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Survival rates and local control after resection of a sarcoma of the pelvis compare poorly to those of the limbs and have a high incidence of complications. The outcome for patients who need a hindquarter amputation (HQA) to treat a pelvic sarcoma is poor. Our aim was to evaluate the patient, tumour, and reconstructive factors that affect the survival of the patients who undergo HQA for primary or recurrent pelvic sarcoma.

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We analyzed the long-term outcomes of patients observed over ten years after resection en bloc and reconstruction with extracorporeal irradiated autografts

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The purpose of this study was to clarify the clinical behaviour, prognosis, and optimum treatment of dedifferentiated low-grade osteosarcoma (DLOS) diagnosed based on molecular pathology.

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The primary aim of this study was to determine the effect of the duration of symptoms (DOS) prior to diagnosis on the overall survival in patients with a primary bone sarcoma.

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We present a retrospective review of patients treated with extracorporeally irradiated allografts for primary and secondary bone tumours with the mid- and long-term survivorship and the functional and radiographic outcomes.

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The aims of this study were to analyse the long-term outcome of vascularised fibular graft (VFG) reconstruction after tumour resection and to evaluate the usefulness of the method.

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Intercalary allografts following resection of a primary diaphyseal tumour have high rates of complications and failures. At our institution intercalary allografts are augmented with intramedullary cement and fixed using compression plating. Our aim was to evaluate their long-term outcomes.

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To assess complications and failure mechanisms of osteoarticular allograft reconstructions for primary bone tumours.

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Instability of the hip is the most common mode of failure after reconstruction with a proximal femoral arthroplasty (PFA) using an endoprosthesis after excision of a tumour. Small studies report improved stability with capsular repair of the hip and other techniques, but these have not been investigated in a large series of patients. The aim of this study was to evaluate variables associated with the patient and the operation that affect post-operative stability. We hypothesised an association between capsular repair and stability.

The aim of this study was to evaluate the prognostic and therapeutic factors which influence the oncological outcome of parosteal osteosarcoma. A total of 80 patients with a primary parosteal osteosarcoma were included in this retrospective study. There were 51 females and 29 males with a mean age of 29.9 years (11 to 78). The mean follow-up was 11.2 years (1 to 40). Overall survival was 91.8% at five years and 87.8% at ten years. Local recurrence occurred in 14 (17.5%) patients and was associated with intralesional surgery and a large volume of tumour. On histological examination, 80% of the local recurrences were dedifferentiated high-grade tumours. A total of 12 (14.8%) patients developed pulmonary metastases, of whom half had either a dedifferentiated tumour or a local recurrence. Female gender and young age were good prognostic factors. Local recurrence was a poor prognostic factor for survival. Medullary involvement or the use of chemotherapy had no impact on survival. The main goal in treating a parosteal osteosarcoma must be to achieve a wide surgical margin, as inadequate margins are associated with local recurrence. Local recurrence has a significant negative effect on survival, as 80% of the local recurrences are high-grade dedifferentiated tumours, and half of these patients develop metastases. The role of chemotherapy in the treatment of parosteal osteosarcoma is not as obvious as it is in the treatment of conventional osteosarcoma. The mainstay of treatment is wide local excision. Cite this article: Bone Joint J 2015;97-B:1698–1703

Bone sarcomas are rare cancers and orthopaedic surgeons come across them infrequently, sometimes unexpectedly during surgical procedures. We investigated the outcomes of patients who underwent a surgical procedure where sarcomas were found unexpectedly and were subsequently referred to our unit for treatment. We identified 95 patients (44 intra-lesional excisions, 35 fracture fixations, 16 joint replacements) with mean age of 48 years (11 to 83); 60% were males (n = 57). Local recurrence arose in 40% who underwent limb salvage surgery versus 12% who had an amputation. Despite achieving local control, overall survival was worse for patients treated with amputation rather than limb salvage (54% vs 75% five-year survival). Factors that negatively influenced survival were invasive primary surgery (fracture fixation, joint replacement), a delay of greater than two months until referral to our oncology service, and high-grade tumours. Survival in these circumstances depends mostly on factors that are determined prior to definitive treatment by a tertiary orthopaedic oncology unit. Limb salvage in this group of patients is associated with a higher rate of inadequate marginal surgery and, consequently, higher local recurrence rates than amputation, but should still be attempted whenever possible, as local control is not the primary determinant of survival.

Cite this article: Bone Joint J 2014;96-B:665–72.

