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1.
J Gastrointest Oncol ; 12(5): 2150-2156, 2021 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-34790381

RESUMO

BACKGROUND: Cancer of the lower intestinal tract, although relatively common, rarely metastasizes to the skeleton. The treatment of metastatic bone disease due to colorectal cancer has thus been poorly described and treatment decisions are therefore difficult. The aim of this study was to describe the outcome of orthopedic surgery in patients with pathological fractures from colorectal cancer and investigate factors that correlate with patient survival, since it influences treatment decisions. METHODS: Retrospective review of data collected in a prospectively collected database. 36 patients (38 fractures) who underwent surgery between 2000 and 2019 for metastatic bone disease caused by colorectal cancer were included. RESULTS: Most metastases were localized in the axial skeleton and 33/36 patients already had visceral metastases. Patients with pathological fractures from colorectal cancer had poor prognosis, with only 5/36 surviving more than 1 year, median survival being 3 months. Patients presenting with a single skeletal metastasis had a superior overall survival (P≤0.001). Post-operative complications were common, noted in 11 patients, and the surgical failure rate was considerable. CONCLUSIONS: Although relatively rare, bone metastases should be suspected in patients with colorectal cancer presenting with signs and symptoms of spinal cord compression or skeletal pain. In this case, the presence of a solitary skeletal lesion is a favorable prognostic sign. Awareness for local complications after surgery should be high.

2.
Clin Sarcoma Res ; 10(1): 22, 2020 Nov 17.
Artigo em Inglês | MEDLINE | ID: mdl-33292545

RESUMO

BACKGROUND: We recently reported outcomes from a Scandinavian Sarcoma Group adjuvant study (SSG XX group A) conducted on localized and operable high risk soft tissue sarcoma (STS) of the extremities and trunk wall. SSG XX, group B, comprised of patients in a defined cohort with locally advanced STS considered at high risk for intralesional surgery. These patients received preoperative accelerated radiotherapy, together with neoadjuvant and adjuvant chemotherapy. Herein we report the results of this group B. METHODS: Twenty patients with high-grade, locally advanced and deep STS located in lower extremities (n = 12), upper extremities (5) or trunk wall (3) were included. The median age was 59 years and 14 patients were males. The treatment regimen consisted of 6 cycles of doxorubicin (60 mg/m2) and ifosfamide (6 g/m2), with three cycles given neoadjuvantly, and preoperative radiotherapy (1, 8 Gyx2/daily to 36 Gy) between cycles 2 and 3. After a repeated MRI surgery was then conducted, and the remaining 3 chemotherapy cycles were given postoperatively at 3 weeks intervals. Survival data, local control, toxicity of chemotherapy and postoperative complications are presented. RESULTS: Median follow-up time for metastasis-free survival (MFS) was 2.8 years (range 0.3-10.4). The 5-year MFS was 49.5% (95% confidence interval [CI] 31.7-77.4). The median follow-up time was 5.4 years (range 0.3-10.4) for overall survival (OS). The 5-year OS was 64.0% (95% CI 45.8-89.4). The median tumour size was 13 cm, with undifferentiated pleomorphic sarcoma (n = 10) and synovial sarcoma (n = 6) diagnosed most frequently. All patients completed surgery. Resection margins were R0 in 19 patients and R1 in 1 patient. No patients had evidence of disease progression preoperatively. Three patients experienced a local recurrence, in 2 after lung metastases had already been diagnosed. Eleven patients (55%) had postoperative wound problems (temporary in 8 and persistent in 3). CONCLUSIONS: Preoperative chemotherapy and radiotherapy were associated with temporary wound-healing problems. Survival outcomes, local control and toxicities were deemed satisfactory when considering the locally advanced sarcoma disease status at primary diagnosis. Trial registration This study was registered at ClinicalTrials.gov Identifier NCT00790244 and with European Union Drug Regulating Authorities Clinical Trials No. EUDRACT 2007-001152-39.

3.
Cancer Genet ; 241: 34-41, 2020 02.
Artigo em Inglês | MEDLINE | ID: mdl-31870844

RESUMO

The dermatofibrosarcoma protuberans family of tumors (DPFT) comprises cutaneous soft tissue neoplasms associated with aberrant PDGFBR signaling, typically through a COL1A1-PDGFB fusion. The aim of the present study was to obtain a better understanding of the chromosomal origin of this fusion and to assess the spectrum of secondary mutations at the chromosome and nucleotide levels. We thus investigated 42 tumor samples from 35 patients using chromosome banding, fluorescence in situ hybridization, single nucleotide polymorphism arrays, and/or massively parallel sequencing (gene panel, whole exome and transcriptome sequencing) methods. We confirmed the age-associated differences in the origin of the COL1A1-PDGFB fusion and could show that it in most cases must arise after DNA synthesis, i.e., in the S or G2 phase of the cell cycle. Whereas there was a non-random pattern of secondary chromosomal rearrangements, single nucleotide variants seem to have little impact on tumor progression. No clear genomic differences between low-grade and high-grade DPFT were found, but the number of chromosomes and chromosomal imbalances as well as the frequency of 9p deletions all tended to be greater among the latter. Gene expression profiling of tumors with COL1A1-PDGFB fusions associated with unbalanced translocations or ring chromosomes identified several transcriptionally up-regulated genes in the amplified regions of chromosomes 17 and 22, including TBX2, PRKCA, MSI2, SOX9, SOX10, and PRAME.


