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1.
Commun Biol ; 7(1): 606, 2024 May 20.
Artigo em Inglês | MEDLINE | ID: mdl-38769442

RESUMO

Well-differentiated liposarcoma (WDLS) displays amplification of genes on chromosome 12 (Chr12) in supernumerary ring or giant marker chromosomes. These structures have been suggested to develop through chromothripsis, followed by circularization and breakage-fusion-bridge (BFB) cycles. To test this hypothesis, we compared WDLSs with Chr12 amplification in rod-shaped chromosomes with WDLSs with rings. Both types of amplicons share the same spectrum of structural variants (SVs), show higher SV frequencies in Chr12 than in co-amplified segments, have SVs that fuse the telomeric ends of co-amplified chromosomes, and lack interspersed deletions. Combined with the finding of cells with transient rod-shaped structures in tumors with ring chromosomes, this suggests a stepwise process starting with the gain of Chr12 material that, after remodeling which does not fit with classical chromothripsis, forms a dicentric structure with other chromosomes. Depending on if and when telomeres from other chromosomes are captured, circularized or linear gain of 12q sequences will predominate.


Assuntos
Amplificação de Genes , Lipossarcoma , Proteínas Proto-Oncogênicas c-mdm2 , Humanos , Lipossarcoma/genética , Lipossarcoma/patologia , Proteínas Proto-Oncogênicas c-mdm2/genética , Cromossomos Humanos Par 12/genética , Cromotripsia , Cromossomos em Anel
2.
Eur J Surg Oncol ; 50(6): 108348, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38677000

RESUMO

BACKGROUND: Immediate vascularized reconstruction after sarcoma resection may reduce wound complications common in primary closure, but previous research is conflicting. The present study analysed wound complication rates and compared wound-related outcomes among immediate vascularized reconstruction with primary closure. MATERIALS AND METHODS: Patient- and tumour characteristics were collected from patients who received primary surgery with curative intent between 2010 and 2020 at the Stockholm Sarcoma Centre. Clinical outcomes were sought in free text in medical records. The primary outcome measures were early (<30 days) wound complications. Secondary outcome measures included late (30-day to 2-year) wound complications, time to wound healing, 30-day postoperative complications according to Clavien Dindo, and health care consumption. RESULTS: A total of 482 patients were included, of whom 69 had immediate vascularized reconstruction. Comparison of early complications for reconstructive surgery with primary closure revealed a significantly higher complication rate for the first group (59.4 % vs 29.8 %, p < 0.01). The groups had comparable health care consumption the first and second postoperative years. In-depth analyses identified BMI >25, smoking and high-grade lesions as factors with adverse effects on wound healing. CONCLUSIONS: Sarcoma patients experience high rates of wound complications and consume considerable health care resources. Reconstructed individuals were more susceptible to such complications than were subjects with primary closure.


Assuntos
Procedimentos de Cirurgia Plástica , Complicações Pós-Operatórias , Sarcoma , Cicatrização , Humanos , Sarcoma/cirurgia , Feminino , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Procedimentos de Cirurgia Plástica/métodos , Idoso , Complicações Pós-Operatórias/epidemiologia , Adulto , Retalhos Cirúrgicos , Neoplasias de Tecidos Moles/cirurgia , Infecção da Ferida Cirúrgica/epidemiologia
3.
Br J Cancer ; 127(10): 1793-1798, 2022 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-36030294

RESUMO

BACKGROUND: Current risk models in solitary fibrous tumour (SFT) were developed using cohorts with short follow-up and cannot reliably identify low-risk patients. We recently developed a novel risk model (G-score) to account for both early and late recurrences. Here, we aimed to validate the G-score in a large international cohort with long-term follow-up. METHODS: Data were collected from nine sarcoma referral centres worldwide. Recurrence-free interval (RFi) was the primary endpoint. RESULTS: The cohort comprised 318 patients with localised extrameningeal SFTs. Disease recurrence occurred in 96 patients (33%). The estimated 5-year RFi rate was 72%, and the 10-year RFi rate was 52%. G-score precisely predicted recurrence risk with estimated 10-year RFi rate of 84% in low risk, 54% in intermediate risk and 36% in high risk (p < 0.001; C-index 0.691). The mDemicco (p < 0.001; C-index 0.749) and SalasOS (p < 0.001; C-index 0.674) models also predicted RFi but identified low-risk patients less accurate with 10-year RFi rates of 72% and 70%, respectively. CONCLUSIONS: G-score is a highly significant predictor of early and late recurrence in SFT and is superior to other models to predict patients at low risk of relapse. A less intensive follow-up schedule could be considered for patients at low recurrence risk according to G-score.


