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1.
Br J Cancer ; 118(12): 1682, 2018 06.
Artigo em Inglês | MEDLINE | ID: mdl-29808016

RESUMO

Since the publication of this paper, the authors noticed an error in Fig. 1. The X-axis on all the figure panels should read 'Time (years)', not 'Time (months)'. The corrected Fig. 1 is shown below.

2.
Bone Joint J ; 100-B(5): 652-661, 2018 05 01.
Artigo em Inglês | MEDLINE | ID: mdl-29701086

RESUMO

Aims: 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. Patients and Methods: In a retrospective analysis of a sarcoma database at a single institution between 1990 and 2014, we identified 1446 patients with non-metastatic and 346 with metastatic bone sarcoma. Low-grade types of tumour were excluded. Our data included the demographics of the patients, the characteristics of the tumour, and the survival outcome of patients. Cox proportional hazards analysis and Kaplan-Meier survival analysis were performed, and the survivorship of the non-metastatic and metastatic cohorts were compared. Results: In the non-metastatic cohort, a longer DOS was associated with a slightly more favourable survival (hazard ratio (HR) 0.996, 95% confidence interval (CI) 0.994 to 0.998, p < 0.001). In all types of tumour, there was no difference in survival between patients with a DOS of greater than four months and those with a DOS of less than four months (p = 0.566). There was no correlation between the year of diagnosis and survival (p = 0.741). A diagnosis of chondrosarcoma (HR 0.636, 95% CI 0.474 to 0.854, p = 0.003) had the strongest positive effect on survival, while location in the axial skeleton (HR 1.76, 95% CI 1.36 to 2.29, p < 0.001) had the strongest negative effect on survival. Larger size of tumour (HR 1.05, 95% CI 1.03 to 1.06, p < 0.001) and increased age of the patient (HR 1.02, 95% CI 1.01 to 1.03, p < 0.001) had a slightly negative effect on survival. Metastatic and non-metastatic cohorts had similar median DOS (16 weeks, p = 0.277), although the median survival (15.5 months vs 41 months) and rates of survival at one year (69% vs 89%) and five years (20% vs 59%) were significantly shorter in the metastatic cohort. Conclusion: A longer DOS prior to diagnosis is not associated with a poorer overall survival in patients with a primary bone sarcoma. Location in the axial skeleton remains the strongest predictor of a worse prognosis. This may be helpful in counselling patients referred for evaluation on a delayed basis. Cite this article: Bone Joint J 2018;100-B:652-61.


Assuntos
Neoplasias Ósseas/diagnóstico , Neoplasias Ósseas/mortalidade , Diagnóstico Tardio , Sarcoma/diagnóstico , Sarcoma/mortalidade , Adolescente , Adulto , Neoplasias Ósseas/cirurgia , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos , Sarcoma/cirurgia , Análise de Sobrevida , Fatores de Tempo , Adulto Jovem
3.
Bone Joint J ; 100-B(5): 662-666, 2018 05 01.
Artigo em Inglês | MEDLINE | ID: mdl-29701096

RESUMO

Aims: The purpose of this study was to describe the effect of histological grade on disease-specific survival in patients with chondrosarcoma. Patients and Methods: A total of 343 patients with a chondrosarcoma were included. The histological grade was assessed on the initial biopsy and on the resection specimen. Where the histology showed a mixed grade, the highest grade was taken as the definitive grade. When only small focal areas showed higher grade, the final grade was considered as both. Results: The concordance between the highest preoperative biopsy grading and the highest final grading of the resection specimen in total was only 43% (146/343). In 102 specimens (30%), a small number of cells or focal areas of higher grade were observed in contrast to the main histology. The disease-specific survival, stratified according to the predominant histological grade, showed greater variation than when stratified according to the highest grade seen in the resection specimen. Conclusion: The diagnostic biopsy in chondrosarcoma is unreliable in assessing the definitive grade and the malignant potential of the tumour. When categorizing the grade of the resection specimen, the prognosis for local recurrence and disease-specific survival should be based on the highest grade seen, even when seen in only a few cells. Cite this article: Bone Joint J 2018;100-B:662-6.


