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1.
Cancer Treat Rev ; 94: 102168, 2021 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-33730627

RESUMO

OBJECTIVE: This systematic review and meta-analysis aimed to develop an evidence-based summary of current knowledge of bone metastases (BMs) in neuroendocrine neoplasms (NENs), inform diagnosis and treatment and standardise management between institutions. METHODS: PubMed, Medline, EMBASE and meeting proceedings were searched for eligible studies reporting data on patients with BMs and NENs of any grade of differentiation and site; poorly-differentiated large/small cell lung cancer were excluded. Data were extracted and analysed using STATA v.12. Meta-analysis of proportions for calculation of estimated pooled prevalence of BM and calculation of weighted pooled frequency and weighted pooled mean for other variables of interest was performed . RESULTS: A total of 149 studies met the eligibility criteria. Pooled prevalence of BMs was 18.4% (95% CI 15.4-21.5). BMs were mainly metachronous with initial diagnosis of NEN (61.2%) and predominantly osteoblastic; around 61% were multifocal, with a predisposition in axial skeleton. PET/CT seemed to provide (together with MRI) the highest sensitivity and specificity for BM detection. Almost half of patients (46.4%) reported BM-related symptoms: pain (66%) and skeletal-related events (SREs, fracture/spinal cord compression) (26.2%; weightedweighted mean time-to-SRE 9.9 months). Management of BMs was multimodal [bisphosphonates and bone-modifying agents (45.2%), external beam radiotherapy (34.9%), surgery (14.8%)] and supported by little evidence. Overall survival (OS) from the time of diagnosis of BMs was long [weighted mean 50.9 months (95% CI 40.0-61.9)]. Patients with BMs had shorter OS [48.8 months (95% CI 37.9-59.6)] compared to patients without BMs [87.4 months (95% CI 74.9-100.0); p = 0.001]. Poor performance status and BM-related symptoms were also associated with worse OS. CONCLUSIONS: BMs in patients with NENs remain underdiagnosed and undertreated. Recommendations for management of BMs derived from current knowledge are provided. Prospective studies to inform management are required.


Assuntos
Neoplasias Ósseas/secundário , Neoplasias Ósseas/terapia , Tumores Neuroendócrinos/patologia , Tumores Neuroendócrinos/terapia , Neoplasias Ósseas/diagnóstico , Humanos , Tumores Neuroendócrinos/diagnóstico , Tumores Neuroendócrinos/secundário
2.
Medicine (Baltimore) ; 100(6): e24471, 2021 Feb 12.
Artigo em Inglês | MEDLINE | ID: mdl-33578541

RESUMO

BACKGROUND: In osteosarcoma, the lung is the most common metastatic organ. Intensive work has been made to illuminate the pathogeny, but the specific metastatic mechanism remains unclear. Thus, we conducted the study to seek to find the key genes and critical functional pathways associated with progression and treatment in lung metastasis originating from osteosarcoma. METHODS: Two independent datasets (GSE14359 and GSE85537) were screened out from the Gene Expression Omnibus (GEO) database and the overlapping differentially expressed genes (DEGs) were identified using GEO2R online platform. Subsequently, the Gene Ontology (GO) annotation and Kyoto Encyclopedia of Genes and Genomes (KEGG) pathways enrichment analysis of DEGs were conducted using DAVID. Meanwhile, the protein-protein interaction (PPI) network constructed by STRING was visualized using Cytoscape. Afterwards, the key module and hub genes were extracted from the PPI network using the MCODE and cytoHubba plugin. Moreover, the raw data obtained from GSE73166 and GSE21257 were applied to verify the expression differences and conduct the survival analyses of hub genes, respectively. Finally, the interaction network of miRNAs and hub genes constructed by ENCORI was visualized using Cytoscape. RESULTS: A total of 364 DEGs were identified, comprising 96 downregulated genes and 268 upregulated genes, which were mainly involved in cancer-associated pathways, adherens junction, ECM-receptor interaction, focal adhesion, MAPK signaling pathway. Subsequently, 10 hub genes were obtained and survival analysis demonstrated SKP2 and ASPM were closely related to poor prognosis of patients with osteosarcoma. Finally, hsa-miR-340-5p, has-miR-495-3p, and hsa-miR-96-5p were found to be most closely associated with these hub genes according to the interaction network of miRNAs and hub genes. CONCLUSION: The key genes and functional pathways identified in the study may contribute to understanding the molecular mechanisms involved in the carcinogenesis and progression of lung metastasis originating from osteosarcoma, and provide potential diagnostic and therapeutic targets.