Assuntos
Cromossomos Humanos Par 17/genética , Cromossomos Humanos Par 22/genética , Dermatofibrossarcoma/genética , Proteínas de Fusão Oncogênica/genética , Neoplasias Cutâneas/genética , Adolescente , Adulto , Idoso , Criança , Pré-Escolar , Dermatofibrossarcoma/patologia , Feminino , Fase G2/genética , Perfilação da Expressão Gênica , Regulação Neoplásica da Expressão Gênica , Genômica , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Pele/patologia , Neoplasias Cutâneas/patologia , Adulto Jovem
4.
Int J Spine Surg ; 13(2): 186-191, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-31131219

RESUMO

BACKGROUND: We investigated the outcome of surgical treatment of patients with radiosensitive hematological malignancies presenting with spinal cord compression. METHODS: Retrospective review of 50 patients who had treatment between 1993 and 2012. RESULTS: The neurological outcome was favorable in 35 patients, stable in 12, whereas 3 patients deteriorated. Decompression within 48 hours from referral was associated with a superior neurological recovery (P = .001). Complications were noted in 11 patients, and 6 of these underwent secondary surgery. Early (30-day) mortality was 8%. Radiotherapy was associated with increased incidence of complications (χ2 = 0.009). Patients who had low blood hemoglobin preoperatively as well as those who remained totally bedridden postoperatively had an inferior overall survival rate (P < .001). CONCLUSION: Patients with cord compression from hematological malignancy benefit from early surgical decompression. There is an inherent high risk for complications, which increases further if radiotherapy is given. Patients failing to ambulate after surgery have a poor prognosis.

5.
Br J Cancer ; 120(4): 435-443, 2019 02.
Artigo em Inglês | MEDLINE | ID: mdl-30745580

RESUMO

BACKGROUND: Heterogeneity and low incidence comprise the biggest challenge in sarcoma diagnosis and treatment. Chemotherapy, although efficient for some sarcoma subtypes, generally results in poor clinical responses and is mostly recommended for advanced disease. Specific genomic aberrations have been identified in some sarcoma subtypes but few of them can be targeted with approved drugs. METHODS: We cultured and characterised patient-derived sarcoma cells and evaluated their sensitivity to 525 anti-cancer agents including both approved and non-approved drugs. In total, 14 sarcomas and 5 healthy mesenchymal primary cell cultures were studied. The sarcoma biopsies and derived cells were characterised by gene panel sequencing, cancer driver gene expression and by detecting specific fusion oncoproteins in situ in sarcomas with translocations. RESULTS: Soft tissue sarcoma cultures were established from patient biopsies with a success rate of 58%. The genomic profile and drug sensitivity testing on these samples helped to identify targeted inhibitors active on sarcomas. The cSrc inhibitor Dasatinib was identified as an active drug in sarcomas carrying chromosomal translocations. The drug sensitivity of the patient sarcoma cells ex vivo correlated with the response to the former treatment of the patient. CONCLUSIONS: Our results show that patient-derived sarcoma cells cultured in vitro are relevant and practical models for genotypic and phenotypic screens aiming to identify efficient drugs to treat sarcoma patients with poor treatment options.


Assuntos
Sarcoma/tratamento farmacológico , Quinases da Família src/antagonistas & inibidores , Adulto , Proteína Tirosina Quinase CSK , Linhagem Celular Tumoral , Resistencia a Medicamentos Antineoplásicos , Feminino , Humanos , Masculino , Sarcoma/genética , Sarcoma/patologia
6.
Eur J Cancer ; 99: 78-85, 2018 08.
Artigo em Inglês | MEDLINE | ID: mdl-29929092