Assuntos
Neoplasias Ósseas/patologia , Condrossarcoma/patologia , Recidiva Local de Neoplasia/patologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Biópsia , Neoplasias Ósseas/mortalidade , Neoplasias Ósseas/cirurgia , Criança , Condrossarcoma/mortalidade , Condrossarcoma/cirurgia , Intervalo Livre de Doença , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Recidiva Local de Neoplasia/mortalidade , Estudos Retrospectivos , Adulto Jovem
4.
Bone Joint J ; 100-B(3): 370-377, 2018 03 01.
Artigo em Inglês | MEDLINE | ID: mdl-29589498

RESUMO

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.


Assuntos
Neoplasias Ósseas/cirurgia , Perna (Membro) , Próteses e Implantes , Implantação de Prótese/métodos , Adolescente , Adulto , Amputação Cirúrgica , Criança , Feminino , Humanos , Desigualdade de Membros Inferiores , Masculino , Complicações Pós-Operatórias , Estudos Prospectivos , Desenho de Prótese , Reoperação , Resultado do Tratamento
5.
Bone Joint J ; 100-B(2): 247-255, 2018 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-29437069

RESUMO

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.


Assuntos
Neoplasias Ósseas/radioterapia , Recidiva Local de Neoplasia/patologia , Radioterapia Adjuvante , Sarcoma de Ewing/radioterapia , Adolescente , Adulto , Neoplasias Ósseas/tratamento farmacológico , Neoplasias Ósseas/cirurgia , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos , Fatores de Risco , Sarcoma de Ewing/tratamento farmacológico , Sarcoma de Ewing/cirurgia , Taxa de Sobrevida , Resultado do Tratamento
6.
Eur J Surg Oncol ; 43(9): 1746-1752, 2017 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-28756018

RESUMO

AIMS: To investigate the impact of the method of treatment on the oncological outcomes in patients with epithelioid sarcomas managed at two international speciality sarcoma centres. METHODS: The databases of two centres were used to identify patients treated for epithelioid sarcomas between 1985 and 2012. Patient, tumor, treatment and outcome data was collected. RESULTS: There were 36 males and 18 females with a mean age of 38.3 years (range 9-79). Of 49 patients who were treated surgically, limb salvage surgery was carried out in 38 patients (78%) and limb amputation in 11 (22%). Of 49 total patients who underwent surgery for ES, 48 (98%) with ES had negative margin resection and 24 (49%) received (neo) adjuvant radiotherapy. Regional lymph node metastases developed in 5 (13%) patients. The five-year risk of local recurrence was 14%. The overall survival rate at five and ten years was 70% and 66% respectively. In multivariate analysis of patients with localized disease and negative margins, survival and risk of metastases was worse in those treated by amputation. CONCLUSION: This series has shown that although the rate of local recurrence is not influenced by the type of surgery, the risk of metastases is higher following amputation. This finding is likely due to patients with larger, deeper and more locally advanced tumors requiring amputation. However, we could not prove that immediate amputation was likely to affect overall survival.


Assuntos
Amputação Cirúrgica , Recidiva Local de Neoplasia , Tratamentos com Preservação do Órgão , Sarcoma/cirurgia , Adolescente , Adulto , Idoso , Criança , Intervalo Livre de Doença , Extremidades , Feminino , Humanos , Metástase Linfática , Masculino , Margens de Excisão , Pessoa de Meia-Idade , Neoplasia Residual , Radioterapia Adjuvante , Estudos Retrospectivos , Sarcoma/radioterapia , Sarcoma/secundário , Taxa de Sobrevida , Carga Tumoral , Adulto Jovem
7.
Br J Cancer ; 117(3): 326-331, 2017 Jul 25.
Artigo em Inglês | MEDLINE | ID: mdl-28654633

RESUMO

BACKGROUND: An increasing number and proportion of cancer patients with apparently localised disease are treated with chemotherapy and radiation therapy in contemporary oncology practice. In a pilot study of radiation-induced sarcoma (RIS) patients, we demonstrated that chemotherapy was associated with a reduced time to development of RIS. We now present a multi-centre collaborative study to validate this association. METHODS: This was a retrospective cohort study of RIS cases across five large international sarcoma centres between 1 January 2000 to 31 December 2014. The primary endpoint was time to development of RIS. RESULTS: We identified 419 patients with RIS. Chemotherapy for the first malignancy was associated with a shorter time to RIS development (HR 1.37; 95% CI: 1.08-1.72; P=0.009). In the multi-variable model, older age (HR 2.11; 95% CI 1.83-2.43; P<0.001) and chemotherapy for the first malignancy (HR 1.61; 95% CI 1.26-2.05; P<0·001) were independently associated with a shorter time to RIS. Anthracyclines and alkylating agents significantly contribute to the effect. CONCLUSIONS: This study confirms an association between chemotherapy given for the first malignancy and a shorter time to development of RIS.