Assuntos
Neoplasias Ósseas/patologia , Neoplasias Pulmonares/secundário , Osteossarcoma/patologia , Neoplasias Ósseas/diagnóstico , Neoplasias Ósseas/genética , Biologia Computacional , Regulação Neoplásica da Expressão Gênica/genética , Redes Reguladoras de Genes/genética , Genes Neoplásicos/genética , Marcadores Genéticos , Humanos , Neoplasias Pulmonares/diagnóstico , Neoplasias Pulmonares/genética , MicroRNAs/genética , Análise de Sequência com Séries de Oligonucleotídeos , Osteossarcoma/diagnóstico , Osteossarcoma/genética , Transdução de Sinais/genética
3.
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 , 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
4.
Nat Commun ; 12(1): 498, 2021 01 21.
Artigo em Inglês | MEDLINE | ID: mdl-33479225

RESUMO

Sarcomas are malignant soft tissue and bone tumours affecting adults, adolescents and children. They represent a morphologically heterogeneous class of tumours and some entities lack defining histopathological features. Therefore, the diagnosis of sarcomas is burdened with a high inter-observer variability and misclassification rate. Here, we demonstrate classification of soft tissue and bone tumours using a machine learning classifier algorithm based on array-generated DNA methylation data. This sarcoma classifier is trained using a dataset of 1077 methylation profiles from comprehensively pre-characterized cases comprising 62 tumour methylation classes constituting a broad range of soft tissue and bone sarcoma subtypes across the entire age spectrum. The performance is validated in a cohort of 428 sarcomatous tumours, of which 322 cases were classified by the sarcoma classifier. Our results demonstrate the potential of the DNA methylation-based sarcoma classification for research and future diagnostic applications.


Assuntos
Algoritmos , Neoplasias Ósseas/genética , Metilação de DNA , Aprendizado de Máquina , Sarcoma/genética , Neoplasias de Tecidos Moles/genética , Neoplasias Ósseas/classificação , Neoplasias Ósseas/diagnóstico , Estudos de Coortes , Variações do Número de Cópias de DNA/genética , Humanos , Internet , Reprodutibilidade dos Testes , Sarcoma/classificação , Sarcoma/diagnóstico , Sensibilidade e Especificidade , Neoplasias de Tecidos Moles/classificação , Neoplasias de Tecidos Moles/diagnóstico
5.
BMJ Case Rep ; 14(1)2021 Jan 25.
Artigo em Inglês | MEDLINE | ID: mdl-33495182

RESUMO

Sclerosing pneumocytomas are rare, benign pulmonary neoplasms that predominantly affect Asian female patients in the age category of 40-70 years, mostly non-smokers. We report on a 72-year-old Caucasian woman with chondrosarcoma of the hand who developed multiple bilateral progressive lung nodules suspicious of lung metastases. Staged lung resections were performed, and pathological diagnosis was confirmed by immunohistochemical analysis of the resected specimens. Next-generation sequencing (NGS) was used to detect gene mutations. Immunohistochemistry demonstrated sclerosing pneumocytomas, and NGS showed an IDH1 mutation. Eventually, the patient developed lung metastases for which rethoracotomy was performed. The differentiation of sclerosing pneumocytoma from lung cancer is a diagnostic challenge, and sclerosing pneumocytoma should be considered in the differential diagnosis of pulmonary nodules. Gene mutation analysis does not always show classical and common mutations, which should be kept in mind when interpreting its results.


