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1.
Child Care Health Dev ; 50(1): e13139, 2024 01.
Artigo em Inglês | MEDLINE | ID: mdl-37269221

RESUMO

BACKGROUND: Neurocognitive sequelae are among the most debilitating effects of cancer observed in children. Yet we know very little about the impact on neurocognitive functioning, especially cancer types that develop outside the central nervous system. This study aimed to assess and compare the cognitive functions (CoF) of children with bone tumours and lymphoma in the treatment process. METHODS: The CoF of children with bone tumours (n = 44), lymphoma (n = 42) and their non-cancer peers (n = 55) were assessed with Dynamic Occupational Therapy Assessment for Children. The CoF of children with cancer were compared with their non-cancer peers. Then, children with bone tumours and lymphoma were compared in binary. RESULTS: One-hundred forty-one children aged 6-12 years with a mean age of 9.4 (SD = 1.5) were included in this study. The orientation and visuomotor construction functions of children with bone tumours and orientation, praxis and visuomotor construction functions of children with lymphoma performed worse than their non-cancer peers (pk < 0.001). While orientation, spatial perception, visuomotor construction and thinking operations functions of children with bone tumours and lymphoma were similar (pk > 0.016), praxis functions of children with lymphoma were found to be worse than children with bone tumours (pk < 0.016). CONCLUSIONS: Our findings show that children with bone tumours and lymphoma in the process of treatment are at risk for impairment of their CoF. The findings highlight the importance of assessing CoF in children with bone tumours and lymphoma and considering specific differences between groups. It is essential to assess CoF and develop early intervention plans in these children.


Assuntos
Neoplasias Ósseas , Linfoma , Criança , Humanos , Cognição
2.
Histopathology ; 80(4): 686-697, 2022 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-34821406

RESUMO

AIMS: To evaluate the diagnostic accuracy of SSX and SSX::SS18 antibodies in decalcified surgical specimens and outcome of synovial sarcomas (SS) of bone. METHODS AND RESULTS: Twenty-five cases were classified as bone SS (prevalence 0.32% among malignant primary bone sarcoma). Median age was 34 years (range = 9-79). Twenty-four of 25 patients presented with non-metastatic tumours, one with lung metastases. The majority of tumours involved the long bones of extremities with metaphyseal origin. Mean size of the tumour was 7.1 cm. Twenty cases (80%) were monophasic and five (20%) were biphasic. SS18::SSX fusion-specific antibody had 92% sensitivity and 99% specificity for primary bone SS, whereas SSX C-terminus antibody had 100% sensitivity and 94% specificity. Fluorescence in-situ hybridisation (FISH) analysis was feasible in nine (36%) cases and detected SS18 rearrangement in all nine cases. All patients underwent surgical removal of their primary tumour, with adequate margins in 18 (72%) patients. Chemotherapy with metothrexate, cisplatin, doxorubicin and ifosfamide was used in the seven patients. Two patients with inadequate surgical margins received radiotherapy. With a median follow-up of 80 months (range = 6-428), 5- and 10-year overall survival (OS) was 66.6% and 47.9%, respectively, and 5 and 10 years' disease-free survival (DFS) was 36.8% [95% confidence interval (CI) = 18.0-55.7%] and 32.2% (95% CI = 14.6-51.2%), respectively. A significant improvement in 10 years' DFS in patients undergoing chemotherapy compared with patients who did not was observed (P = 0.039). CONCLUSIONS: Our series highlights the utility of SS18::SSX fusion-specific and SSX C-terminus antibodies to support the diagnosis of SS. Adjustment chemotherapy was associated with improved prognosis in this series.


Assuntos
Anticorpos/análise , Neoplasias Ósseas/diagnóstico , Proteínas de Fusão Oncogênica/imunologia , Sarcoma Sinovial/diagnóstico , Adolescente , Adulto , Idoso , Neoplasias Ósseas/patologia , Criança , Feminino , Humanos , Imuno-Histoquímica , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Sarcoma Sinovial/patologia , Adulto Jovem
3.
Surgeon ; 20(5): e236-e240, 2022 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-34446348

RESUMO

BACKGROUND: Endoprosthetic replacement of the proximal femur plays a vital role in managing metastatic and primary bone tumours1. Length of stay (LOS) has important resource implications but is driven by patient and disease factors over and above the procedure itself. The aim of this project was to identify factors that drive LOS in patients undergoing proximal femoral replacement (PFR). METHODS: This was a retrospective analysis of clinical records from a single centre (RNOH). 144 cases were identified over a 4 year-period. These were divided into 3 diagnostic categories: primary bone tumour with chemotherapy, primary bone tumour without chemotherapy and metastatic bone disease. Several factors were considered that could influence the length of stay including age, ASA grade, gender, admission to the high dependency unit (HDU), diagnosis, saving the greater trochanter, pre-operative radiotherapy, admission with a fracture and return to theatre. RESULTS: The median LOS for PFR was 15 days, with 79% admitted to HDU. LOS was almost doubled for patients returning to theatre (P = 0.04). Patients with ASA grades of 3 and 4 had a 75% longer LOS compared to those with grade 1. Additionally, a 10-year increase in age was associated with a 6-8% increase in LOS. Incorporating these factors produced a model which explained 27% of the variability of LOS. CONCLUSION: Majority of the variables which were tested were significantly associated with LOS. However, factors other than those in our model drive length of stay. This analysis can support conversations with patients and service planning around LOS.