RESUMO

PURPOSE: To investigate the outcome following adjuvant doxorubicin and ifosfamide in a prospective non-randomised study based on a soft tissue sarcoma (STS) patient subgroup defined by specific morphological characteristics previously shown to be at a high-risk of metastatic relapse. The expected 5-year cumulative incidence of metastases in patients with this risk profile has previously been reported to be about 50% without adjuvant chemotherapy. METHODS: High-risk STS was defined as high-grade morphology (according to the Fédération Nationale des Centres de Lutte Contre le Cancer [FNCLCC] grade II-III) and either vascular invasion or at least two of the following criteria: tumour size ≥8.0 cm, infiltrative growth and necrosis. Six cycles of doxorubicin (60 mg/m2) and ifosfamide (6 g/m2) were given. Postoperative accelerated radiotherapy was applied and scheduled between cycles 3 and 4. RESULTS: For the 150 eligible patients, median follow-up time for metastases-free survival was 3.9 years (range 0.2-8.7). Five-year metastases-free survival (MFS) was 70.4% (95% confidence interval [CI]: 63.1-78.4) with a local recurrence rate of 14.0% (95% CI: 7.8-20.2). For overall survival (OS), the median follow-up time was 4.4 years (range: 0.2-8.7). The five-year OS was 76.1% (95% CI: 68.8-84.2). Tumour size, deep location and reduced dose intensity (<80%) had a negative impact on survival. Toxicity was moderate with no treatment-related death. CONCLUSIONS: A benefit of adjuvant chemotherapy, compared to similar historical control groups, was demonstrated in STS patients with defined poor prognostic factors. Vascular invasion, tumour size, growth pattern and necrosis may identify patients in need of adjuvant chemotherapy.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Recidiva Local de Neoplasia/epidemiologia , Sarcoma/epidemiologia , Neoplasias de Tecidos Moles/terapia , Adulto , Idoso , Protocolos de Quimioterapia Combinada Antineoplásica/farmacologia , Quimioterapia Adjuvante/métodos , Relação Dose-Resposta a Droga , Doxorrubicina/farmacologia , Doxorrubicina/uso terapêutico , Feminino , Seguimentos , Humanos , Ifosfamida/farmacologia , Ifosfamida/uso terapêutico , Incidência , Masculino , Pessoa de Meia-Idade , Invasividade Neoplásica/patologia , Recidiva Local de Neoplasia/patologia , Recidiva Local de Neoplasia/prevenção & controle , Estudos Prospectivos , Radioterapia Adjuvante/métodos , Fatores de Risco , Sarcoma/irrigação sanguínea , Sarcoma/prevenção & controle , Sarcoma/secundário , Neoplasias de Tecidos Moles/irrigação sanguínea , Neoplasias de Tecidos Moles/mortalidade , Neoplasias de Tecidos Moles/patologia , Análise de Sobrevida , Resultado do Tratamento , Adulto Jovem
7.
Acta Orthop ; 89(2): 229-233, 2018 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-29231771

RESUMO

Background and purpose - A significant number of patients with spinal metastases are treated non-surgically, but may need surgical treatment at a later stage due to progression of symptoms. Therefore, we investigated the need for late surgical decompression in patients with spinal metastasis who were initially deemed as non-surgical candidates, as well as the outcome of late surgery. Patients and methods - 116 patients who were referred to the orthopedic oncology department between 2002 and 2011 due to spinal metastasis with neurologic symptoms were deemed to be non-surgical candidates. The primary reason was minor neurologic deficits in 40 patients (M) and short survival (S) in 76 patients. Results - 8 patients underwent a late operation due to progression of the neurologic symptoms, all of them belonged to group M. M-patients with a modified Bauer score of less than 2 had both an inferior survival as well as a higher risk for late surgery. Postoperative improvement in neurologic function was noted in 5/8 operated patients, whilst 2 patients had stationary symptoms and 1 deteriorated. Interpretation - The need for late surgery arises in a minority of patients with spinal metastasis primarily treated non-surgically, and only in patients with minor neurologic compromise rather than poor general condition. An established prognostic score (modified Bauer) can be used to guide decision-making. Late surgical decompression is effective in restoring the neurologic status.


Assuntos
Descompressão Cirúrgica , Vértebras Lombares , Compressão da Medula Espinal/cirurgia , Neoplasias da Coluna Vertebral/secundário , Neoplasias da Coluna Vertebral/cirurgia , Vértebras Torácicas , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Seleção de Pacientes , Estudos Retrospectivos , Compressão da Medula Espinal/etiologia , Neoplasias da Coluna Vertebral/mortalidade , Taxa de Sobrevida , Resultado do Tratamento
8.
Acta Orthop ; 88(3): 341-347, 2017 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-28266233

RESUMO

Purpose - We wanted to examine the potential of the Scandinavian Sarcoma Group (SSG) Central Register, and evaluate referral and treatment practice for soft-tissue sarcomas in the extremities and trunk wall (STS) in the Nordic countries. Background - Based on incidence rates from the literature, 8,150 (7,000-9,300) cases of STS of the extremity and trunk wall should have been diagnosed in Norway, Finland, Iceland, and Sweden from 1987 through 2011. The SSG Register has 6,027 cases registered from this period, with 5,837 having complete registration of key variables. 10 centers have been reporting to the Register. The 5 centers that consistently report treat approximately 90% of the cases in their respective regions. The remaining centers have reported all the patients who were treated during certain time periods, but not for the entire 25-year period. Results - 59% of patients were referred to a sarcoma center untouched, i.e. before any attempt at open biopsy. There was an improvement from 52% during the first 5 years to 70% during the last 5 years. 50% had wide or better margins at surgery. Wide margins are now achieved less often than 20 years ago, in parallel with an increase in the use of radiotherapy. For the centers that consistently report, 97% of surviving patients are followed for more than 4 years. Metastasis-free survival (MFS) increased from 67% to 73% during the 25-year period. Interpretation - The Register is considered to be representative of extremity and trunk wall sarcoma disease in the population of Scandinavia, treated at the reporting centers. There were no clinically significant differences in treatment results at these centers.