Assuntos
Neoplasias Ósseas/diagnóstico , Condrossarcoma/diagnóstico , Neoplasias Pulmonares/diagnóstico , Ossos Metacarpais , Nódulos Pulmonares Múltiplos/diagnóstico , Neoplasias Primárias Múltiplas/diagnóstico , Hemangioma Esclerosante Pulmonar/diagnóstico , Idoso , Neoplasias Ósseas/patologia , Neoplasias Ósseas/cirurgia , Condrossarcoma/secundário , Condrossarcoma/cirurgia , Feminino , Humanos , Neoplasias Pulmonares/patologia , Neoplasias Pulmonares/secundário , Neoplasias Pulmonares/cirurgia , Imagem por Ressonância Magnética , Nódulos Pulmonares Múltiplos/patologia , Nódulos Pulmonares Múltiplos/cirurgia , Recidiva Local de Neoplasia/cirurgia , Neoplasias Primárias Múltiplas/patologia , Neoplasias Primárias Múltiplas/cirurgia , Pneumonectomia , Tomografia Computadorizada com Tomografia por Emissão de Pósitrons , Hemangioma Esclerosante Pulmonar/patologia , Hemangioma Esclerosante Pulmonar/cirurgia , Toracotomia , Tomografia Computadorizada por Raios X
7.
Methods Mol Biol ; 2226: 3-14, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33326089

RESUMO

Ewing sarcoma (EwS) is a rare bone or soft tissue tumor that occurs early in life and as such genetic variation is a major contributor to EwS risk. To date, genetic investigations have identified key somatic mutations and germline variants of importance for EwS risk. While substantial progress is being made in uncovering the genetic etiology of EwS, considerable gaps in knowledge remain. Herein, we provide a summary of methodological approaches for future genomic investigations of EwS. We anticipate this recommended analytical framework for germline and somatic investigations, along with genomic data from growing EwS case series, will aid in accelerating new genomic discoveries in EwS and expand knowledge of the genetic architecture of EwS.


Assuntos
Biomarcadores Tumorais , Neoplasias Ósseas/genética , Predisposição Genética para Doença , Variação Genética , Mutação em Linhagem Germinativa , Sarcoma de Ewing/genética , Animais , Neoplasias Ósseas/diagnóstico , Estudos de Associação Genética , Genômica/métodos , Sequenciamento de Nucleotídeos em Larga Escala , Humanos , Mutação , Proteínas de Fusão Oncogênica/genética , Sarcoma de Ewing/diagnóstico
8.
Methods Mol Biol ; 2226: 39-45, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33326092

RESUMO

Liquid biopsies enable noninvasive therapy monitoring in patients with solid tumors. Specific serum markers such as proteins, hormones, or enzymes released from tumor cells or in response to tumor growth can be used for quantification of the tumor burden. However, only a fraction of pediatric tumors has none of these serum markers, but tumor-specific genetic alterations represent reliable alternatives. Here we describe a method for using genomic fusion sequences as liquid biopsy markers in Ewing sarcoma patients.


Assuntos
Neoplasias Ósseas/diagnóstico , Biópsia Líquida , Sarcoma de Ewing/diagnóstico , Biomarcadores Tumorais , DNA Tumoral Circulante/isolamento & purificação , Humanos , Biópsia Líquida/métodos , Reação em Cadeia da Polimerase
9.
Methods Mol Biol ; 2226: 49-64, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33326093

RESUMO

The diagnosis of Ewing sarcoma requires the integration of the information generated from numerous techniques, some of them being very sophisticated. However, the first steps of the diagnostic process are crucial to achieve the maximum possible diagnostic performance. In this chapter we will review how to handle the diagnostic specimen from its collection, how to prepare it for diagnosis, how to make a complete pathology report, and provide guidance for the reasonable use of immunohistochemical techniques in this malignancy.


Assuntos
Neoplasias Ósseas/diagnóstico , Imuno-Histoquímica , Sarcoma de Ewing/diagnóstico , Biomarcadores Tumorais , Diagnóstico Diferencial , Humanos , Imuno-Histoquímica/métodos
10.
Methods Mol Biol ; 2226: 65-83, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33326094

RESUMO

The differential diagnosis of small round cell tumors (SRCT) crucially relies on the synoptic evaluation of morphology, immunohistochemical patterns, and molecular features. Though the implementation of broad RNA sequencing in diagnostic molecular pathology routines has substantially changed the standards of molecular affirmation of diagnoses, fluorescence in situ hybridization (FISH) on formalin-fixed, paraffin-embedded (FFPE) tissue sections is still an elementary tool to provide a rapid molecular corroboration of diagnoses, essentially required for therapeutic decisions. We discuss here the major FISH approaches currently employed in diagnostic molecular pathology, addressing classic Ewing sarcoma and differential diagnoses among SRCT which cannot sufficiently be ruled out by immunohistochemistry. This chapter will approach technical issues but particularly strategies and pitfalls in the interpretation of FISH patterns.