Assuntos
Neoplasias Ósseas , Fêmur , Neoplasias Ósseas/cirurgia , Fêmur/cirurgia , Humanos , Tempo de Internação , Estudos Retrospectivos , Resultado do Tratamento
4.
Biochem Cell Biol ; 99(6): 717-724, 2021 12.
Artigo em Inglês | MEDLINE | ID: mdl-34096319

RESUMO

An understanding of the biochemistry of the giant cell tumour of bone (GCTB) provides an opportunity for the development of prognostic markers and identification of therapeutic targets. Based on metabolomic analysis, we proposed glycerophospholipid metabolism as the altered pathway in GCTB., The objective of this study was to identify these altered metabolites. Using phosphorus-31 nuclear magnetic resonance spectroscopy (31P-NMR), sphingomyelin was determined to be the most dysregulated phospholipid in tissue samples from six patients with GCTB. Enzymes related to its biosynthesis and hydrolysis were examined using immunodetection techniques. High expression of sphingomyelin synthases 1 and 2, but low expression of neutral sphingomyelinase 2 (nSMase2) was found in GCTB tissues compared to non-neoplastic bone tissues. Sphingomyelin/ceramide biosynthesis is dysregulated in GCTB due to alterations in the expression of SMS1, SMS2, and nSMase2.


Assuntos
Neoplasias Ósseas , Tumor de Células Gigantes do Osso , Osso e Ossos , Humanos , Espectroscopia de Ressonância Magnética , Esfingomielinas
5.
Histopathology ; 79(5): 880-885, 2021 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-34148257

RESUMO

AIMS: Because of the efficacy of tropomyosin receptor kinase (Trk) inhibitor therapy in tumours with rearrangements of the neurotrophic tyrosine kinase receptor genes (NRTK genes), there has been a surge in demand for NTRK fusion screening. To date, most studies involving mesenchymal tumours have focused on soft tissue tumours, and data on bone tumours are sparse. Hence, we aimed to explore the frequency of NTRK fusions in a large series of primary bone tumours. METHODS AND RESULTS: Immunohistochemical expression of pan-Trk was successfully assessed in 354 primary bone tumours by the use of tissue microarrays. In a selection of positive cases, additional molecular analysis for NTRK fusions was performed with anchored multiplex polymerase chain reaction-based targeted next-generation sequencing. Positivity was found in 19 cases (5%), which comprised Ewing sarcoma (n = 6, 33%), osteosarcoma (n = 11, 13%), and giant-cell tumour of bone (n = 2, 3%). In all except one case, cytoplasmic staining was observed. Weak staining was most often observed (n = 13), although five cases showed moderate staining and one case showed focal strong staining. Molecular analysis was successful in six cases, all of which were negative for NTRK fusions. CONCLUSION: The likelihood of finding an NTRK fusion in bone tumours in clinical practice is extremely low. This may imply that, if more comprehensive large-scale molecular studies confirm this, routine predictive NTRK testing in bone tumour patients with advanced disease may be reconsidered.


Assuntos
Neoplasias Ósseas , Proteínas de Fusão Oncogênica , Receptor trkA , Biomarcadores Tumorais/genética , Biomarcadores Tumorais/metabolismo , Neoplasias Ósseas/diagnóstico , Neoplasias Ósseas/genética , Neoplasias Ósseas/metabolismo , Humanos , Imuno-Histoquímica , Proteínas de Fusão Oncogênica/genética , Proteínas de Fusão Oncogênica/metabolismo , Receptor trkA/genética , Receptor trkA/metabolismo
6.
BMC Musculoskelet Disord ; 22(1): 18, 2021 Jan 05.
Artigo em Inglês | MEDLINE | ID: mdl-33402136