The aim of this study was to assess a specific protocol for the treatment of patients with a parosteal osteosarcoma of the distal femur with limb salvage involving hemicortical resection and reconstruction using recycled pasteurised autograft and internal fixation. Between January 2000 and January 2010, 13 patients with a mean age of 26.5 years (17 to 39) underwent this procedure. All the tumours were staged according to Enneking’s criteria: there were eight stage IA tumours and five stage IB tumours. The mean follow-up was 101.6 months (58 to 142), and mean post-operative Musculoskeletal Tumour Society functional score was 88.6% (80% to 100%) at the final follow-up. All the patients had achieved bony union; the mean time to union was 11.2 months (6 to 18). Local recurrence occurred in one patient 27 months post-operatively. No patient had a pulmonary metastasis. A hemicortical procedure for the treatment of a parosteal osteosarcoma is safe and effective. Precise pre-operative planning using MRI is essential in order to define the margins of resection. Although it is a technically demanding procedure, gratifying results make it worthwhile for selected patients. Cite this article: Bone Joint J 2013;95-B:1275–9

Amputation was once widely practised for primary bone tumours of the limbs. Yet this situation has changed with limb salvage surgery becoming increasingly popular in the last 30 years. Many different techniques are now available. These include allografts, autografts, endoprostheses and allograft-prosthesis composites. This article reviews these methods, concentrating on the functional outcomes and complications that have been reported.

Aim. To compare the functional outcome of proximal femoral reconstruction using endoprosthetic replacement and hip arthrodesis using a vascularised fibular graft. Material and Methods. The study included thirty-five patients who had proximal femoral reconstruction following resection of a malignant bone tumour. Patients were divided into 2 groups according to the reconstructive modality used. Group 1 (15 patients) reconstructed by hip arthrodesis using a vascularised fibular graft. Group 2 (20 patients) reconstructed by endoprosthesis. The mean age of group I was 14.9 years (range, 7-25). 8 patients had Ewing's sarcoma, 5 osteogenic sarcoma, and 2 chondrosarcoma. In group 2, the mean age was 35 years (range, 14-61). Eight patients had osteogenic sarcoma, 2 chondrosarcoma, 2 Ewing's sarcoma, 1 lymphoma, 1 MFH, 1 synovial sarcoma, 1 parosteal osteosarcoma, and 4 metastatic carcinomas. Results. In group 1 failure of the fixation occurred in two patients, and deep infection developed in one patient. These patients were managed with revision of internal fixation and debridement respectively. The mean MSTS functional score was 87% (range 70 – 96%) at the time of the latest follow-up (mean = 64 months). The average limb length discrepancy was 2 cm (range 1 to 4). In group 2, two patients developed deep infection, one patient had a dislocation, and one had loosening of the implant with a re-operation rate of 20%. After a mean follow up of 49.1 months, the mean MSTS functional score was 79% (range 60 – 93%). Conclusion. The functional outcome of hip arthrodesis using a vascularized fibular graft is comparable to endoprosthetic replacement after proximal femoral resections. It should be considered as a durable reconstructive modality in children and young active patients with long life expectancy

There are eight reported cases in the literature of osteosarcomas secreting β-hCG. Our primary aim was to investigate the rate of β-hCG expression in osteosarcoma and attempt to understand the characteristics of osteosarcomas that secrete β-hCG. We reviewed 37 histopathology slides (14 biopsies and 23 surgical specimens) from 32 patients with osteosarcoma. The slides were retrospectively stained for β-hCG expression. Patient and tumour characteristics, including age, gender, tumour location, subtype, proportion of necrosis, presence of metastases and recurrence were recorded. A total of five of the 32 tumours were found to be positive for β-hCG expression (one strongly and four weakly). This incidence of this expression was found in tumours with poor histological response to neoadjuvant chemotherapy.

The use of β-hCG expression as a diagnostic, prognostic or follow-up marker is questionable and needs further investigation with a larger sample size.

AIMS: Parosteal osteosarcoma is a surface osteogenic sarcoma less agressive than conventional osteosarcoma. Most cases begin to show symptoms in the third and fourth decades, and it affects females more often than males. The tumor affected long bones of the limbs, with evident predilection for the distal femur. The aim of this study is to analyze the prognosis of parosteal osteosarcoma, studying its evolution to know its better treatment. Methods: This is a retrospective study of 25 patients with parosteal osteosarcoma. 23 cases have been treated at our institution from 1983 through June 2009, and 2 cases were treated in another center with a follow-up of at least 1 year. We studied the relation between different aspects of these patients: time of evolution, medullar canal invasion, size of tumor, dedifferentiated parosteal osteosarcoma or recurrence, images of RMI and kind of parosteal osteosarcoma, diagnoses with biopsy, relation between surgery and recurrences. Results: We had 4 men and 21 women with a mean age of 30,48 years (13–56).6 cases were dedifferentiated parosteal osteosarcoma. We observed hyperintensity sign in 57,1% cases of dedifferentiated parosteal osteosarcoma. From 18 trephine biopsies, only 9 cases were diagnosed with parosteal osteosarcoma, 8 cases out of 8 incisional biopsies were also diagnosed as parosteal osteosarcoma. There were 5 local recurrences in the first year follow-up. All patients with marginal excision recurred. All patients were alive, free from disease and without metastatic lesions when the study finished. There was relation between the time of evolution of the tumor and the medular canal invasion and the dedifferentiation, but we not found any relation with the tumoral recurrence. The presence of dedifferentiation and the recurrence were more common in bigger tumours. 3 patients were treated with chemotherapy. Conclusions: Parosteal osteosarcoma is a slow-growing tumor with a significantly better prognosis than the conventional osteosarcoma. The diagnosis of parosteal osteosarcoma requires an overall evaluation of the clinical, radiology and pathology findings. The initial study by RMI could help to guide our selective biopsy. First surgical procedure performed is a very important prognosis factor. Wide resection allows a better local control of the disease with fewer recurrences. Time of evolution and canal medullar invasion are more frequent in dedifferentiated parosteal osteosarcoma, but they not affect the rate of recurrence if the initial resection has been appropiate. In our opinion, postoperative chemotherapy should be given to patients with dedifferentiation and canal medular invasion in parosteal osteosarcoma