Assuntos
Antineoplásicos Alquilantes/efeitos adversos , Antineoplásicos/efeitos adversos , Neoplasias Induzidas por Radiação/etiologia , Segunda Neoplasia Primária/etiologia , Sarcoma/etiologia , Adolescente , Adulto , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Antraciclinas/efeitos adversos , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Neoplasias/tratamento farmacológico , Neoplasias/radioterapia , Estudos Retrospectivos , Fatores de Tempo , Adulto Jovem
8.
Bone Joint J ; 99-B(4): 538-543, 2017 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-28385945

RESUMO

AIMS: The aim of the study was to investigate the controversial issue of whether the pelvic ring should be reconstructed following resection of the sarcomas of the ilium. PATIENTS AND METHODS: From our database, we identified 64 patients who underwent excision of a tumour involving the ilium between 1976 and 2015. A total of 35 underwent complete resection, of whom 24 were reconstructed with a non-vascularised fibula graft, and four with extracorporeal irradiation and reimplantation. A total of 29 patients had a partial resection. The mean follow-up was 9.2 years (1.1 to 25.6). Functional outcomes were assessed using the Toronto Extremity Salvation Score (TESS) at final follow-up. In all, 32 patients (50%) had a chondrosarcoma. RESULTS: The mean TESS for all patients was 71.6% (17% to 100%). The mean TESS for those who underwent total resection with reconstruction was 72.0% (17% to 100%) and without reconstruction it was 53.3% (20% to 90%) and for those who underwent partial resection it was 76.3% (31.3% to 100%). The rate of local recurrence was 42.2% and this was more common in those treated by partial resection (p = 0.048). The risk of local recurrence was related to the margin achieved at resection. CONCLUSIONS: Given the high rate of local recurrence following excision of a tumour from the ilium, obtaining wide surgical margins should be a priority even if this requires more aggressive surgery. In young patients, where late recurrence may occur, more radical complete resection should be considered. When total resection of the ilium is considered, reconstruction should also be considered as it confers a higher functional outcome than total resection without reconstruction. Cite this article: Bone Joint J 2017;99-B:538-43.


Assuntos
Neoplasias Ósseas/cirurgia , Ílio/cirurgia , Ossos Pélvicos/cirurgia , Sarcoma/cirurgia , Adulto , Neoplasias Ósseas/diagnóstico por imagem , Neoplasias Ósseas/reabilitação , Transplante Ósseo/métodos , Estudos de Casos e Controles , Condrossarcoma/diagnóstico por imagem , Condrossarcoma/reabilitação , Condrossarcoma/cirurgia , Feminino , Seguimentos , Humanos , Ílio/diagnóstico por imagem , Masculino , Margens de Excisão , Recidiva Local de Neoplasia , Osteossarcoma/diagnóstico por imagem , Osteossarcoma/reabilitação , Osteossarcoma/cirurgia , Ossos Pélvicos/diagnóstico por imagem , Radiografia , Recuperação de Função Fisiológica , Sarcoma/diagnóstico por imagem , Sarcoma/reabilitação
9.
Bone Joint J ; 99-B(3): 409-416, 2017 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-28249983

