Lessons Learned: Patient Communication During the Pandemic.

A series of mortalities among musculoskeletal tumour patients secondary to medical illnesses during the first few months of the pandemic highlighted the need to review our methods of communication with patients. Prominent among patients' concerns had been a fear of consulting at hospitals and a lack of ready access to health care. Recommendations are made for proactive consultation and patient education, identifying at-risk patients for follow-up and probing for possible co-morbidities. Telemedicine use is encouraged bearing in mind its inherent limitations. A network of physicians and pharmaceutical representatives is an added help we can offer our patients who may be isolated by community quarantine.

The use of a non-invasive extendable prosthesis at the time of revision arthroplasty.

Aims: The use of a noninvasive growing endoprosthesis in the management of primary bone tumours in children is well established. However, the efficacy of such a prosthesis in those requiring a revision procedure has yet to be established. The aim of this series was to present our results using extendable prostheses for the revision of previous endoprostheses. Patients and Methods: All patients who had a noninvasive growing endoprosthesis inserted at the time of a revision procedure were identified from our database. A total of 21 patients (seven female patients, 14 male) with a mean age of 20.4 years (10 to 41) at the time of revision were included. The indications for revision were mechanical failure, trauma or infection with a residual leg-length discrepancy. The mean follow-up was 70 months (17 to 128). The mean shortening prior to revision was 44 mm (10 to 100). Lengthening was performed in all but one patient with a mean lengthening of 51 mm (5 to 140). Results: The mean residual leg length discrepancy at final follow-up of 15 mm (1 to 35). Two patients developed a deep periprosthetic infection, of whom one required amputation to eradicate the infection; the other required two-stage revision. Implant survival according to Henderson criteria was 86% at two years and 72% at five years. When considering revision for any cause (including revision of the growing prosthesis to a non-growing prosthesis), revision-free implant survival was 75% at two years, but reduced to 55% at five years. Conclusion: Our experience indicates that revision surgery using a noninvasive growing endoprosthesis is a successful option for improving leg length discrepancy and should be considered in patients with significant leg-length discrepancy requiring a revision procedure. Cite this article: Bone Joint J 2018;100-B:370-7.

Risk analysis factors for local recurrence in Ewing's sarcoma: when should adjuvant radiotherapy be administered?

AIMS: The aim of this study was to analyse a group of patients with non-metastatic Ewing's sarcoma at presentation and identify prognostic factors affecting the development of local recurrence, in order to assess the role of radiotherapy. PATIENTS AND METHODS: A retrospective review of all patients with a Ewing's sarcoma treated between 1980 and 2012 was carried out. Only those treated with chemotherapy followed by surgery and/or radiotherapy were included. Patients were grouped according to site (central or limb) for further analysis of the prognostic factors. RESULTS: A total of 388 patients were included in the study. Of these, 60 (15%) developed local recurrence at a mean median of 27 months (sd 24, range 7 to 150) and the five-year local recurrence-free survival (5yrLRFS) was 83%. For central tumours, the size of the tumour and histological response to chemotherapy were found to be significant factors for local recurrence. For limb tumours, local recurrence was affected by intralesional and marginal resections, but not by the histological response to chemotherapy. Radiotherapy in those with a marginal resection reduced the risk of local recurrence (5yrLRFS: 96% versus 81%, p = 0.044). CONCLUSION: Local recurrence significantly affects the overall survival in patients with a Ewing's sarcoma. For those with a tumour in a limb, radiotherapy reduced the risk of local recurrence, especially in those with a marginal margin of excision, but the effect in central tumours was less clear. Radiotherapy for those who have had a wide margin of resection does not reduce the risk of local recurrence, regardless of the histological response to chemotherapy. Cite this article: Bone Joint J 2018;100-B: 247-55.

What is the morbidity of a non-invasive growing prosthesis?

AIMS: Extendible endoprostheses have been available for more than 30 years and have become more sophisticated with time. The latest generation is 'non-invasive' and can be lengthened with an external magnetic force. Early results have shown a worryingly high rate of complications such as infection. This study investigates the incidence of complications and the need for further surgery in a cohort of patients with a non-invasive growing endoprosthesis. PATIENTS AND METHODS: Between 2003 and June 2014, 50 children (51 prostheses) had a non-invasive growing prosthesis implanted for a primary bone sarcoma. The minimum follow-up was 24 months for those who survived. Their mean age was 10.4 years (6 to 14). The incidence of complications and further surgery was documented. RESULTS: The mean follow-up was 64 months (20 to 145). The overall survivorship of the patients was 84% at three years and 70% at five years. Revision-free survival was 81.7% at three years and 61.6% at five years with competing risk analysis. Deep infection occurred in 19.6% of implants at a mean of 12.5 months (0 to 55). Other complications were a failure of the lengthening mechanism in five prostheses (9.8%) and breakage of the implant in two (3.9%). Overall, there were 53 additional operations (0 to 5 per patient). A total of seven patients (14%) underwent amputation, three for local recurrence and four for infection. Their mean limb length discrepancy was 4.3 mm (0 to 25) and mean Musculoskeletal Tumor Society Score functional score was 26.5 (18 to 30) at the final follow-up. CONCLUSIONS: When compared with previously published early results, this mid-term series has shown continued good functional outcomes and compensation for leg-length discrepancy. Infection is still the most common complication: post-operative wound healing problems, central line infection and proximal tibial location are the main risk factors. Cite this article: Bone Joint J 2016;98-B:1697-1703.