Assuntos
Biomarcadores Tumorais , Neoplasias Ósseas/diagnóstico , Técnicas de Diagnóstico Molecular , Sarcoma de Ewing/diagnóstico , Neoplasias Ósseas/etiologia , Aberrações Cromossômicas , Diagnóstico Diferencial , Humanos , Imuno-Histoquímica , Hibridização in Situ Fluorescente , Microscopia de Fluorescência , Sarcoma de Ewing/etiologia
11.
Methods Mol Biol ; 2226: 85-103, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33326095

RESUMO

Ewing sarcoma is a rare and aggressive tumor that affects children and young adults. Ewing sarcomas are characterized by specific chromosomal translocations that give rise to fusion transcripts that codify for aberrant transcription factors. More than 95% of Ewing sarcoma harbor translocations that produce the fusion of the EWSR1 gene with the transcription factors FLI1 or ERG. This feature can be used to diagnose this entity unambiguously.In this chapter we describe a RT-PCR method that allows for the detection of the most frequent alterations with elevated specificity and sensitivity which is able to distinguish among the different types of fusions. The method is fast and economical, and can be carried out with the conventional equipment available in any molecular biology laboratory.


Assuntos
Biomarcadores Tumorais , Neoplasias Ósseas/diagnóstico , Técnicas de Diagnóstico Molecular , Reação em Cadeia da Polimerase Via Transcriptase Reversa , Sarcoma de Ewing/diagnóstico , Neoplasias Ósseas/genética , Loci Gênicos , Humanos , Proteínas de Fusão Oncogênica/genética , Reação em Cadeia da Polimerase Via Transcriptase Reversa/métodos , Sarcoma de Ewing/genética , Translocação Genética
12.
Methods Mol Biol ; 2226: 105-116, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33326096

RESUMO

Molecular testing of pathognomonic gene fusions is mandatory for small round cell tumor diagnosis, including Ewing sarcoma which is indeed defined by a variety of chimeric genes. Reference laboratories are increasingly implementing NGS-based techniques to overcome several limitations of conventional singleplex determinations. We have been early adopters of a targeted-RNA sequencing method based on Anchored multiplex PCR, which allows assessing several fusion transcripts simultaneously with previous knowledge of only one partner gene. Here we describe in detail our protocol and tips for nucleic acid extraction, library preparation, sequencing, and reporting of gene fusions.


Assuntos
Biomarcadores Tumorais , Neoplasias Ósseas/diagnóstico , Sequenciamento de Nucleotídeos em Larga Escala , Técnicas de Diagnóstico Molecular , Sarcoma de Ewing/diagnóstico , Neoplasias Ósseas/genética , Análise de Dados , Sequenciamento de Nucleotídeos em Larga Escala/métodos , Humanos , Técnicas de Diagnóstico Molecular/métodos , Reação em Cadeia da Polimerase Multiplex , Proteínas de Fusão Oncogênica/genética , Medicina de Precisão/métodos , Reação em Cadeia da Polimerase em Tempo Real , Sarcoma de Ewing/genética
13.
Methods Mol Biol ; 2226: 119-138, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33326097

RESUMO

Gene expression and knockdown systems are powerful tools to study the function of single genes and their pathway interaction. Plasmid transfection and viral transduction have revolutionized the field of molecular biology and paved the ground for various gene-editing strategies such as TALEN, zinc finger nucleases, and ultimately CRISPR. In Ewing sarcoma (EwS), almost as many genes are repressed by the expression of EWSR1-FLI1 as are upregulated by the fusion oncogene. Here we present a useful point-to-point protocol for the generation of transgene expression systems in EwS that allow (conditional) reexpression of a gene of interest. We provide an extensive instruction on molecular cloning, plasmid generation, viral transduction, and expression validation. Finally, we address common problems and highlight potential pitfalls, which can easily be avoided by thoughtful guidance.