RESUMO

BACKGROUND: Like with all cancers, multidisciplinary team (MDT) meetings are the norm in bone and soft tissue tumour (BST) management too. Problem in attendance of specialists due to geographical location is the one of the key barriers to effective functioning of MDTs. To overcome this problem, virtual MDTs involving videoconferencing or telemedicine have been proposed, but however this has been seldom used and tested. The COVID-19 pandemic forced the implementation of virtual MDTs in the Oxford sarcoma service in order to maintain normal service provision. We conducted a survey among the participants to evaluate its efficacy. METHODS: An online questionnaire comprising of 24 questions organised into 4 sections was circulated among all participants of the MDT after completion of 8 virtual MDTs. Opinions were sought comparing virtual MDTs to the conventional face-to-face MDTs on various aspects. A total of 36 responses were received and were evaluated. RESULTS: 72.8% were satisfied with the depth of discussion in virtual MDTs and 83.3% felt that the decision-making in diagnosis had not changed following the switch from face-to-face MDTs. About 86% reported to have all essential patient data was available to make decisions and 88.9% were satisfied with the time for discussion of patient issues over virtual platform. Three-fourths of the participants were satisfied (36.1% - highly satisfied; 38.9% - moderately satisfied) with virtual MDTs and 55.6% of them were happy to attend MDTs only by the virtual platform in the future. Regarding future, 77.8% of the participants opined that virtual MDTs would be the future of cancer care and an overwhelming majority (91.7%) felt that the present exercise would serve as a precursor to global MDTs involving specialists from abroad in the future. CONCLUSION: Our study shows that the forced switch to virtual MDTs in sarcoma care following the unprecedented COVID-19 pandemic to be a viable and effective alternative to conventional face-to-face MDTs. With effective and efficient software in place, virtual MDTs would also facilitate in forming extended MDTs in seeking opinions on complex cases from specialists abroad and can expand cancer care globally.


Assuntos
Neoplasias Ósseas/terapia , COVID-19 , Comunicação Interdisciplinar , Oncologia/organização & administração , Neoplasias Musculares/terapia , Equipe de Assistência ao Paciente/organização & administração , Sarcoma/terapia , Telemedicina/organização & administração , Comunicação por Videoconferência/organização & administração , Atitude do Pessoal de Saúde , Atitude Frente aos Computadores , Neoplasias Ósseas/diagnóstico , Tomada de Decisão Clínica , Prestação Integrada de Cuidados de Saúde/organização & administração , Conhecimentos, Atitudes e Prática em Saúde , Humanos , Neoplasias Musculares/diagnóstico , Sarcoma/diagnóstico , Centros de Atenção Terciária
7.
Cytopathology ; 32(2): 243-249, 2021 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-33090567

RESUMO

INTRODUCTION: Fine needle aspiration is a well-established technique for evaluating primary and secondary bony lesions. With use in selected cases, it achieves a diagnostic yield comparable to biopsies. METHODS: Cases of osteosarcoma (OS) with available histological follow-up were retrieved over a 10-year period. Detailed morphological evaluation was done, with special emphasis on pitfalls in the diagnosis of OS on cytology and the various variants of OS. RESULTS: Of the 41 cases with available follow-up histology, 56% were correctly diagnosed as OS on cytology. The most common false-negative cytological diagnosis of OS, in 17% cases, was giant cell tumour. The possible explanations for this included low cellularity, minimal atypia, absence of typical osteoid, misinterpretation of metachromatic osteoid material as fibro-collagenous material and non-availability of radiology at time of aspiration. CONCLUSION: A triple-phase evaluation including clinical evaluation, appropriate radiological correlation and cytology/histopathology, is important to clinch an accurate diagnosis.


Assuntos
Neoplasias Ósseas/patologia , Tumores de Células Gigantes/patologia , Osteossarcoma/diagnóstico , Osteossarcoma/patologia , Biópsia por Agulha Fina/métodos , Citodiagnóstico/métodos , Técnicas Citológicas , Humanos
8.
Int Orthop ; 45(3): 779-789, 2021 03.
Artigo em Inglês | MEDLINE | ID: mdl-33094400

RESUMO

INTRODUCTION: Although consensus has been reached regarding the main aspects of intralesional surgery for giant cell tumour of the bone (GCTB), debates continue about the most effective combination of local adjuvants. The purpose of study was to analyze the previous experience and determine the most effective curettage approach for GCTB. METHODS: We summarized the findings from 89 papers published from 1962 to 2020 related to this subject. Database consisted of 137 treated groups that included 6441 patients who underwent different curettage techniques without pre-operative administration of bisphosphonates or RANKL inhibitors. RESULTS: Recurrence rates after simple curettage ranged between 27 and 82% with a median value at 47%. The use of one or two local adjuvants reduced the incidence of recurrences approximately by 50% when compared with simple curettage. High-speed burring combined with chemical adjuvants or followed by poly(methyl methacrylate) cementation with or without bone grafting further improved the local control leading to good and excellent results; however, these were not documented in all studies. Simultaneous use of burring, chemical adjuvants, and cementation, which we named here as combined curettage, allowed to down local relapses to the range of 0-26%, with a median at 11%. Oncologic outcomes after combined curettage are significantly better when compared with simple curettage (p < 0.0001) and other variants of enhancement (p = 0.001). CONCLUSIONS: Combined curettage appears to provide the most potent and comprehensive impact on residual tumour cells located in risk zones. This approach should be considered for locally advanced tumours when function-preserving surgery is planned. Additional comparative studies are required to define the optimal curettage enhancement for each individual patient.