RESUMO

AIMS: The aim of this study was to identify any progression between benign osteofibrous dysplasia (OFD), OFD-like adamantinoma and malignant adamantinoma, and to investigate the rates of local recurrence, metastases and survival, in order to develop treatment algorithms for each. PATIENTS AND METHODS: A single institution retrospective review of all patients presenting with OFD, OFD-like adamantinoma and adamantinoma between 1973 and 2012 was undertaken. Complete data were available for 73 patients (42 with OFD; ten with an OFD-like adamantinoma and 21 with an adamantinoma). The mean follow-up was 10.3 years (3 to 25) for OFD, 9.2 years (3.0 to 26.3) for OFD-like and 11.6 years (0.25 to 33) for adamantinoma. RESULTS: The mean age at diagnosis for OFD was 13.5 years (1 to 49), 10.5 years (6 to 28) for OFD-like and 34 years (14 to 86) for adamantinoma. A total of 24 of the 42 patients with OFD (57%) have not required any treatment and have been managed with observation. A total of 18 of the 42 patients with OFD underwent surgery, 13 with curettage and five with resection. In all, three patients developed recurrence following curettage (23%) but none following resection. All these patients were cured with further limited surgery. A total of six patients initially diagnosed with OFD were subsequently found to have OFD-like adamantinoma. Of the ten patients initially diagnosed with OFD-like adamantinoma, three (30%) were managed with observation alone and seven underwent surgery, two with curettage and five with resection. Local recurrence arose in two patients, one each after curettage and resection. No patients with either OFD or an OFD-like adamantinoma developed metastases or had progression to adamantinoma. All patients with an adamantinoma were treated by surgery, three with curettage, six with amputation and 12 with excision. In all, two of the three treated with curettage developed local recurrence, requiring further surgery. Late development of both local recurrence and metastases led to a ten year disease specific survival of 93% which had dropped to 39% by 20 years. CONCLUSION: We found no evidence of progression from OFD to adamantinoma. Conservative management with observation or curettage is often successful for patients with OFD and OFD-like adamantinoma. Resection with clear margins is required for patients with adamantinoma. Late tumour recurrence is not uncommon in adamantinoma and prolonged follow-up should be considered. Cite this article: Bone Joint J 2017;99-B:409-16.


Assuntos
Adamantinoma/diagnóstico , Doenças do Desenvolvimento Ósseo/diagnóstico , Adamantinoma/secundário , Adamantinoma/terapia , Adolescente , Doenças do Desenvolvimento Ósseo/terapia , Transformação Celular Neoplásica , Criança , Pré-Escolar , Progressão da Doença , Feminino , Seguimentos , Humanos , Lactente , Estimativa de Kaplan-Meier , Masculino , Recidiva Local de Neoplasia , Radiografia , Estudos Retrospectivos , Adulto Jovem
10.
Bone Joint J ; 99-B(2): 261-266, 2017 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-28148671

RESUMO

AIMS: Due to the complex anatomy of the pelvis, limb-sparing resections of pelvic tumours achieving adequate surgical margins, can often be difficult. The advent of computer navigation has improved the precision of resection of these lesions, though there is little evidence comparing resection with or without the assistance of navigation. Our aim was to evaluate the efficacy of navigation-assisted surgery for the resection of pelvic bone tumours involving the posterior ilium and sacrum. PATIENTS AND METHODS: Using our prospectively updated institutional database, we conducted a retrospective case control study of 21 patients who underwent resection of the posterior ilium and sacrum, for the treatment of a primary sarcoma of bone, between 1987 and 2015. The resection was performed with the assistance of navigation in nine patients and without navigation in 12. We assessed the accuracy of navigation-assisted surgery, as defined by the surgical margin and how this affects the rate of local recurrence, the disease-free survival and the effects on peri-and post-operative morbidity. RESULTS: The mean age of the patients was 36.4 years (15 to 66). The mean size of the tumour was 10.9 cm. In the navigation-assisted group, the margin was wide in two patients (16.7%), marginal in six (66.7%) and wide-contaminated in one (11.1%) with no intralesional margin. In the non-navigated-assisted group; the margin was wide in two patients (16.7%), marginal in five (41.7%), intralesional in three (25.0%) and wide-contaminated in two (16.7%). Local recurrence occurred in two patients in the navigation-assisted group (22.2%) and six in the non-navigation-assisted group (50.0%). The disease-free survival was significantly better when operated with navigation-assistance (p = 0.048). The blood loss and operating time were less in the navigated-assisted group, as was the risk of a foot drop post-operatively. CONCLUSION: The introduction of navigation-assisted surgery for the resection of tumours of the posterior ilium and sacrum has increased the safety for the patients and allows for a better oncological outcome. Cite this article: Bone Joint J 2017;99-B:261-6.