Assuntos
Biomarcadores Tumorais , Neoplasias Ósseas/genética , Regulação Neoplásica da Expressão Gênica , Sarcoma de Ewing/genética , Neoplasias Ósseas/diagnóstico , Clonagem Molecular , Elementos Facilitadores Genéticos , Vetores Genéticos/genética , Humanos , Repetições de Microssatélites , Proteínas de Fusão Oncogênica/genética , Plasmídeos/genética , Regiões Promotoras Genéticas , Reação em Cadeia da Polimerase em Tempo Real , Sarcoma de Ewing/diagnóstico , Transdução Genética
14.
Int J Nanomedicine ; 15: 10171-10181, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-33363373

RESUMO

Background: In recent years, nanomaterials have justified their dissemination for biosensor application towards the sensitive and selective detections of clinical biomarkers at the lower levels. MXene is a two-dimensional layered transition metal, attractive for biosensing due to its chemical, physical and electrical properties along with the biocompatibility. Materials and Methods: This work was focused on diagnosing osteosarcoma (OS), a common bone cancer, on MXene-modified multiple junction triangles by dielectrode sensing. Survivin protein gene is highly correlated with OS, identified on this sensing surface. Capture DNA was immobilized on MXene by using 3-glycidoxypropyltrimethoxysilane as an amine linker and duplexed by the target DNA sequence. Results: The limitation and sensitivity of detection were found as 1 fM with the acceptable regression co-efficient value (y=1.0037⨰ + 0.525; R2=0.978) and the current enhancement was noted when increasing the target DNA concentrations. Moreover, the control sequences of single- and triple-mismatched and noncomplementary to the target DNA sequences failed to hybridize on the capture DNA, confirming the specificity. In addition, different batches were prepared with capture probe immobilized sensing surfaces and proved the efficient reproducibility. Conclusion: This microgap device with Mxene-modified multiple junction triangles dielectrode surface is beneficial to quantify the survivin gene at its lower level and diagnosing OS complication levels.


Assuntos
Biomarcadores Tumorais/metabolismo , Técnicas Biossensoriais/métodos , Neoplasias Ósseas/diagnóstico , Osteossarcoma/diagnóstico , Elementos de Transição/química , Neoplasias Ósseas/patologia , Linhagem Celular Tumoral , DNA de Neoplasias/metabolismo , Eletrodos , Humanos , Microeletrodos , Nanoestruturas/química , Osteossarcoma/patologia , Espectroscopia Fotoeletrônica , Reprodutibilidade dos Testes , Survivina/metabolismo
15.
BMJ Case Rep ; 13(12)2020 Dec 22.
Artigo em Inglês | MEDLINE | ID: mdl-33370985

RESUMO

Osteochondroma of the talus is a rare entity that can cause pain, swelling, restriction of movements, synovitis and tarsal tunnel syndrome (TTS). We present three such cases with varying presentation. Case 1 presented with synovitis of the ankle along with a bifocal origin of the talar osteochondroma. Case 2 presented with TTS as a result of compression of the posterior tibial nerve. Case 3 presented with deformity of the foot. In all the three cases, the mass was excised en bloc and histologically proven to be osteochondroma. In case 3, the ankle joint was reconstructed with plate, bone graft and arthrodesis of the inferior tibiofibular joint. All the three cases had good clinical outcomes.


Assuntos
Neoplasias Ósseas/diagnóstico , Deformidades Adquiridas do Pé/etiologia , Osteocondroma/diagnóstico , Sinovite/etiologia , Tálus/patologia , Síndrome do Túnel do Tarso/etiologia , Adolescente , Adulto , Articulação do Tornozelo/diagnóstico por imagem , Articulação do Tornozelo/patologia , Articulação do Tornozelo/cirurgia , Artrodese/instrumentação , Neoplasias Ósseas/complicações , Neoplasias Ósseas/patologia , Neoplasias Ósseas/cirurgia , Placas Ósseas , Transplante Ósseo , Criança , Feminino , Deformidades Adquiridas do Pé/cirurgia , Humanos , Masculino , Osteocondroma/complicações , Osteocondroma/patologia , Osteocondroma/cirurgia , Osteotomia , Sinovite/patologia , Sinovite/cirurgia , Tálus/diagnóstico por imagem , Tálus/cirurgia , Síndrome do Túnel do Tarso/cirurgia , Tomografia Computadorizada por Raios X , Resultado do Tratamento
16.
Medicine (Baltimore) ; 99(46): e23050, 2020 Nov 13.
Artigo em Inglês | MEDLINE | ID: mdl-33181669