Assuntos
Neoplasias Ósseas , Tumor de Células Gigantes do Osso , Neoplasias Ósseas/cirurgia , Curetagem , Tumor de Células Gigantes do Osso/cirurgia , Humanos , Recidiva Local de Neoplasia/epidemiologia , Recidiva Local de Neoplasia/prevenção & controle , Estudos Retrospectivos
9.
Histopathology ; 77(3): 391-401, 2020 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-32506447

RESUMO

AIMS: To evaluate the available diagnostic histological criteria for synovial chondrosarcoma and to screen for the presence of IDH1/IDH2 mutations in a series of cases of this malignant cartilaginous neoplasm. METHODS AND RESULTS: Ten cases of synovial chondrosarcoma diagnosed at our institute were reviewed. At presentation, all tumours occurred in adults (median age, 62 years). The most common location was the knee joint (five cases), and the size at diagnosis ranged from 30 mm to 170 mm. Eight patients had secondary synovial chondrosarcomas associated with pre-existing/recurrent or concomitant synovial chondromatosis. Five patients had local recurrences and three had lung metastases. All patients with intralesional excisions developed local recurrences, whereas those who underwent wide resections did not. At last follow-up (mean, 91 months), available for nine patients, seven patients were alive and disease-free, one patient had died of disease, and one was alive with paravertebral metastases. Frequent histological features observed included loss of clustering of chondrocytes (nine cases), the presence of variable amounts of myxoid matrix (eight cases), peripheral hypercellularity (eight cases), tumour necrosis (six cases), and spindling of chondrocytes (four cases). Of the seven cases for which it was possible to evaluate bone permeation, six showed infiltration of bone marrow. All seven cases screened for mutations of exon 4 of IDH1 and IDH2 were found to be wild-type. CONCLUSIONS: Histological criteria in correlation with clinical and radiological features allow the recognition of synovial chondrosarcoma. IDH1/IDH2 mutations were not present in synovial chondrosarcoma. Adequate surgical margins are important for disease control.


Assuntos
Condrossarcoma , Adulto , Idoso , Neoplasias Ósseas/diagnóstico , Neoplasias Ósseas/diagnóstico por imagem , Neoplasias Ósseas/genética , Neoplasias Ósseas/patologia , Cartilagem/patologia , Condromatose Sinovial/complicações , Condromatose Sinovial/patologia , Condrossarcoma/diagnóstico , Condrossarcoma/diagnóstico por imagem , Condrossarcoma/genética , Condrossarcoma/patologia , Feminino , Histologia , Humanos , Isocitrato Desidrogenase/genética , Articulação do Joelho/patologia , Masculino , Margens de Excisão , Pessoa de Meia-Idade , Mutação , Metástase Neoplásica/patologia , Recidiva Local de Neoplasia/patologia , Estudos Retrospectivos , Tomografia Computadorizada por Raios X , Adulto Jovem
10.
Pathologica ; 112(4): 184-190, 2020 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-33179613

RESUMO

OBJECTIVE: To report the exceptional occurrence of ossifying fibromyxoid tumour (OFMT) as a primary bone lesion. OFMT is a rare soft tissue tumour of uncertain differentiation and variable malignant potential, that occurs in adults with a slight male predominance. It is typically located in the subcutis or in the skeletal muscles of the extremities, followed by trunk or head and neck. METHODS: Two cases of OFMT proven to arise from bone are presented. The first is a 65-year old female with a history of rib "osteosarcoma", presenting with an inferior lobe left lung mass. The second is a man with a lytic lesion of the 5th cervical vertebra that recurred shortly after resection. Following H&E and immunohistochemical examination, tumour samples were analysed by NGS and by break-apart FISH to detect rearrangement of the PHF1 and TFE3 genes. RESULTS: PHF1 gene-rearrangement was identified by FISH on both the primary and the metastatic lesion of first patient. NGS identified a PHF1(intron1) and EPC1 (exon 10) fusion transcript later confirmed by positive PHF1 rearrangement on FISH in the second case. CONCLUSIONS: The demonstration of PHF1 gene rearrangements represents a fundamental ancillary diagnostic test when presented with challenging examples of OFMT.


