture. Methods: A retrospective review was performed, and 182 patients (39 pathological fractures) that had been treated by oncologic surgery for femoral chondrosarcoma were included. The mean age of the series was 50 years (range, eight to 90) and 114 patients were male (63 %). Mean follow-up was 113 months (range, three to 216). Cancer-specific overall survival, development of metastasis and local recurrence were analysed, grouping patients by grade (grade I / grade 2-3 / dedifferentiated). Results: Disease-specific survival in the entire group of chondrosarcoma of the femur was 69 % (CI95 % 63-76) at 5 years. Five-year disease-specific survival in the fracture group was 49 % lower than in the control group 75 % (p = 0.0001). Survival of patients with grade 1 chondrosarcoma with fracture was significantly less than those without fracture ( p = 0.02) but there was no difference in those with grade 2-3 ( p = 0.49) and dedifferentiated tumours ( p = 0.09). The local recurrence rate of the entire series was 27 %. Only dedifferentiated chondrosarcomas with an associated pathological fracture had a significantly higher rate of local recurrence. There was no relationship between development of metastases and fracture. Conclusion: A pathological fracture of the femur has a negative prognostic influence in grade 1 chondrosarcoma and increases the risk of local recurrence in dedifferentiated femur chondrosarcomas. [ABSTRACT FROM AUTHOR]

Background: Controversy exists regarding the ideal method of reconstruction after proximal humeral resection and several reconstructive techniques have been reported. The reconstructive options are very limited when resection of the entire humerus is required. One option is endoprosthetic reconstruction, but there have been few published studies on the outcome of total humeral endoprosthetic reconstruction. Questions/purposes : The purposes of this study were (1) to assess the longevity of total humerus prostheses in those patients who survived their disease; (2) to review the complications associated with this prosthesis; and (3) to assess the Musculoskeletal Tumor Society functional score in survivors. Methods: Thirty-four patients (10 males, 24 females) with a mean age of 26 years (range, 7-86 years) were included in this study. Histological diagnosis was osteosarcoma in 15 patients, chondrosarcoma in seven, Ewing's sarcoma in seven, metastatic carcinoma in three, liposarcoma in one, and giant cell tumor of bone in one remaining patient. Twenty-nine patients had their total humeral endoprosthetic replacement for primary reconstruction, whereas the remaining five patients received their implants for failures with other reconstructive techniques. At a minimum followup of 3 months (mean, 8.2 years; range, 3 months to 29 years), 16 patients were alive with no evidence of disease, whereas 13 of the remaining 18 died with metastatic disease. Local recurrence was seen in five patients and all eventually died of disease progression. Results: According to the Kaplan-Meier survival analysis, the cumulative 10-year implant survival rate was 90%. Periprosthetic infection was seen in four patients, postoperative radial nerve palsy in one, and proximal migration of the prosthesis in three, whereas three patients needed a change of the articular elbow bushings at a mean of 16 years after the implant insertion. The mean Musculoskeletal Tumor Society functional score of the 28 patients who survived their disease for more than 12 months after the index procedure and could therefore be functionally assessed was 83% (range, 60%-93%). Conclusions: From this small, preliminary report, we suggest that total humeral endoprosthetic replacement may be a reasonable option of reconstruction after tumor resection. We have shown that this prosthesis preserves the function of the hand. The local recurrence rate observed suggests that careful selection of patients is crucial. Infection was our most common surgical complication, but we showed that in those who survived their tumor, this prosthesis offers a method to preserve a functional upper extremity in some patients. Further study with more patients is necessary to confirm the value of this reconstruction method. Level of Evidence: Level IV, therapeutic study. See Guidelines for Authors for a complete description of levels of evidence. [ABSTRACT FROM AUTHOR]

Purpose: We report on a group of patients with tumours in the Hoffa's fat pad (HFP), their clinical presentation, histological type and treatment, including two synovial sarcomas with their clinical follow-up, which have not been described previously in the literature. Methods: We performed a retrospective review of our prospectively collected database of 25 cases of HFP tumours with at least six months follow-up. Results: The gender, age at presentation (over and under 16 years of age), clinical features, history of trauma, treatment chosen, and complications were recorded. The mean age of the patients was 32 years (three to 47). Six patients were under 16 years old. Pain was the most common symptom, present in 92 % ( n = 23/25). The final diagnoses included 23 (92 %) benign tumours and two (8 %) malignant tumours. The most common benign tumour was pigmented villonodular synovitis (PVNS) (48 % n = 12). The two malignant tumours were synovial sarcomas and both presented in patients under 16 years old. Conclusions: Hoffa's fat pad tumours are an uncommon and rarely diagnosed group of lesions that can be misinterpreted as any knee pathology. Although the majority of HFP tumours are benign, malignant tumours should be considered in the differential diagnosis for the paediatric population. [ABSTRACT FROM AUTHOR]