Assuntos
Neoplasias Ósseas/cirurgia , Ílio/cirurgia , Ossos Pélvicos/cirurgia , Sacro/cirurgia , Sarcoma/cirurgia , Cirurgia Assistida por Computador , Adolescente , Adulto , Idoso , Neoplasias Ósseas/diagnóstico por imagem , Neoplasias Ósseas/patologia , Estudos de Casos e Controles , Feminino , Humanos , Ílio/diagnóstico por imagem , Ílio/patologia , Masculino , Pessoa de Meia-Idade , Recidiva Local de Neoplasia/patologia , Ossos Pélvicos/diagnóstico por imagem , Ossos Pélvicos/patologia , Estudos Retrospectivos , Sacro/diagnóstico por imagem , Sacro/patologia , Sarcoma/diagnóstico por imagem , Sarcoma/patologia , Análise de Sobrevida , Resultado do Tratamento , Adulto Jovem
11.
Bone Joint J ; 98-B(12): 1697-1703, 2016 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-27909134

RESUMO

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.


Assuntos
Alongamento Ósseo/instrumentação , Neoplasias Ósseas/cirurgia , Próteses e Implantes , Implantação de Prótese/efeitos adversos , Sarcoma/cirurgia , Adolescente , Distribuição por Idade , Alongamento Ósseo/métodos , Neoplasias Ósseas/patologia , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Estimativa de Kaplan-Meier , Masculino , Fraturas Periprotéticas/etiologia , Próteses e Implantes/efeitos adversos , Desenho de Prótese , Falha de Prótese , Implantação de Prótese/métodos , Infecções Relacionadas à Prótese/etiologia , Reoperação/métodos , Sarcoma/patologia , Distribuição por Sexo
12.
Acta Orthop Belg ; 82(2): 216-221, 2016 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-27682283

RESUMO

We are reporting our experience on patients with -pelvic Ewing's Sarcoma treated in our unit. We retrospectively reviewed a series of patients with non-metastatic pelvic Ewing's sarcoma treated between 1977 and 2009. Patients were classified into three groups according to the local treatment received : Group 1. radiotherapy-chemo ; Group 2. surgery-chemo and Group 3. radiotherapy-surgery-chemo. Recurrence free and overall survival rates were calculated using the Kaplan-Meier method. Influence of various factors (age at diagnosis, gender, tumour site and size, chemotherapy response, surgical margins and type of treatment) on survival was assessed with a logistic regression model. A total of 85 patients were treated with a mean follow-up of 65.8 months and mean -tumour volume of 435ml. The 5-year survival for all patients was 40.7% decreased to 36.2% at 10 years. A significant prognostic factor identified was chemotherapy response only. There was a trend for improved survival and local control rates for patients who had chemotherapy and surgery and the results were apparent for all tumours irrespective of size but not statistically significant. Currently, the optimal management of pelvic Ewing's sarcoma is contro-versial but our study shows a trend for improved -survival for patients treated with chemotherapy and surgery.


Assuntos
Neoplasias Ósseas/mortalidade , Neoplasias Ósseas/terapia , Ossos Pélvicos , Sarcoma de Ewing/mortalidade , Sarcoma de Ewing/terapia , Adolescente , Adulto , Neoplasias Ósseas/patologia , Criança , Pré-Escolar , Terapia Combinada , Intervalo Livre de Doença , Feminino , Humanos , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Recidiva Local de Neoplasia , Estudos Retrospectivos , Sarcoma de Ewing/patologia , Adulto Jovem
13.
Bone Joint J ; 98-B(8): 1138-44, 2016 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-27482030

RESUMO

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.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Neoplasias Ósseas/tratamento farmacológico , Sarcoma de Ewing/tratamento farmacológico , Adolescente , Adulto , Neoplasias Ósseas/mortalidade , Neoplasias Ósseas/cirurgia , Criança , Pré-Escolar , Intervalo Livre de Doença , Feminino , Humanos , Lactente , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Necrose , Neoplasias Pélvicas/tratamento farmacológico , Neoplasias Pélvicas/mortalidade , Neoplasias Pélvicas/cirurgia , Estudos Prospectivos , Sarcoma de Ewing/mortalidade , Sarcoma de Ewing/cirurgia , Neoplasias da Coluna Vertebral/tratamento farmacológico , Neoplasias da Coluna Vertebral/mortalidade , Neoplasias da Coluna Vertebral/cirurgia , Neoplasias Torácicas/tratamento farmacológico , Neoplasias Torácicas/mortalidade , Resultado do Tratamento , Adulto Jovem
14.
Bone Joint J ; 98-B(6): 857-64, 2016 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-27235533