Assuntos
Sarcoma/terapia , Neoplasias de Tecidos Moles/terapia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Biópsia , Criança , Pré-Escolar , Extremidades , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Pessoa de Meia-Idade , Gradação de Tumores , Recidiva Local de Neoplasia/epidemiologia , Encaminhamento e Consulta/estatística & dados numéricos , Sistema de Registros , Sarcoma/diagnóstico , Sarcoma/epidemiologia , Sarcoma/patologia , Sarcoma/secundário , Países Escandinavos e Nórdicos/epidemiologia , Neoplasias de Tecidos Moles/diagnóstico , Neoplasias de Tecidos Moles/epidemiologia , Neoplasias de Tecidos Moles/patologia , Tronco
9.
J Bone Joint Surg Am ; 98(3): 199-210, 2016 Feb 03.
Artigo em Inglês | MEDLINE | ID: mdl-26842410

RESUMO

BACKGROUND: Treatment of Ewing sarcoma of the pelvic bones remains one of the most difficult tasks in the treatment of bone sarcomas. Whether surgery or radiation therapy is the best local treatment is still a matter of debate. The aim of the present study was to compare sacral and nonsacral sites with regard to the treatment and outcome of pelvic Ewing sarcomas. METHODS: Patients with Ewing sarcoma of the osseous pelvis diagnosed between 1986 and 2011 were identified through the Scandinavian Sarcoma Group registry. Data regarding tumor size, local treatment (surgery or radiation therapy), metastatic disease, surgical margins, local recurrence, and overall survival were analyzed. RESULTS: Of the 117 patients examined, eighty-eight had tumors in the innominate bones and twenty-nine, in the sacrum. Radiation therapy was the sole local treatment for 40% of the innominate bone tumors in contrast to 79% of the sacral tumors. The five-year disease-free survival rate in the latter group (66%) was greater than that in the group with tumors in the innominate bones (40%) (p = 0.02 adjusted for size). CONCLUSIONS: Disease-free survival among patients with Ewing sarcoma was improved when the tumor was localized in the sacrum compared with the innominate bones, where these tumors are generally larger. Local radiation therapy alone appears to result in good local tumor control and may be the treatment of choice for sacral tumors.


Assuntos
Ossos Pélvicos , Sacro , Sarcoma de Ewing/mortalidade , Neoplasias Ósseas/mortalidade , Neoplasias Ósseas/patologia , Neoplasias Ósseas/radioterapia , Neoplasias Ósseas/cirurgia , Intervalo Livre de Doença , Feminino , Humanos , Masculino , Recidiva Local de Neoplasia , Prognóstico , Estudos Prospectivos , Sarcoma de Ewing/patologia , Sarcoma de Ewing/radioterapia , Adulto Jovem
10.
J Bone Joint Surg Am ; 97(21): 1756-66, 2015 Nov 04.
Artigo em Inglês | MEDLINE | ID: mdl-26537163

RESUMO

BACKGROUND: The Swedish Cancer Registry (founded in 1958) constitutes a unique resource for epidemiological studies of giant cell tumor of bone with potential for use for population-based studies of incidence over time. The aim of this study was to provide what we believe is the first modern population-based assessment of the incidence trends of giant cell tumor, a unique osteoclastogenic lytic stromal tumor with both benign and malignant histological forms, and to compare the findings with data from the same registry on osteosarcoma, a tumor that may display similar histological characteristics. METHODS: Cases were identified with use of codes for pathological bone tumor (International Classification of Diseases [ICD]-7 196). Specific morphological coding distinguishes benign (PAD 741) from malignant giant cell tumor (PAD 746) and osteosarcoma (PAD 766). RESULTS: During the period of 1958 to 2011, 4625 bone tumors were reported, including 505 giant cell tumors (383 benign and 122 malignant) and 1152 osteosarcomas. From 1958 to 1982 the ratio of malignant to benign giant cell tumors was 1.3, whereas from 1983 to 2011 the ratio inverted to 0.09, suggesting a change in the reporting or diagnosis of malignant or benign cases. Cases of giant cell tumor diagnosed from 1983 to 2011 displayed an age and sex distribution (median age at diagnosis, 34.0 years; 54% female) that were consistent with those in large published case series but differed from those in 1958 to 1982 (median age at diagnosis, 31.5 years; 48% female). The most current data (1983 to 2011) showed the giant cell tumor incidence in Sweden to be 1.3 per million per year, while the osteosarcoma incidence was 2.3 per million per year. CONCLUSIONS: Early Swedish Cancer Registry data (1958 to 1982) revealed a higher proportion of malignant giant cell tumors than seen in large sequential case series and a distinct age and sex profile compared with more recent data (1983 to 2011). This likely represents changes in the diagnostic workup and introduction of multidisciplinary review of giant-cell-containing tumors around 1982. Recent data may reflect the impact of expert centralized biopsy and multidisciplinary case review and more comprehensive reporting of benign giant cell tumors.