Assuntos
Neoplasias Ósseas , Proteínas de Ligação a DNA/genética , Fibroma Ossificante , Proteínas do Grupo Polycomb/genética , Adulto , Idoso , Biomarcadores Tumorais/análise , Neoplasias Ósseas/diagnóstico , Neoplasias Ósseas/genética , Neoplasias Ósseas/patologia , DNA de Neoplasias/análise , Feminino , Fibroma Ossificante/diagnóstico , Fibroma Ossificante/genética , Fibroma Ossificante/patologia , Rearranjo Gênico , Sequenciamento de Nucleotídeos em Larga Escala , Humanos , Imuno-Histoquímica , Hibridização in Situ Fluorescente , Masculino , Recidiva Local de Neoplasia/patologia , Neoplasias de Tecidos Moles/diagnóstico , Neoplasias de Tecidos Moles/genética , Neoplasias de Tecidos Moles/patologia , Fatores de Transcrição/genética
11.
Pol J Radiol ; 85: e409-e419, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32999694

RESUMO

Similarly to soft tissue tumours, the World Health Organisation (WHO) classification categorises bone tumours based on their similarity to normal adult tissue. The most recent WHO classification provides an updated classification scheme that integrates the biological behaviour of bone tumours, particularly cartilage-forming tumours, and tumours are now further subdivided as benign, intermediate (locally aggressive or rarely metastasising), and malignant. Radiologists play an important role in the detection and initial characterisation of bone tumours, with careful analysis of their matrix mineralisation, location, and overall anatomic extent including extra-compartmental extension and neurovascular invasion. Radiography remains central to the detection and characterisation of bone tumours; however, magnetic resonance imaging (MRI) is the ideal modality for local staging. This review will discuss the most recent updates to the WHO classification of bone tumours that are relevant to radiologists in routine clinical practice. The utility of advanced MRI sequences such as diffusion-weighted imaging, dynamic contrast enhanced sequences, and magnetic resonance spectroscopy that may provide insight into the biological behaviour of various bone tumours is highlighted.

12.
Br J Clin Pharmacol ; 85(6): 1169-1179, 2019 06.
Artigo em Inglês | MEDLINE | ID: mdl-30471134

RESUMO

Fibrous dysplasia (FD) is a genetic, noninheritable rare bone disease caused by a postzygotic activating mutation of the α subunit of the stimulatory G-protein causing increased abnormal bone formation leading to pain, deformity and fractures. To date, no cure has been identified for FD/McCune-Albright syndrome (MAS) and treatment is symptomatic and aimed at decreasing pain and/or local bone turnover. Various drugs have been used to achieve clinical improvement in FD/MAS patients including bisphosphonates and denosumab, however further translational studies are also warranted to address unresolved pathophysiological issues and explore novel pharmacological targets for the management of FD/MAS. In this article, we review literature on the medical treatment of FD/MAS, discuss the unresolved pathophysiological issues and explore novel pharmacological targets for the management of FD/MAS.


Assuntos
Conservadores da Densidade Óssea/uso terapêutico , Remodelação Óssea/efeitos dos fármacos , Osso e Ossos/efeitos dos fármacos , Displasia Fibrosa Óssea/tratamento farmacológico , Animais , Conservadores da Densidade Óssea/efeitos adversos , Osso e Ossos/patologia , Osso e Ossos/fisiopatologia , Descoberta de Drogas , Displasia Fibrosa Óssea/patologia , Displasia Fibrosa Óssea/fisiopatologia , Humanos , Pesquisa Translacional Biomédica , Resultado do Tratamento
13.
Arch Orthop Trauma Surg ; 139(8): 1033-1038, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-30715569

RESUMO

INTRODUCTION: Curettage with cement augmentation is a technique used in the treatment of bone tumours. Thermal energy released during the cement polymerisation process can damage surrounding tissues. This study aims to record temperature changes at various sites on and around bone during the cementing process. We hypothesised that adjacent structures, such as the radial nerve, may be threatened by this process in the clinical setting. MATERIALS AND METHODS: Using 18 porcine femurs as a model of the human humerus, we used thermocouples and a thermal imaging camera to measure changes in temperature during the cementing process. Fractures were created in nine samples to establish whether a discontinuity of the cortex had an effect on thermal conduction. RESULTS: Significantly higher temperatures were recorded in samples with a fracture compared to those without a fracture. The site overlying the centre of the cement bolus (hypothetical site of the radial nerve) demonstrated higher temperatures than all other sites on the same cortex. When considering the radial nerve site, over half the samples demonstrated temperatures exceeding 47 °C for over a minute. When a threshold of 50 °C for more than 30 s was considered, three samples without a fracture exceeded this value compared to two with a fracture. CONCLUSION: The temperatures recorded were sufficient to cause damage to neural tissue. Limiting thermal exposure to soft tissues is recommended. Increased attention is required when using larger cement boluses, or where bone quality is poor or a fracture, iatrogenic or preexisting, is present.