RESUMO

AIMS: The aim of this study was to establish what happens to patients in the long term after endoprosthetic replacement for a primary malignant tumour of bone. PATIENTS AND METHODS: We conducted a retrospective analysis of a prospectively maintained database to identify all patients who had undergone an endoprosthetic replacement more than 25 years ago and who were still alive. Their outcomes were investigated with reference to their complications and need for further surgery. A total of 230 patients were identified. Their mean age at diagnosis was 20.7 years (five to 62). The most common diagnosis was osteosarcoma (132). The most common site was the distal femur (102). RESULTS: The mean follow-up was 29.4 years (25 to 43). A total of 610 further operations were undertaken, an average of 2.7 further operations per patient. A total of 42 patients (18%) still had the original prosthesis in place. The risk of amputation was 16% at 30 years (31 patients). Those without infection had a mean of 2.1 further operations (one to nine) while those with infection had a mean of 4.6 further operations (two to 11). The risk of infection persisted throughout the life of the prosthesis with a mean of 1% per year becoming infected. Of the 60 patients who developed an infection, 21 (35%) developed this following the primary procedure at a mean of 50 months, but another 19 developed this within a year of another surgical procedure. The risk of infection after any further surgery was 2.7%. The site with the highest risk of infection was the proximal tibia (43.3%). TAKE HOME MESSAGE: This study highlights the inevitable need for further surgery following first-generation endoprosthetic reconstruction, although in most cases, limb salvage is maintained. Late complications, especially infection, continue for the lifetime of the implant. Cite this article: Bone Joint J 2016;98-B:857-64.


Assuntos
Neoplasias Ósseas/cirurgia , Salvamento de Membro , Próteses e Implantes , Adolescente , Adulto , Amputação Cirúrgica/estatística & dados numéricos , Criança , Pré-Escolar , Bases de Dados Factuais , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Infecções Relacionadas à Prótese/epidemiologia , Infecções Relacionadas à Prótese/terapia , Reoperação/estatística & dados numéricos , Estudos Retrospectivos , Reino Unido/epidemiologia , Adulto Jovem
15.
Bone Joint J ; 98-B(4): 555-63, 2016 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-27037440

RESUMO

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.


Assuntos
Neoplasias Ósseas/terapia , Fraturas Espontâneas/prevenção & controle , Osteossarcoma/terapia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Neoplasias Ósseas/complicações , Criança , Terapia Combinada , Feminino , Seguimentos , Fraturas Espontâneas/etiologia , Humanos , Masculino , Pessoa de Meia-Idade , Osteossarcoma/complicações , Prognóstico , Estudos Retrospectivos , Resultado do Tratamento , Adulto Jovem
16.
Eur J Surg Oncol ; 42(7): 1064-70, 2016 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-26924782

RESUMO

BACKGROUND: Extra-pleural and extra-meningeal solitary fibrous tumour (SFT) is a rare sarcoma histotype curable with surgery in the majority of patients. The behaviour of these tumours ranges from indolent/very low grade to malignant/high grade but it is still not possible to accurately predict prognosis after surgery. We have investigated a multi-centre series to stratify the risk of recurrence to patients with SFTs. METHODS: We retrospectively analysed the data from 243 patients who underwent surgery (2002-2011) at four sarcoma referral centres. RESULTS: Upon univariate analysis, hypercellularity, atypia, necrosis, high mitotic rate (ie >4 mitoses/10 HPF) were associated with both disease-free and overall survival. Surgical margins were a significant prognostic factor for disease-free (P = 0.007) but not for overall survival. Unexpectedly, larger tumour size was associated with a better prognosis (P = 0.038) and fewer recurrences (P = 0.024). Upon multivariable analysis, high mitotic rate (hazard ratio, HR = 2.85, P = 0.002), cellular atypia (HR = 1.62, P = 0.015) and hypercellularity (HR = 1.82, P = 0.031) were significantly associated with recurrences. A SFT recurrence score has been provided to stratify risk of recurrence. CONCLUSION: This study provides a prognostic model to stratify risk of recurrence in patients with resectable SFTs. This allows clinician to decide on an optimal follow-up strategy and to select patients that may benefit from adjuvant treatments.