Assuntos
Neoplasias Ósseas/epidemiologia , Tumor de Células Gigantes do Osso/epidemiologia , Osteossarcoma/epidemiologia , Adolescente , Adulto , Distribuição por Idade , Neoplasias Ósseas/patologia , Criança , Pré-Escolar , Feminino , Tumor de Células Gigantes do Osso/patologia , Humanos , Incidência , Lactente , Recém-Nascido , Estudos Longitudinais , Masculino , Pessoa de Meia-Idade , Osteossarcoma/patologia , Sistema de Registros , Estudos Retrospectivos , Distribuição por Sexo , Suécia/epidemiologia , Fatores de Tempo , Adulto Jovem
11.
J Surg Oncol ; 112(8): 853-60, 2015 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-26482729

RESUMO

AIMS: The objectives of this study were to present changes in referral patterns, treatment and survival in patients with high-grade malignant bone sarcoma in Sweden and Norway based on data in the Scandinavian Sarcoma Group (SSG) Central Register. METHOD: Data on 1,437 patients with diagnosis 1986-2010 was analyzed. RESULTS: Osteosarcoma was the most frequentl diagnosis (45%), followed by Ewing sarcoma (21%) and chondrosarcoma (17%). Thirty-one percent of Swedish and 41% of Norwegian patients had tumors in the axial skeleton. Eighty-six percent of extremity tumors and 66% of axial tumors were referred to a sarcoma center prior to unplanned surgery or biopsy. During the past decade, limb salvage surgery has risen from under 50% to over 80%. Five-year overall survival in non-metastatic osteosarcoma was 70% for extremity tumors, and 35% for axial tumors. No improvement in osteosarcoma survival was observed during the last decade. Five-year survival in Ewing sarcoma improved from 50% to 69%. CONCLUSION: Referral patterns in bone sarcomas have improved. However, greater efforts should be dedicated to improving referral of patients with possible tumors in the axial skeleton to multidisciplinary teams (MDTs). Overall survival of patients with high-grade malignant bone sarcomas in Sweden and Norway is in line with other reports.


Assuntos
Neoplasias Ósseas/diagnóstico , Neoplasias Ósseas/terapia , Encaminhamento e Consulta/estatística & dados numéricos , Sistema de Registros , Sarcoma/diagnóstico , Sarcoma/terapia , Adolescente , Adulto , Idoso , Neoplasias Ósseas/mortalidade , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Noruega/epidemiologia , Sarcoma/mortalidade , Suécia/epidemiologia , Fatores de Tempo , Resultado do Tratamento , Adulto Jovem
12.
Acta Orthop ; 86(6): 690-4, 2015.
Artigo em Inglês | MEDLINE | ID: mdl-26220078

RESUMO

BACKGROUND AND PURPOSE: Metastases engaging the acetabulum result in significant disability. We investigated the outcome after curettage and reconstruction of the defect with a protrusio cage, retrograde screws, and a cemented total hip arthroplasty. PATIENTS AND METHODS: We retrospectively identified 70 consecutive patients who were surgically treated for metastatic disease of the acetabulum between 1995 and 2012 using the above technique. The type of primary tumor, extent of the disease, degree of acetabular erosion, and type of implant used were identified. Patient and implant survival, complications, and functional outcome were recorded. RESULTS: There were no mortalities in the perioperative period (30 days after surgery). Median overall patient survival was 12 months. Prosthesis survival was 92% at 1 year and 89% at 5 years. One third of the patients suffered a complication, the most frequent one being dislocation. The functional outcome was good. Multiple skeletal or visceral metastases and specific types of cancer were associated with poor patient survival. INTERPRETATION: Reconstruction of metastatic acetabular defects using a protrusio cage stabilized with retrograde screws and a cemented total hip arthroplasty is a safe procedure that provides efficient relief of symptoms. Patients with extensive disease, especially when diagnosed with specific types of cancer, have a very poor prognosis. The complication rate is substantial, the most frequent being dislocation. However, revision surgery is seldom required and prosthesis survival is high.


Assuntos
Acetábulo/cirurgia , Neoplasias Ósseas/cirurgia , Acetabuloplastia/métodos , Adulto , Idoso , Idoso de 80 Anos ou mais , Artroplastia de Quadril/métodos , Parafusos Ósseos , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Procedimentos de Cirurgia Plástica/métodos , Estudos Retrospectivos , Análise de Sobrevida , Resultado do Tratamento
13.
J Surg Oncol ; 111(8): 951-6, 2015 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-26040651

RESUMO

BACKGROUND AND OBJECTIVES: Our study aimed to describe the clinical outcome of patients with superficial soft-tissue sarcomas (SSTS), define prognostic factors and provide evidence for a rational surveillance scheme. METHODS: Data for 622 consecutive, surgically treated SSTS patients were retrieved from the Scandinavian Sarcoma Group Register. We assessed the rates of local recurrence (LR) and metastasis (M), as well as overall survival (OS), local recurrence free-survival (LRFS) and metastasis-free survival (MFS) of the cohort. RESULTS: The incidence of LR and M was 9% and 12%, respectively. OS at 5 years was 79%, LRFS was 74% and MFS 76%. Factors that affected OS, LRFS, and MFS were tumor size and patient age. Additionally, tumor grade was an independent prognostic factor for LRFS. The majority of LR and M events were observed the first 2 years of follow-up. Clear surgical margins were correlated to lower risk for LR. Selected patients benefited from adjuvant radiotherapy. CONCLUSIONS: SSTS have a favourable prognosis, which is mainly determined by tumour-associated factors. Adequate surgical margins are important for local control, whereas radiotherapy has a secondary role. The data support current surveillance schemes, with a closer follow-up the first 2 years after surgery.