Florid reactive periostitis is a pronounced periosteal reaction, usually affecting the hands and feet, for which there is no obvious cause. It is rare in children and in long bones. We report an unusual case of florid reactive periostitis in a ten-year-old girl that involved both bones of the forearm. The lesion resolved over a period of one year, leaving a residual exostosis. She developed a physeal bar in the distal ulna in the region of the lesion at one-year follow-up. This was thought to be a complication of the biopsy procedure and was treated by resection and proximal ulnar lengthening.

Objective: To assess whether hemicortical excision and biological reconstruction instead of the conventional wide resection for selected cases of malignant surface tumors, may give adequate oncologic clearance with less morbidity and better function. Methods: From January 2000 to June 2007 a total of 29 malignant surface tumors were managed at our institute. Fifteen of these were treated with hemicortical excision and reconstruction. They consisted of 10 parosteal osteosarcomas, 1 periosteal osteosarcoma, 1 high grade surface osteosarcoma, 1 adamantinoma and 2 surface chondrosarcomas. Nine were in the distal femur, 3 in tibia, 2 in the humerus and 1 in the radius. Four of these were residual/recurrent lesions following earlier intervention. After hemicortical excision with adequate margins the bone defect was reconstructed with allograft/autograft and suitable internal fixation where indicated. Results: Margins were reported free in all cases. There were no infections. Three of five patients who did not have fixation with a plate at index surgery sustained a subsequent fracture which was then reduced and fixed. Follow up duration was from 24 to 90 months. Two patients had isolated soft tissue recurrences. Both underwent re excision and are currently disease free at 36 and 38 months respectively after recurrence. Eleven patients had a maximum possible score of 30 (MSTS functional scoring); other four had a score of 29. None of the cases have developed distant metastasis. Discussion: Early results indicate that in selected cases, hemicortical excision is an oncologically sound procedure. The ability to do a biological reconstruction with bone helps avoid some of the serious complications of a megaprosthetic reconstruction while permitting full active loading of the extremity and near normal function. Internal fixation at index surgery permits early mobilization and minimises the incidence of subsequent fractures

Introduction: Parosteal osteosarcoma is a rare, mostly low grade tumor arising from the surface of the bone. The aim of the study was to establish risk factors for local recurrence (LR) and death. Method: Retrospective review of a prospectively collected database with complete up-to-date follow up. Results: 63 patients were diagnosed and treated at our centre from 1978 to 2007. The most common site was the femur (37) followed by the tibia (10) and humerus (9). The median size was 7cm (2 – 16). At the time of diagnosis 27 (43%) were low grade, 13 (21%) were low grade but had invaded the medullary canal and 8 (13%) had a high grade component; the data was missing in 15 patients (24%). All patients were operated. LR arose in 11 patients (17.5%) at a mean of 38 months and was related to surgical margins. There were no LR in the 26 wide resections, but the risk of LR was 20% in those with a marginal and 46% in those with an intralesional excision. LR appeared as a higher grade than the primary tumor in 3 patients (27%). 9 (14.3%) patients developed metastases at a median of 22 months (6 – 123). Five patients developed both LR and metastases. Seven patients died from the tumor, 2 patients are alive with metastatic disease. None of the patients with a low grade tumor and wide or marginal margins died of tumor, but 2 patients (18%) operated intralesional died of tumor. Discussion: There was a statistically significant relationship between surgical margins and LR (p< 0.001). All patients with low grade parosteal osteosarcoma operated with wide margins have been cured. LR appeared often in higher grade than the primary tumor. Patients with low grade tumor and intralesional margins had 18% risk to tumor-ralated death