Assuntos
Tumores Fibrosos Solitários/patologia , Tumores Fibrosos Solitários/cirurgia , Adulto , Idoso , Feminino , Humanos , Masculino , Margens de Excisão , Pessoa de Meia-Idade , Índice Mitótico , Necrose , Recidiva Local de Neoplasia/diagnóstico , Valor Preditivo dos Testes , Prognóstico , Estudos Retrospectivos
17.
Bone Joint J ; 97-B(9): 1284-90, 2015 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-26330598

RESUMO

Patients who have limb amputation for musculoskeletal tumours are a rare group of cancer survivors. This was a prospective cross-sectional survey of patients from five specialist centres for sarcoma surgery in England. Physical function, pain and quality of life (QOL) outcomes were collected after lower extremity amputation for bone or soft-tissue tumours to evaluate the survivorship experience and inform service provision. Of 250 patients, 105 (42%) responded between September 2012 and June 2013. From these, completed questionnaires were received from 100 patients with a mean age of 53.6 years (19 to 91). In total 60 (62%) were male and 37 (38%) were female (three not specified). The diagnosis was primary bone sarcoma in 63 and soft-tissue tumour in 37. A total of 20 tumours were located in the hip or pelvis, 31 above the knee, 32 between the knee and ankle and 17 in the ankle or foot. In total 22 had hemipelvectomy, nine hip disarticulation, 35 transfemoral amputation, one knee disarticulation, 30 transtibial amputation, two toe amputations and one rotationplasty. The Toronto Extremity Salvage Score (TESS) differed by amputation level, with poorer scores at higher levels (p < 0.001). Many reported significant pain. In addition, TESS was negatively associated with increasing age, and pain interference scores. QOL for Cancer Survivors was significantly correlated with TESS (p < 0.001). This relationship appeared driven by pain interference scores. This unprecedented national survey confirms amputation level is linked to physical function, but not QOL or pain measures. Pain and physical function significantly impact on QOL. These results are helpful in managing the expectations of patients about treatment and addressing their complex needs.


Assuntos
Amputação Cirúrgica/reabilitação , Neoplasias Ósseas/cirurgia , Extremidade Inferior/cirurgia , Qualidade de Vida , Neoplasias de Tecidos Moles/cirurgia , Adulto , Idoso , Idoso de 80 Anos ou mais , Amputação Cirúrgica/efeitos adversos , Amputados/psicologia , Amputados/reabilitação , Membros Artificiais , Estudos Transversais , Feminino , Inquéritos Epidemiológicos , Humanos , Masculino , Pessoa de Meia-Idade , Medição da Dor/métodos , Dor Pós-Operatória/etiologia , Psicometria , Recuperação de Função Fisiológica , Adulto Jovem
18.
Ann R Coll Surg Engl ; 97(6): 434-8, 2015 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-26274753

RESUMO

INTRODUCTION: This study investigated the accuracy of general practitioner (GP) referrals under the two-week wait pathway for soft tissue sarcomas and whether the current National Institute for Health and Care Excellence criteria should be refined. METHODS: All patients referred under the two-week wait system to one centre over the course of one year were reviewed. Comparison was made between the criteria identified by the GP and those confirmed by the centre to assess the accuracy of the referrals, and to identify what criteria predicted malignancy. RESULTS: Overall, 135 patients were referred to our unit with a mean age of 56.4 years. Of these, 45 (33%) were found to have a malignant tumour. Factors identified by the GP were accurate in 74% of cases. The best predictor of malignancy was 'size >5cm' (76% sensitivity) while 'pain' was the least useful (27% sensitivity). Lowering the threshold for concern to a size of >4cm increased sensitivity to 89%. Although 106 patients had undergone some form of imaging prior to referral, this did not increase the likelihood of malignancy being detected. The combination of factors most likely to predict malignancy was a size of >5cm, increase in size, deep location and no pain (10 out of 13 referrals, 77% accuracy). CONCLUSIONS: Based on the results of this study, we recommend an adaption of the existing features for concern. The new feature for concern should be 'size >4cm' and the factor 'pain' should be removed from the urgent referral form.