Assuntos
Sarcoma/epidemiologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Metástase Neoplásica , Recidiva Local de Neoplasia/epidemiologia , Vigilância da População , Prognóstico , Sistema de Registros , Sarcoma/patologia , Sarcoma/cirurgia , Países Escandinavos e Nórdicos/epidemiologia , Análise de Sobrevida , Adulto Jovem
14.
Orthopedics ; 38(5): e401-6, 2015 May.
Artigo em Inglês | MEDLINE | ID: mdl-25970367

RESUMO

Limb-preserving surgery using modular megaprostheses for the reconstruction of large skeletal defects is currently the preferred treatment for sarcomas. The authors report the postoperative outcomes after skeletal resection for lower extremity sarcomas and the use of the METS cemented modular implant system (Stanmore Implants, Hertfordshire, United Kingdom) for reconstruction. They retrospectively studied 52 consecutive patients operated on from 2003 to 2012. There were 27 distal femur prostheses, 13 proximal femur, 11 proximal tibia, and 1 total femur implants. Patients were followed for a mean of 4.3 years. Overall patient survival, prosthesis survival, limb salvage rate, and secondary complications were documented. Five years postoperatively, prosthesis survival was 79%. Complications warranting implant revision surgery were documented in 15% of patients, whereas complications warranting surgery of any kind were observed in 27% of the patients. Nonmechanical complications, namely local relapse of the tumor and prosthetic infection, were the most common cause of prosthetic failure, accounting for 88% of major revision surgeries and 100% of amputations. Mechanical complications were rare, observed in only 6% of patients. No patients required secondary revision surgery. The limb salvage rate was 89%. Overall patient survival was 79% at 5 years and 71% at 10 years. The low risk for mechanical complications and the high limb salvage rate support the use of the METS modular megaprostheses for the reconstruction of skeletal defects following lower limb sarcoma surgery.


Assuntos
Membros Artificiais/classificação , Neoplasias Ósseas/cirurgia , Salvamento de Membro/métodos , Extremidade Inferior/cirurgia , Procedimentos Ortopédicos/métodos , Procedimentos de Cirurgia Plástica/métodos , Sarcoma/cirurgia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Amputação Cirúrgica , Neoplasias Ósseas/mortalidade , Criança , Feminino , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Desenho de Prótese , Falha de Prótese , Estudos Retrospectivos , Sarcoma/mortalidade , Taxa de Sobrevida , Resultado do Tratamento , Reino Unido , Adulto Jovem
15.
Breast ; 23(3): 286-90, 2014 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-24684891

RESUMO

The aim was to identify prognostic variables associated with survival in 301 breast cancer patients after surgical treatment of skeletal metastases. The study period was 1986-2012. The median age at surgery was 61 (interquartile-range [IQR] 52-70) years. The cumulative 1-, 2-, and 5-year survival after surgery was 45% (95% CI 39-51), 27% (22-32), and 8% (5-12), respectively. The median follow-up time was 1 (IQR 0.2-2) year. Age over 60 years (Hazard ratio [HR] 1.9) and hemoglobin levels <110 g/L (HR 2) increased the risk of death after surgery. Patients with impending fractures (HR 0.4) had a lower death rate. The overall neurological function in patients with spinal metastases improved after surgery (p < 0.001). The complication rate was 25%, including 14% re-operations. Survival data and analysis of complications of this large cohort of surgically treated breast cancer patients help to set appropriate expectations for the patients, families, and medical staff.


Assuntos
Neoplasias Ósseas , Hemoglobinas/análise , Expectativa de Vida , Procedimentos Ortopédicos , Complicações Pós-Operatórias , Fatores Etários , Neoplasias Ósseas/complicações , Neoplasias Ósseas/mortalidade , Neoplasias Ósseas/secundário , Neoplasias Ósseas/cirurgia , Neoplasias da Mama/sangue , Neoplasias da Mama/mortalidade , Neoplasias da Mama/patologia , Feminino , Fraturas Espontâneas/epidemiologia , Fraturas Espontâneas/etiologia , Humanos , Pessoa de Meia-Idade , Procedimentos Ortopédicos/efeitos adversos , Procedimentos Ortopédicos/métodos , Procedimentos Ortopédicos/estatística & dados numéricos , Complicações Pós-Operatórias/classificação , Complicações Pós-Operatórias/epidemiologia , Prognóstico , Sistema de Registros , Reoperação , Medição de Risco , Fatores de Risco , Taxa de Sobrevida , Suécia/epidemiologia
16.
Eur J Orthop Surg Traumatol ; 24(4): 553-7, 2014 May.
Artigo em Inglês | MEDLINE | ID: mdl-23689913