Ewing's sarcoma: only patients with 100% of necrosis after chemotherapy should be classified as having a good response.

AIMS: The purpose of this study was to review a large cohort of patients and further assess the correlation between the histological response to chemotherapy in patients with Ewing's sarcoma with the overall (OS) and event-free survival (EFS). PATIENTS AND METHODS: All patients treated for Ewing's sarcoma between 1980 and 2012 were reviewed. Of these, 293 patients without metastases at the time of diagnosis and treated with chemotherapy and surgery were included. Patients were grouped according to the percentage of necrosis after chemotherapy: Group I: 0% to 50%, Group II: 51% to 99% and Group III: 100%. RESULTS: The mean age at diagnosis was 16 years (1 to 62) and the mean follow-up was 9.1 years (six months to 32.6 years). The OS and EFS for the series were 75% and 65% at five years. There were significant differences in survival between the groups of necrosis: 0% to 50% (OS: 49% and EFS: 45% at five years, respectively) compared with 51% to 99% (OS: 72% and EFS: 59% at five years, respectively) and 100% (OS: 94% and EFS: 81% at five years, respectively) (p < 0.001). There were no significant differences in survival between patients treated between 1980 and 1989 compared with those treated between 1990 and 1999, and those treated between 2000 and 2012 (p = 0.55). CONCLUSION: Only patients with 100% necrosis after chemotherapy should be classified as having a good response to chemotherapy because they have significantly better rates of survival compared with those with any viable tumour in the surgical specimen. Cite this article: Bone Joint J 2016;98-B:1138-44.

Osteosarcoma of the pelvis.

AIMS: Osteosarcoma of the pelvis is a particularly difficult tumour to treat as it often presents late, may be of considerable size and/or associated with metastases when it presents, and is frequently chondroid in origin and resistant to chemotherapy. The aim of this study was to review our experience of managing this group of patients and to identify features predictive of a poor outcome. PATIENTS AND METHODS: Between 1983 and 2014, 121 patients, (74 females and 47 males) were treated at a single hospital: 74 (61.2%) patients had a primary osteosarcoma and 47 (38.8%) had an osteosarcoma which was secondary either to Paget's disease (22; 18.2%) or to previous pelvic irradiation (25; 20.7%). The mean age of those with a primary osteosarcoma was 29.3 years (nine to 76) and their mean follow-up 2.9 years (0 to 29). The mean age of those with a secondary sarcoma was 61.9 years (15 to 85) and their mean follow-up was one year (0 to 14). A total of 22 patients with a primary sarcoma (52.4%) and 20 of those with a secondary sarcoma (47.6%) had metastases at the time of presentation. RESULTS: The disease-specific survival at five years for all patients was 27.2%. For those without metastases at the time of diagnosis, the five-year survival was 32.7%. Factors associated with a poor outcome were metastases at diagnosis and secondary tumours. In primary osteosarcoma, sacral location, surgical margin and a diameter > 10 cm were associated with a poor outcome. CONCLUSION: In this, the largest single series of patients with an osteosarcoma of the pelvis treated in a single hospital, those with secondary tumours and those with metastases at presentation had a particularly poor outcome. For those with a primary sarcoma, sacral location, an intralesional margin and a diameter of > 10 cm were poor prognostic indicators.

Does amputation offer any survival benefit over limb salvage in osteosarcoma patients with poor chemonecrosis and close margins?

A poor response to chemotherapy (≤ 90% necrosis) for osteosarcomas leads to poorer survival and an increased risk of local recurrence, particularly if there is a close margin of excision. We evaluated whether amputation confers any survival benefit over limb salvage surgery (LSS) with narrow margins in patients who respond poorly to chemotherapy. We only analysed patients with an osteosarcoma of the limb, a poor response to chemotherapy and close margins on LSS (marginal/intralesional) or primary amputation: 360 patients (36 LSS (intralesional margins), 197 LSS (marginal margins) and 127 amputations) were included. Local recurrence developed in 13 (36%) following LSS with intralesional margins, and 39 (20%) following LSS with marginal margins. There was no local recurrence in patients who underwent amputation. The five-year survival for all patients was 41% (95% confidence interval (CI) 35 to 46), but for those treated by LSS with marginal margins was 46.2% (95% CI 38 to 53), 36.3% (95% CI 27 to 45) for those treated by amputation, and 28% (95 CI 14 to 44) for those treated by LSS with intralesional margins. Patients who had LSS and then developed local recurrence as a first event had the same survival as those who had primary amputation without local recurrence. Prophylactic adjuvant radiotherapy was used in 40 patients but had no discernible effect in preventing local recurrence. Although amputation offered better local control, it conferred no clear survival benefit over LSS with marginal margins in these patients with a poor overall prognosis.