Assuntos
Medicina de Família e Comunidade/normas , Encaminhamento e Consulta/normas , Sarcoma/diagnóstico , Neoplasias de Tecidos Moles/diagnóstico , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Pré-Escolar , Progressão da Doença , Inglaterra , Medicina de Família e Comunidade/organização & administração , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Dor/etiologia , Encaminhamento e Consulta/organização & administração , Fatores de Risco , Sarcoma/complicações , Sarcoma/patologia , Neoplasias de Tecidos Moles/complicações , Neoplasias de Tecidos Moles/patologia , Listas de Espera , Adulto Jovem
19.
Bone Joint J ; 97-B(1): 115-20, 2015 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-25568424

RESUMO

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.


Assuntos
Amputação Cirúrgica/mortalidade , Neoplasias Ósseas/mortalidade , Neoplasias Ósseas/cirurgia , Tratamentos com Preservação do Órgão/mortalidade , Osteossarcoma/mortalidade , Osteossarcoma/cirurgia , Adolescente , Adulto , Amputação Cirúrgica/métodos , Análise de Variância , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Neoplasias Ósseas/tratamento farmacológico , Neoplasias Ósseas/patologia , Criança , Pré-Escolar , Intervalos de Confiança , Bases de Dados Factuais , Intervalo Livre de Doença , Feminino , Humanos , Estimativa de Kaplan-Meier , Extremidade Inferior/patologia , Extremidade Inferior/cirurgia , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Invasividade Neoplásica/patologia , Estadiamento de Neoplasias , Tratamentos com Preservação do Órgão/métodos , Osteonecrose/induzido quimicamente , Osteonecrose/patologia , Osteossarcoma/tratamento farmacológico , Osteossarcoma/patologia , Prognóstico , Estudos Retrospectivos , Medição de Risco , Análise de Sobrevida , Resultado do Tratamento , Adulto Jovem
20.
Bone Joint J ; 97-B(2): 252-7, 2015 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-25628291

RESUMO

We conducted a case-control study to examine the merit of silver-coated tumour prostheses. We reviewed 85 patients with Agluna-treated (silver-coated) tumour implants treated between 2006 and 2011 and matched them with 85 control patients treated between 2001 and 2011 with identical, but uncoated, tumour prostheses. In all, 106 men and 64 women with a mean age of 42.2 years (18.4 to 90.4) were included in the study. There were 50 primary reconstructions (29.4%); 79 one-stage revisions (46.5%) and 41 two-stage revisions for infection (24.1%). The overall post-operative infection rate of the silver-coated group was 11.8% compared with 22.4% for the control group (p = 0.033, chi-square test). A total of seven of the ten infected prostheses in the silver-coated group were treated successfully with debridement, antibiotics, and implant retention compared with only six of the 19 patients (31.6%) in the control group (p = 0.048, chi-square test). Three patients in the silver-coated group (3.5%) and 13 controls (15.3%) had chronic periprosthetic infection (p = 0.009, chi-square test). The overall success rates in controlling infection by two-stage revision in the silver-coated group was 85% (17/20) compared with 57.1% (12/21) in the control group (p = 0.05, chi-square test). The Agluna-treated endoprostheses were associated with a lower rate of early periprosthetic infection. These silver-treated implants were particularly useful in two-stage revisions for infection and in those patients with incidental positive cultures at the time of implantation of the prosthesis. Debridement with antibiotic treatment and retention of the implant appeared to be more successful with silver-coated implants.


Assuntos
Neoplasias Ósseas/cirurgia , Materiais Revestidos Biocompatíveis , Próteses e Implantes/efeitos adversos , Infecções Relacionadas à Prótese/epidemiologia , Infecções Relacionadas à Prótese/prevenção & controle , Prata/administração & dosagem , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Estudos de Casos e Controles , Desbridamento , Feminino , Neoplasias Femorais/cirurgia , Humanos , Incidência , Masculino , Análise por Pareamento , Pessoa de Meia-Idade , Reoperação , Tíbia , Adulto Jovem
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