RESUMO

Patients diagnosed with Ewing sarcoma (ES) usually experience poor outcomes. Accurate prediction of ES patients' prognosis is essential to improve their survival. Given that ES is a relatively rare tumor with a low incidence, we aim at developing a prognostic nomogram of ES patients based on a large sample analysis.We used the Surveillance, Epidemiology, and End Results (SEER) database to screen eligible patients diagnosed ES of bone. This retrospective study presented the clinicopathological characteristics and prognosis of ES. We randomly assigned all ES patients to 2 sets (training set and validation set) with an equal number of patients. In order to identify independent factors of survival, we performed univariate and multivariate Cox analysis in the training set. Then, we constructed novel nomograms to predict survival of ES patients by integrating significant independent variables from the training set. The prognostic performance of constructed nomograms was examined using concordance index (C-index) and calibration curves in both training and validation set.We included a total of 988 eligible cases diagnosed ES of bone between 2000 and 2015. Age >18 years, distant metastasis, tumor size >10 cm, and no surgery were independent risk factors for poorer survival. Our survival prediction nomograms were established based on those 4 independent risk factors. Good calibration plots were achieved in internal and external validation. The internal validation C-indexes of the nomogram for overall survival (OS) and cancer-specific survival (CSS) were 0.733 and 0.737, respectively. Similar good results were also achieved in external validation setting.The established nomograms show good performance and allow for better evaluating the prognosis of ES patients and recommending appropriate instructions.


Assuntos
Neoplasias Ósseas/diagnóstico , Regras de Decisão Clínica , Nomogramas , Medição de Risco/métodos , Sarcoma de Ewing/diagnóstico , Adolescente , Adulto , Neoplasias Ósseas/mortalidade , Feminino , Humanos , Estimativa de Kaplan-Meier , Masculino , Análise Multivariada , Estadiamento de Neoplasias , Valor Preditivo dos Testes , Prognóstico , Modelos de Riscos Proporcionais , Fatores de Risco , Programa de SEER , Sarcoma de Ewing/mortalidade , Adulto Jovem
17.
Crit Rev Oncol Hematol ; 155: 103108, 2020 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-33038694

RESUMO

Soft tissue sarcomas in elderly patients beyond the age of 65 years account for almost 50 % of the sarcomas of the total patient population in this rare tumor entity. Distinct clinical and biological characteristics of the different sarcoma subtypes have to be taken into account in the various age groups. Old age should not per se be a contraindication to chemotherapy administration or to undergo surgery; however, particular aspects regarding safety, comorbidities, toxicity, and quality of life have to be considered. The use of a geriatric assessment and especially the use of predictive tools for estimating the expected toxicity may be helpful in this challenging situation. On the other side, osteosarcomas in the elderly patient population represent an extreme rarity. The adequate primary surgical care of these patients seems crucial in terms of prognosis as the addition of chemotherapy does not significantly improve patients' overall survival.


Assuntos
Neoplasias Ósseas , Osteossarcoma , Sarcoma , Neoplasias de Tecidos Moles , Idoso , Neoplasias Ósseas/diagnóstico , Neoplasias Ósseas/tratamento farmacológico , Neoplasias Ósseas/epidemiologia , Humanos , Osteossarcoma/diagnóstico , Osteossarcoma/tratamento farmacológico , Qualidade de Vida , Sarcoma/diagnóstico , Sarcoma/tratamento farmacológico , Sarcoma/epidemiologia , Neoplasias de Tecidos Moles/diagnóstico , Neoplasias de Tecidos Moles/tratamento farmacológico
18.
Medicine (Baltimore) ; 99(40): e22505, 2020 Oct 02.
Artigo em Inglês | MEDLINE | ID: mdl-33019449

RESUMO

RATIONALE: Neuroblastoma (NB) can occur in any part of the sympathetic nervous system, and it is highly heterogeneous. Tumors that only involve bone marrow and bone lesions without solid masses have rarely been reported. PATIENT CONCERNS: A 2-year-old girl child presented with recurrent fever, accompanied by pain in both lower limbs for more than 1 month. DIAGNOSE: Bone marrow examination revealed NB cell invasion. Femoral and multiple vertebral lesions were observed by MRI, while head MRI, chest CT, abdominal CT, and pelvic CT showed no solid mass. INTERVENTIONS: The child received the standard therapy for high-risk NB. OUTCOMES: She was sensitive to the initial chemotherapy protocol. Two years later, a bone marrow examination confirmed NB recurrence. LESSONS: The prognosis of this special type of NB was not improved mainly based on common chemotherapy and local radiotherapy, and new treatment strategies should be explored.