RESUMO

OBJECTIVES: To present the experience of a tertiary referral hospital in the management of a case series with hip or knee fractures by using modular megaprosthesis. PATIENTS AND METHODS: Seventeen consecutive patients with highly comminuted fractures of the knee (n = 2), periprosthetic fractures of knee (n = 10) or hip (n = 5) were included. Fractures were managed with modular megaprosthesis (including total hip in 2 cases). Postoperative complications like infection and instability and outcome measures like return to previous mobility and living were recorded. RESULTS: The mean age at time of surgery was 77 years (25-91), and mean follow-up was 44 months (13-98). We had no intra-operative complications. There were 3 deep periprosthetic infections, 1 hip and 2 knee. In the hip group, including total femur patients, we had 2 dislocations (2/7), both managed with closed reduction. No aseptic loosening was seen. 15/17 patients regained walking ability, and 16 were discharged to independent living. Nine patients have died at the time of follow-up. CONCLUSIONS: In these often old and physically compromised patients with highly comminuted fractures or complicated periprosthetic fractures, modular megaprosthesis could be a good surgical option. It can provide immediate stability and allow early mobilization.


Assuntos
Artroplastia de Quadril/métodos , Artroplastia do Joelho/métodos , Fraturas do Quadril/cirurgia , Prótese de Quadril , Traumatismos do Joelho/cirurgia , Prótese do Joelho , Adulto , Idoso , Idoso de 80 Anos ou mais , Artroplastia de Quadril/efeitos adversos , Artroplastia do Joelho/efeitos adversos , Feminino , Seguimentos , Fraturas Cominutivas/diagnóstico por imagem , Fraturas Cominutivas/cirurgia , Fraturas do Quadril/diagnóstico por imagem , Humanos , Traumatismos do Joelho/diagnóstico por imagem , Masculino , Pessoa de Meia-Idade , Falha de Prótese , Implantação de Prótese/métodos , Radiografia , Recuperação de Função Fisiológica , Estudos Retrospectivos , Resultado do Tratamento
17.
Acta Orthop ; 84(5): 502-5, 2013 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-24171682

RESUMO

BACKGROUND AND PURPOSE: Recent data suggest that percutaneous sclerotherapy is a safe alternative to surgery for treatment of aneurysmal bone cysts (ABCs). We present our experience of this method. METHODS: We retrospectively analyzed data from 38 consecutive patients treated with repeated injections of polidocanol. Each injection consisted of 2-4 mg polidocanol per kg body weight. Radiological and clinical assessments were performed until healing. RESULTS: All cycts except 1 healed after a median of 4 (1-11) injections. A lesion failed to heal in 1 patient, who was operated. 3 patients experienced minor local inflammatory reactions. INTERPRETATION: Our results show that percutaneus sclerotherapy with polidocanol has high efficacy in the treatment of ABCs, with a low frequency of side effects. Our findings corroborate data presented in previous publications. We believe that the method will be especially valuable in ABCs of the pelvis and sacrum, where surgery is associated with considerable morbidity.


Assuntos
Cistos Ósseos Aneurismáticos/terapia , Polietilenoglicóis/administração & dosagem , Soluções Esclerosantes/administração & dosagem , Adolescente , Adulto , Cistos Ósseos Aneurismáticos/diagnóstico por imagem , Criança , Pré-Escolar , Feminino , Humanos , Injeções Intralesionais , Masculino , Dor Musculoesquelética/etiologia , Polidocanol , Radiografia , Estudos Retrospectivos , Resultado do Tratamento , Adulto Jovem
18.
Cancer Genet ; 206(7-8): 299-303, 2013.
Artigo em Inglês | MEDLINE | ID: mdl-23938179

RESUMO

Soft tissue tumors in children under one year of age (infants) are rare. The etiology is usually unknown, with external factors or congenital birth defects and hereditary syndromes being recognized in only a small proportion of the cases. We ascertained the cytogenetic findings in 16 infants from whom tumor tissue had been obtained during a 25-year period. In eight of them, single nucleotide polymorphism (SNP) array analyses could also be performed. No constitutional chromosome aberrations were detected, and assessment of clinical files did not reveal any congenital or later anatomical defects. Three tumors--one infantile fibrosarcoma, one embryonal rhabdomyosarcoma, and one angiomatoid fibrous histiocytoma (AFH)--had abnormal karyotypes. As the AFH had an exchange between chromosome arms 12p and 15q, additional fluorescence in situ hybridization and reverse transcription-polymerase chain reaction analyses were performed, unexpectedly revealing an ETV6/NTRK3 fusion. Three of the eight tumors, including the AFH with an abnormal karyotype, analyzed by SNP array showed aberrations (loss of heterozygosity or imbalances). The present series suggests that the addition of array-based technologies is valuable for detecting underlying pathogenetic mechanisms.