Assuntos
Cimentos Ósseos , Curetagem , Neoplasias Femorais/cirurgia , Temperatura Alta/efeitos adversos , Nervo Radial/lesões , Animais , Fraturas Espontâneas/patologia , Modelos Animais , Polimerização , Neuropatia Radial/etiologia , Suínos , Temperatura
14.
Eur Spine J ; 27(4): 902-912, 2018 04.
Artigo em Inglês | MEDLINE | ID: mdl-29445946

RESUMO

PURPOSE: Cervicothoracic paravertebral neoplasms extending into the mediastinum pose a surgical challenge due the complex regional anatomy, their biological nature, rarity and surgeon's unfamiliarity with the region. We aim to define a surgical access framework addressing the aforementioned complexities whilst achieving oncological clearance. METHODS: We carried out a retrospective review of 28 consecutive patients operated in two tertiary referral centres between 1998 and 2015. Pathology was located paravertebrally from C6 to T4 with superior mediastinum invasion. Patients were operated jointly by a spinal and a thoracic surgeon. RESULTS: Tumours were classified according to subclavian fossa involvement as anteromedial, anterolateral and posterior and according to histology in benign nerve sheath tumour group (n = 10) and malignant bone or soft tissue tumours (n = 18). Three surgical routes were utilised: (1) median sternotomy (n = 11), (2) anterior cervical transsternal approach (n = 7) and (3) high posterolateral thoracotomy (n = 10). Resection was en bloc with wide margins in 22 cases, marginally complete in 3 and incomplete in 3. Complications included Horner's syndrome (n = 3), infection (n = 2) and transient neurological deficit (n = 4). In the nerve sheath tumour group, no recurrence or reoperation took place with a median follow-up of 4.5 years. In the malignant bone and soft tissue group, 96% of the patients were alive at 1 year, 67% at 2 years and 33% at 5 years. No vascular injuries or operative related deaths were observed. CONCLUSIONS: Classification of cervicothoracic paravertebral neoplasms with mediastinal extension according to the relationship with the subclavicular fossa and dual speciality involvement allows for a structured surgical approach and provides minimal morbidity/maximum resection and satisfactory oncological outcomes. These slides can be retrieved under Electronic Supplementary Material.


Assuntos
Neoplasias do Mediastino/patologia , Neoplasias de Bainha Neural/patologia , Neoplasias de Tecidos Moles/patologia , Neoplasias da Coluna Vertebral/patologia , Procedimentos Cirúrgicos Torácicos/estatística & dados numéricos , Adolescente , Adulto , Idoso , Vértebras Cervicais/patologia , Vértebras Cervicais/cirurgia , Criança , Feminino , Humanos , Masculino , Neoplasias do Mediastino/mortalidade , Neoplasias do Mediastino/cirurgia , Mediastino/patologia , Mediastino/cirurgia , Pessoa de Meia-Idade , Recidiva Local de Neoplasia/epidemiologia , Neoplasias de Bainha Neural/mortalidade , Neoplasias de Bainha Neural/cirurgia , Complicações Pós-Operatórias/epidemiologia , Reoperação , Estudos Retrospectivos , Neoplasias de Tecidos Moles/mortalidade , Neoplasias de Tecidos Moles/cirurgia , Neoplasias da Coluna Vertebral/mortalidade , Neoplasias da Coluna Vertebral/cirurgia , Análise de Sobrevida , Procedimentos Cirúrgicos Torácicos/efeitos adversos , Vértebras Torácicas/patologia , Vértebras Torácicas/cirurgia , Adulto Jovem
15.
Int Orthop ; 42(8): 1999-2002, 2018 08.
Artigo em Inglês | MEDLINE | ID: mdl-29468264

RESUMO

PURPOSE: Resection length should be designed before limb salvage surgery of bone tumours. The aim of this study was to evaluate the accuracy of free hand resections. METHODS: Two hundred forty-eight cases were enrolled, including 173 osteosarcomas, 24 giant cell tumours, 16 chondrosarcomas, seven spindle cell sarcomas, 14 bone metastases, three undifferentiated pleomorphic sarcomas, three Ewing sarcomas, two angiosarcomas, and six other bone and soft tissue tumours. One hundred forty-six were located in the femur, 75 in the tibia, 19 in the humerus, six in the radius, one in the ulna, and one in the fibula. The resection length was included in the pre-operative plan. After surgery, we measure the length of specimens. Both lengths were compared. The patients were classified by tumour location. RESULTS: The range of length difference was from - 21 to 29 mm. The mean absolute value of the differences was 8.0 ± 6.3 mm. Altogether, 173 cases (69.8%) had an absolute difference value of ≤ 10 mm, 66 cases (26.6%) of 10-20 mm, and only 9 cases (3.6%) of > 20 mm. The average length of gross specimens (164.1 ± 43.3 mm) was longer than planned lengths (160.7 ± 44.2 mm); p < 0.001. CONCLUSIONS: The differences were significant in the distal femur and proximal tibia. Even though, the accuracy of free hand resection is acceptable in this method.