Assuntos
Medula Óssea/patologia , Neoplasias Ósseas/diagnóstico por imagem , Neoplasias Ósseas/patologia , Neuroblastoma/patologia , Exame de Medula Óssea , Neoplasias Ósseas/diagnóstico , Pré-Escolar , Feminino , Humanos , Imagem por Ressonância Magnética , Neuroblastoma/diagnóstico , Tomografia Computadorizada por Raios X
19.
J Cancer Res Ther ; 16(4): 713-717, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32930108

RESUMO

Background and Objectives: Currently, one of the most useful prognostic indicators in Ewing sarcomas (ES) is the presence of metastatic disease at diagnosis. According to various clinical guidelines, the assessment of bone marrow (BM) metastases, using light microscopy examination of bone marrow aspirates and biopsies (BMAB) is mandatory. However, the prognostic value of BM positivity is discussed controversially. Therefore, the primary aim of this study was to retrospectively review BM samples from patients with ES. Materials and Methods: This retrospective single centre study included 31 patients that were newly diagnosed with ES between 2000 and 2014. Twenty-seven patients had skeletal ES and in 4 patients the tumour was localized in the soft tissue only. Metastases at diagnosis were present in 5 out of 31 patients. BM samples were morphologically and immunohistochemically searched and screened for the presence or absence of BM metastases. Furthermore, in 15 of the 31 patients BM samples were still available and were reanalysed, using nested-polymerase chain reaction. Results: All BM samples of our 31 ES patients, including the 5 metastatic patients, were, morphologically and immunohistochemically tested negative for tumour cell appearance. The nested-PCR results were also negative in all of our 15 retested patients, including two patients with metastatic disease. Conclusions: Based on our results and on the contradictory results reported in the literature we recommend a re-evaluation of the necessity and the prognostic value of BMAB in the initial staging process of newly diagnosed ES patients.


Assuntos
Neoplasias Ósseas/patologia , Sarcoma de Ewing/patologia , Adolescente , Adulto , Biópsia por Agulha Fina , Exame de Medula Óssea/métodos , Neoplasias Ósseas/diagnóstico , Neoplasias Ósseas/genética , Neoplasias Ósseas/metabolismo , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Estadiamento de Neoplasias , Prognóstico , Estudos Retrospectivos , Sarcoma de Ewing/diagnóstico , Sarcoma de Ewing/genética , Sarcoma de Ewing/metabolismo , Punção Espinal , Adulto Jovem
20.
J Cancer Res Ther ; 16(4): 926-929, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32930143

RESUMO

Benign and malignant bone tumors arise in small bones of the hands and feet. Nevertheless, secondary deposits at these sites are extremely rare. We report a peculiar case of an adult man who presented with thumb swelling, which was later discovered to be a metastasis from renal cell carcinoma. Such cases have a sinister prognosis with a survival rate of 6-12 months from the time of diagnosis. We intend to discuss the diagnostic dilemma and treatment of acrometastases.


Assuntos
Neoplasias Ósseas/secundário , Carcinoma de Células Renais/diagnóstico , Neoplasias Renais/diagnóstico , Neoplasias Pulmonares/secundário , Polegar/patologia , Neoplasias Ósseas/diagnóstico , Neoplasias Ósseas/cirurgia , Carcinoma de Células Renais/patologia , Carcinoma de Células Renais/cirurgia , Humanos , Neoplasias Renais/patologia , Neoplasias Renais/cirurgia , Neoplasias Pulmonares/diagnóstico , Neoplasias Pulmonares/cirurgia , Masculino , Pessoa de Meia-Idade , Prognóstico
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