Diaphyseal osteosarcomas have distinct clinical features from metaphyseal osteosarcomas.

AIMS: The aim of this study was to clarify the clinical features and outcomes of diaphyseal osteosarcoma. METHODS: Patients with newly-diagnosed high-grade osteosarcoma occurring in the long bone were eligible for this retrospective study. Clinicopathological information was collected from our database and compared with 36 diaphyseal, 405 proximal and 519 distal metaphyseal, and 14 whole bone osteosarcoma patients. Additionally, case-control study matching by age, gender, site, and metastatic status at diagnosis with 1:3 ratio of 36 diaphyseal to 108 metaphyseal osteosarcomas patients was also conducted. RESULTS: Five-year overall survival and metastasis-free survival of the three groups including diaphyseal, metaphyseal, and whole bone osteosarcoma patients showed significant difference (P = .029 and P = .013, respectively), although there is no difference for the survivals between proximal and distal metaphyseal osteosarcoma patients. Case-control study showed that patients with diaphyseal osteosarcomas had a significantly larger tumour (mean 13.5 cm vs 10 cm, P = .026), and demonstrated higher pathologic fracture rate (28% vs 12%, P = .033), superior 5-year metastasis-free survival (74% vs 40%, P = .0068), and slightly better 5-year overall survival (68% vs 46%, P = .074). Prognostic factor analysis showed that a pathologic fracture significantly decreased the survival of the patients with diaphyseal osteosarcoma. CONCLUSIONS: The current study showed that diaphyseal osteosarcoma has distinct clinical features from metaphyseal osteosarcoma having an increased risk of pathologic fractures but with favorable survival outcome.

Is limb salvage surgery safe for bone sarcomas identified after a previous surgical procedure?

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.

The prognostic value of the serum level of C-reactive protein for the survival of patients with a primary sarcoma of bone.

The aim of this study was to determine whether the level of circulating C-reactive protein (CRP) before treatment predicted overall disease-specific survival and local tumour control in patients with a sarcoma of bone. We retrospectively reviewed 318 patients who presented with a primary sarcoma of bone between 2003 and 2010. Those who presented with metastases and/or local recurrence were excluded. Elevated CRP levels were seen in 84 patients before treatment; these patients had a poorer disease-specific survival (57% at five years) than patients with a normal CRP (79% at five years) (p < 0.0001). They were also less likely to be free of recurrence (71% at five years) than patients with a normal CRP (79% at five years) (p = 0.04). Multivariate analysis showed the pre-operative CRP level to be an independent predictor of survival and local control. Patients with a Ewing's sarcoma or chondrosarcoma who had an elevated CRP before their treatment started had a significantly poorer disease-specific survival than patients with a normal CRP (p = 0.02 and p < 0.0001, respectively). Patients with a conventional osteosarcoma and a raised CRP were at an increased risk of poorer local control. We recommend that CRP levels are measured routinely in patients with a suspected sarcoma of bone as a further prognostic indicator of survival.

Does the addition of cement improve the rate of local recurrence after curettage of giant cell tumours in bone?

We retrospectively compared the outcome after the treatment of giant cell tumours of bone either with curettage alone or with adjuvant cementation. Between 1975 and 2008, 330 patients with a giant cell tumour were treated primarily by intralesional curettage, with 84 (25%) receiving adjuvant bone cement in the cavity. The local recurrence rate for curettage alone was 29.7% (73 of 246) compared with 14.3% (12 of 84) for curettage and cementation (p = 0.001). On multivariate analysis both the stage of disease and use of cement were independent significant factors associated with local recurrence. The use of cement was associated with a higher risk of the subsequent need for joint replacement. In patients without local recurrence, 18.1% (13 of 72) of those with cement needed a subsequent joint replacement compared to 2.3% (4 of 173) of those without cement (p = 0.001). In patients who developed local recurrence, 75.0% (9 of 12) of those with previous cementation required a joint replacement, compared with 45.2% (33 of 73) of those without cement (p = 0.044).

Distal femoral physeal growth arrest secondary to a cemented proximal femoral endoprosthetic replacement.

We report a case of spontaneous physeal growth arrest of the distal femur in a nine-year-old child with Ewing's sarcoma of the proximal femur treated with chemotherapy and endoprosthetic replacement. Owing to the extent of disuse osteoporosis at the time of surgery, the entire intramedullary canal up to the distal femoral physis was filled with cement. Three years later, the femur remained at its pre-operative length of 19 cm. Pre-operative calculations of further growth failed to account for the growth arrest, and the initial expandable growing prosthesis inserted has been revised to a longer one in order to address the leg-length discrepancy. To our knowledge, this is the only reported case of distal femoral physeal growth arrest following cemented endoprosthetic replacement of the proximal femur.