Assuntos
Neoplasias Ósseas/cirurgia , Extremidades/cirurgia , Salvamento de Membro/métodos , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Extremidades/patologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Adulto Jovem
16.
J Pak Med Assoc ; 68(10): 1502-1507, 2018 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-30317350

RESUMO

Over the last century, there has been a remarkable development in the study of benign bone tumours. This is primarily due to the improved knowledge of the nature of these lesions and improved imaging technology. They present as a diverse group of clinical and pathological entities, which vary in their clinical behaviour and aggressiveness and, hence, multidisciplinary approach is necessary in their management. Combined opinion from an orthopaedic surgeon, radiologist and a pathologist is therefore required. Incidence of these tumours is debatable because they are often asymptomatic. Many protocols have been reported in studies with respect to the management of these tumours based on the experience of different centres and different surgeons with no set guidelines. English-language studies, including case reports, case series and systemic reviews, from PubMed, ERIC, MEDLINE, EMBASE and Cochrane Reviews databases from 2002 to 2016 were included in the current. Articles reporting all levels of evidence - Level I to V - were included.


Assuntos
Neoplasias Ósseas , Osso e Ossos/diagnóstico por imagem , Diagnóstico por Imagem/métodos , Neoplasias Ósseas/classificação , Neoplasias Ósseas/diagnóstico , Neoplasias Ósseas/epidemiologia , Diagnóstico Diferencial , Saúde Global , Humanos , Incidência
17.
Cancer Metastasis Rev ; 35(3): 475-87, 2016 09.
Artigo em Inglês | MEDLINE | ID: mdl-27566370

RESUMO

Currently used surgical techniques to reconstruct tissue defects after resection of musculoskeletal tumours are associated with high complication rates. This drives a strong demand for innovative therapeutic concepts that are able to improve the clinical outcomes of patients suffering from bone and soft tissue tumours. Tissue engineering and regenerative medicine (TE&RM) provides a technology platform based on biochemical, molecular, cellular and biomaterials modules to selectively direct tissue healing processes for improved defect regeneration. At the same time, precautionary measures have to be taken when these instruments are used in cancer patients to prevent any promotion of tumour growth or metastatic spread. On the other hand, several innovative TE&RM tools are being developed such as multi-functionalized biomaterials, drug-delivering nanomaterials or genetically engineered stem cells that per se have the potential to mediate anti-cancer effects, act synergistically with currently used chemotherapeutics and/or radiotherapy regimens and reduce their side effects. Recently, scientists became conscious that TE&RM strategies may not only be utilized to advance contemporary tissue reconstruction techniques but also to develop personalized diagnostic tools and clinically relevant disease models for cancer patients. Eventually, prospective randomized clinical trials combined with comparative outcome analyses are a conditio sine qua non to shape the benefits of personalized regenerative therapies for the standardized management of patients with musculoskeletal tumours.


Assuntos
Neoplasias Ósseas/terapia , Neoplasias Musculares/terapia , Medicina Regenerativa , Engenharia Tecidual , Animais , Neoplasias Ósseas/diagnóstico , Neoplasias Ósseas/cirurgia , Humanos , Modelos Animais , Neoplasias Musculares/diagnóstico , Neoplasias Musculares/cirurgia , Cuidados Pós-Operatórios , Medicina de Precisão , Medicina Regenerativa/métodos , Engenharia Tecidual/métodos
18.
Histopathology ; 71(1): 134-142, 2017 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-28239886

RESUMO

AIMS: Fibrocartilaginous mesenchymoma is a rare intraosseous lesion, with a total of 26 cases described in the literature. This study describes the clinical, radiological and histological features of eight new cases of fibrocartilaginous mesenchymoma collected at a single institution between 1982 and 2016. The presence of GNAS and IDH1/2 mutations and MDM2 amplification was explored to evaluate possible links between fibrocartilaginous mesenchymoma, fibrous dysplasia, de-differentiated chondrosarcoma and low-grade osteosarcoma. METHODS AND RESULTS: Eight new cases of fibrocartilaginous mesenchymoma of bone identified in our archives, dating from 1982 to 2016, were reviewed. The diagnosis was not performed on the initial biopsy in any of these cases, due mainly to the absence of obvious cartilaginous differentiation. On imaging, the tumour contained cartilaginous calcifications and showed a very strong uptake of contrast medium after injection. Histologically, the tumour was characterized by spindle cell proliferation mimicking a low-grade spindle cell sarcoma, associated with epiphyseal growth-plate-like nodules of cartilage and bone production. Molecularly, no GNAS and IDH1/2 mutations or MDM2 amplification were found in the cases analysed. Only one case recurred 1 year following intralesional resection. None died of disease. CONCLUSIONS: This very rare bone tumour has a typical radiological and histological pattern and a favourable survival outcome after treatment. Local recurrences can be prevented with complete surgery. Fibrocartilaginous mesenchymoma does not seem to be related genetically to fibrous dysplasia, low-grade osteosarcoma and de-differentiated chondrosarcoma.