Purpose: We report the outcome of intercalary resection of the femoral diaphysis and extracorporeal irradiated autologous bone graft reconstruction, without the use of vascularized fibular graft. Methods: Six patients with Ewing sarcoma of the mid-shaft femur who were treated by limb sparing tumour resection and reconstruction with extracorporeal irradiated autologous bone graft with intramedullary cement between 2002 and 2010 were studied. Results: Mean age at the time of surgery was ten years (range, four-23). The length of resected femoral bone averaged 23 cm (15-32 cm). The ratio of bone resection length to total femoral length averaged 60 % (56-66 %). The patients had been followed up for between 16 and 79 months (mean, 41 months) at the time of the study. There was no infection nor fracture in this series. Primary union of the distal and proximal osteotomy sites was achieved in three patients. Delayed union of the proximal osteotomy site occurred in one patient that was successfully treated with iliac crest bone grafting. One patient developed non-union at the distal osteotomy site which failed to heal with bone grafting and was therefore converted to endoprosthetic replacement, and another patient was converted to rotationplasty at five months post-surgery because of contaminated margins. Function was excellent in all patients with surviving re-implanted bone. Local recurrence arose in one patient. Conclusion: Our experience suggests that cement augmentation of extracorporeal irradiated and re-implanted bone autografts offer a useful method of reconstructing large femoral diaphyseal bone defects after excision of primary malignant bone tumours. [ABSTRACT FROM AUTHOR]

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]

Background: Children rarely require hip replacement, and therefore very little is known about the most effective procedure for children who require hip replacement after proximal femoral resection for a tumor. Methods: We reviewed the outcome of forty hip replacements in children between two and fifteen years old who had a proximal femoral replacement for malignant disease. Twenty-one children survived the illness and were followed for three to twenty-one years. Cemented acetabular components were used initially to restore hip function, but, in more recent procedures, unipolar replacements and uncemented implants were used. Results: Children who were over the age of eleven years at the time of surgery had a rate of failure (defined as revision of the acetabular component) of 25% at ten years compared with a rate of 75% in younger children. Unipolar replacements in children of all ages failed by ten years, either because of pain or subluxation that led to revision. In children over the age of eleven years, both cemented and uncemented acetabular implants performed well. Conclusions: The preferred method for restoration of hip function in children under the age of eleven years following proximal femoral resection for a tumor remains unclear. We recommend that at the initial hip surgery an attempt should be made to cause as little damage as possible to the acetabulum, but most children will inevitably need revision surgery as they get older. We hope that this study will guide others in their decision-making with regard to this relatively rare condition. Level of Evidence: Therapeutic Level III. See Instructions to Authors for a complete description of levels of evidence. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

The indications for primary amputation of a localized soft tissue sarcoma (STS) of the extremity are not well defined in the literature. However, it has been suggested that patients who require an amputation to treat an STS are at increased risk for developing metastases. We categorized the main indications for primary amputation in our patient population and compared their oncological outcome with the outcome of patients who underwent limb-sparing surgery. 413 consecutive patients treated surgically at a single center for primary, nonmetastatic, deep, intermediate-, or high-grade STS of the extremity were reviewed. Indications for primary amputation were identified. Demographics and outcomes were compared between the amputation and limb-salvage groups. Multivariate Cox model analysis was used to identify independent risk factors for systemic relapse. Twenty-five (6%) of 413 patients with STS underwent primary amputation: they were older ( P = .05), had larger tumors ( P = .001), and had a significantly greater risk of developing metastatic disease than patients who underwent limb-sparing procedures ( P = .008). However, multivariate analysis demonstrated that the only independent predictors of systemic relapse were tumor size ( P = .0001) and tumor grade ( P = .0001). Primary amputation was not an independent risk factor for metastatic disease. The decision to perform a primary amputation for an STS of the extremity is based on the location and local extent of the tumor, and the expected function of the extremity after tumor resection. The higher risk of metastases for patients who require primary amputation is accounted for by independent risk factors associated with their tumors—predominantly large tumor size. [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]