Assuntos
Neoplasias de Tecidos Moles/genética , Idade de Início , Análise Citogenética , Feminino , Fibrossarcoma/epidemiologia , Fibrossarcoma/genética , Fibrossarcoma/patologia , Humanos , Lactente , Recém-Nascido , Masculino , Análise em Microsséries/métodos , Proteínas de Fusão Oncogênica/genética , Polimorfismo de Nucleotídeo Único , Proteínas Proto-Oncogênicas c-ets/genética , Receptor trkC/genética , Proteínas Repressoras/genética , Estudos Retrospectivos , Rabdomiossarcoma Embrionário/epidemiologia , Rabdomiossarcoma Embrionário/genética , Rabdomiossarcoma Embrionário/patologia , Neoplasias de Tecidos Moles/diagnóstico , Neoplasias de Tecidos Moles/epidemiologia , Variante 6 da Proteína do Fator de Translocação ETS
19.
Dent Mater J ; 32(4): 592-9, 2013.
Artigo em Inglês | MEDLINE | ID: mdl-23903641

RESUMO

This study investigated the effects of aging and irradiation time on the macro- and micro-mechanical properties of a highly translucent nanohybrid composite (IPS Empress Direct, Trans Opal shade, Ivoclar Vivadent). Flexural strength, flexural modulus, indentation modulus, Vickers hardness, and creep were measured after being irradiated with different durations (5, 10, 20, and 40 s) and aged under different conditions (24 h at 37°C in water; 5,000 times of thermocycling between 5°C and 55°C followed by 4-week storage in artificial saliva or alcohol). Rate of cure was also measured for these four irradiation times at composite specimen surface and at 2 mm depth. Effects of aging and irradiation time were statistically analyzed using one-way ANOVA with Tukey's HSD post hoc test (α=0.05), partial eta-squared statistic, and Weibull analysis. Alcohol aging significantly reduced the mechanical properties. Aging in saliva produced a positive effect on micro-mechanical properties. Irradiation time should be at least 20 s to yield favorable mechanical properties.


Assuntos
Resinas Compostas/química , Materiais Dentários/química , Resinas Compostas/efeitos da radiação , Materiais Dentários/efeitos da radiação , Análise do Estresse Dentário/instrumentação , Módulo de Elasticidade , Etanol/química , Dureza , Humanos , Luz , Teste de Materiais , Metacrilatos/química , Maleabilidade , Polimerização , Saliva Artificial/química , Dióxido de Silício/química , Espectroscopia de Infravermelho com Transformada de Fourier , Estresse Mecânico , Propriedades de Superfície , Temperatura , Fatores de Tempo , Água/química
20.
Int J Radiat Oncol Biol Phys ; 86(5): 949-55, 2013 Aug 01.
Artigo em Inglês | MEDLINE | ID: mdl-23725998

RESUMO

PURPOSE: To study the impact of dose fractionation of adjuvant radiation therapy (RT) on local recurrence (LR) and the relation of LR to radiation fields. METHODS AND MATERIALS: LR rates were analyzed in 462 adult patients with soft tissue sarcoma who underwent surgical excision and adjuvant RT at five Scandinavian sarcoma centers from 1998 to 2009. Medical records were reviewed for dose fractionation parameters and to determine the location of the LR relative to the radiation portals. RESULTS: Fifty-five of 462 patients developed a LR (11.9%). Negative prognostic factors included intralesional surgical margin (hazard ratio [HR]: 7.83, 95% confidence interval [CI]: 3.08-20.0), high malignancy grade (HR: 5.82, 95% CI: 1.31-25.8), age at diagnosis (HR per 10 years: 1.27, 95% CI: 1.03-1.56), and malignant peripheral nerve sheath tumor histological subtype (HR: 6.66, 95% CI: 2.56-17.3). RT dose was tailored to margin status. No correlation between RT dose and LR rate was found in multiple Cox regression analysis. The majority (65%) of LRs occurred within the primary RT volume. CONCLUSIONS: No significant dose-response effect of adjuvant RT was demonstrated. Interestingly, patients given 45-Gy accelerated RT (1.8 Gy twice daily/2.5 weeks) had the best local outcome. A total dose of 50 Gy in 25 fractions seemed adequate following wide margin surgery. The risk of LR was associated with histopathologic subtype, which should be included in the treatment algorithm of adjuvant RT in soft tissue sarcoma.


Assuntos
Extremidades , Recidiva Local de Neoplasia , Sarcoma/radioterapia , Tronco , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Análise de Variância , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Fracionamento da Dose de Radiação , Doxorrubicina/administração & dosagem , Feminino , Seguimentos , Humanos , Ifosfamida/administração & dosagem , Masculino , Pessoa de Meia-Idade , Recidiva Local de Neoplasia/patologia , Radioterapia Adjuvante , Sarcoma/tratamento farmacológico , Sarcoma/patologia , Sarcoma/cirurgia , Análise de Sobrevida , Adulto Jovem
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