Assuntos
Neoplasias Ósseas/patologia , Mesenquimoma/patologia , Adolescente , Adulto , Neoplasias Ósseas/diagnóstico , Neoplasias Ósseas/genética , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Mesenquimoma/diagnóstico , Mesenquimoma/genética , Adulto Jovem
19.
J Shoulder Elbow Surg ; 26(11): 1990-1994, 2017 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-28684229

RESUMO

BACKGROUND: The Bayley Walker (Stanmore Implants, Elstree, UK) reversed polarity, linked shoulder replacement is designed to provide stable function in the treatment of a painful shoulder with poor soft tissue coverage. We reviewed the results of the prosthesis in destructive pathologic lesions of the proximal humerus at a United Kingdom tumor center. METHODS: We identified 8 patients (2 men, 6 women) in our database. Clinical information and functional outcome scores were collected, including range of movement, Toronto Extremity Salvage Score, the Musculoskeletal Tumor Score. Radiographs from the last clinic follow-up were analyzed. RESULTS: Of the 8 patients, 2 were revisions for aseptic loosening around proximal humeral endoprosthetic replacements. Indications for surgery included chondrosarcoma in 4, metastatic disease in 2, Ewing sarcoma in 1, and osteomyelitis in 1. Patients were a mean age at diagnosis of 49 years (range, 16-78 years). One patient died of metastatic disease during follow-up. Mean follow-up was 49 months (range, 36-90 months). At the latest follow-up, there was 100% survivorship using revision as the end point. There were no local recurrences. Three of 5 patients returned to their previous occupation. Neuropathic pain developed in 1 patient postoperatively, but no other postoperative complications were noted. Radiographs showed no progressive lucencies or scapula notching. Mean range of movement at final follow-up was abduction, 62°; forward flexion, 71°; and external and internal rotation, 50°. CONCLUSION: The Bayley Walker prosthesis gives excellent medium-term survivorship and pain relief in patients with pathologic lesions of the proximal humerus requiring wide local excision.


Assuntos
Artroplastia do Ombro , Neoplasias Ósseas/cirurgia , Úmero/cirurgia , Osteomielite/cirurgia , Prótese de Ombro , Adolescente , Adulto , Idoso , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Amplitude de Movimento Articular , Articulação do Ombro/cirurgia , Adulto Jovem
20.
Int Orthop ; 41(7): 1481-1487, 2017 07.
Artigo em Inglês | MEDLINE | ID: mdl-28343291

RESUMO

PURPOSE: In 1999, we developed a technique using frozen autografts-tumour-containing bone treated with liquid nitrogen-for the reconstruction of malignant bone tumours. The purpose of this study was to evaluate the functional and oncological outcomes of frozen autografts for intercalary reconstruction of malignant bones and soft tissue tumours. METHODS: This retrospective study was designed to assess 34 patients of mean age 35 (range, 6-79) years. The mean follow-up period was 62 (24-214) months. The median length of the frozen autografts was 138.4 ± 60.39 (50-290) mm. RESULTS: Postsurgically, 20 patients remained disease-free, seven patients survived with no evidence of disease, five patients were alive with disease, and two patients died of disease. The five- and ten-year survival rates of the frozen autografts were 91.2% and the mean International Society of Limb Salvage score was 90%. Complete bony union was achieved in 97% of the patients. There were five cases of nonunion, six cases of fracture, two cases of deep infection and four cases of local recurrence. CONCLUSION: Utilizing intercalary frozen autografts for patients with a nonosteolytic primary or secondary bone tumour without involvement of the subchondral bone is a good alternative treatment, because it is a straightforward biological technique and can provide excellent limb function.


Assuntos
Neoplasias Ósseas/cirurgia , Transplante Ósseo/métodos , Procedimentos de Cirurgia Plástica/métodos , Neoplasias de Tecidos Moles/cirurgia , Transplante Autólogo/métodos , Adolescente , Adulto , Idoso , Autoenxertos , Neoplasias Ósseas/mortalidade , Transplante Ósseo/efeitos adversos , Osso e Ossos/patologia , Criança , Feminino , Seguimentos , Congelamento , Humanos , Salvamento de Membro/métodos , Masculino , Pessoa de Meia-Idade , Procedimentos de Cirurgia Plástica/efeitos adversos , Estudos Retrospectivos , Neoplasias de Tecidos Moles/mortalidade , Análise de Sobrevida , Taxa de Sobrevida , Transplante Autólogo/efeitos adversos , Adulto Jovem
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