Assuntos
Recidiva Local de Neoplasia , Tumores Fibrosos Solitários , Humanos , Prognóstico , Recidiva Local de Neoplasia/patologia , Tumores Fibrosos Solitários/cirurgia , Tumores Fibrosos Solitários/patologia , Fatores de Risco , Estudos de Coortes , Doença Crônica
4.
Mod Pathol ; 34(11): 2020-2027, 2021 11.
Artigo em Inglês | MEDLINE | ID: mdl-34108637

RESUMO

Chondrosarcomas are the second most common malignant bone tumor. Activating promoter mutations in telomerase reverse transcriptase (TERT) was recently described by us and others as a frequent mutation in high-grade chondrosarcoma. In this study, we investigate the prognostic significance of TERT promoter mutations in 241 chondrosarcomas from 190 patients collected over 24 years (1994-2017). The TERT promoter was sequenced after microdissection of 135 chondrosarcomas from 106 patients in addition to data from our previous cohort. The TERT promoter mutation at -124 C > T was found in 45% of all patients and was significantly associated (p > 0,001) with higher tumor grade, shorter metastasis-free survival, and disease-specific survival. Additionally, TERT promoter-mutated tumors were associated with a more aggressive metastatic pattern. Shorter survival was observed in patients with wild-type primary tumors who developed a mutated metastasis indicative of tumor progression. Primary tumor genetic heterogeneity and altering mutational status between nonsynchronous metastatic lesions suggests that chondrosarcoma is a multiclonal disease progressing through a branching evolution. Conclusion: TERT promoter mutation seems to be a central event in chondrosarcoma progression with association to metastatic disease and disease-related mortality. As an easily analyzed marker, there is future potential to utilize TERT promoter mutation status as a prognostic marker and investigate telomerase-targeted therapy in chondrosarcomas.


Assuntos
Biomarcadores Tumorais/genética , Neoplasias Ósseas/diagnóstico , Condrossarcoma/diagnóstico , Mutação/genética , Regiões Promotoras Genéticas , Telomerase/genética , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Neoplasias Ósseas/genética , Criança , Condrossarcoma/genética , Progressão da Doença , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico
5.
Cancers (Basel) ; 13(6)2021 Mar 10.
Artigo em Inglês | MEDLINE | ID: mdl-33801953

RESUMO

Ewing sarcoma is the second most common bone sarcoma in children after osteosarcoma. It is a very aggressive malignancy for which systemic treatment has greatly improved outcome for patients with localized disease, who now see survival rates of over 70%. However, for the quarter of patients presenting with metastatic disease, survival is still dismal with less than 30% of patients surviving past 5 years. Patients with disease relapse, local or distant, face an even poorer prognosis with an event-free 5-year survival rate of only 10%. Unfortunately, Ewing sarcoma patients have not yet seen the benefit of recent years' technical achievements such as next-generation sequencing, which have enabled researchers to study biological systems at a level never seen before. In spite of large multinational studies, treatment of Ewing sarcoma relies entirely on chemotherapeutic agents that have been largely unchanged for decades. As many promising modern therapies, including monoclonal antibodies, small molecules, and immunotherapy, have been disappointing to date, there is no clear candidate as to which drug should be investigated in the next large-scale clinical trial. However, the mechanisms driving tumor development in Ewing sarcoma are slowly unfolding. New entities of Ewing-like tumors, with fusion transcripts that are related to the oncogenic EWSR1-FLI1 fusion seen in the majority of Ewing tumors, are being mapped. These tumors, although sharing much of the same morphologic features as classic Ewing sarcoma, behave differently and may require a different treatment. There are also controversies regarding local treatment of Ewing sarcoma. The radiosensitive nature of the disease and the tendency for Ewing sarcoma to arise in the axial skeleton make local treatment very challenging. Surgical treatment and radiotherapy have their pros and cons, which may give rise to different treatment strategies in different centers around the world. This review article discusses some of these controversies and reproduces the highlights from recent publications with regard to diagnostics, systemic treatment, and surgical treatment of Ewing sarcoma.

6.
Mol Oncol ; 14(5): 1101-1117, 2020 05.
Artigo em Inglês | MEDLINE | ID: mdl-32115849

RESUMO

Ewing sarcomas (ESs) are aggressive sarcomas driven by EWS fusion genes. We sought to investigate whether whole-transcriptome sequencing (RNA-seq) could be used to detect patterns associated with chemotherapy response or tumor progression after first-line treatment. Transcriptome sequencing (RNA-seq) of 13 ES cases was performed. Among the differentially expressed pathways, we identified IGF2 expression as a potential driver of chemotherapy response and progression. We investigated the effect of IGF2 on proliferation, radioresistance, apoptosis, and the transcriptome pattern in four ES cell lines and the effect of IGF2 expression in a validation series of 14 patients. Transcriptome analysis identified differentially expressed genes (adj. P < 0.005) and pathways associated with chemotherapy response (285 genes), short overall survival (662 genes), and progression after treatment (447 genes). Imprinting independent promoter P3-mediated IGF2 expression was identified in a subset of cases with aggressive clinical course. In ES cell lines, IGF2 induced proliferation, but promoted radioresistance only in CADO cells. High IGF2 expression was also significantly associated with shorter overall survival in patients with ES. Transcriptome analysis of the clinical samples and the cell lines revealed an IGF-dependent signature, potentially related to a stem cell-like phenotype. Transcriptome analysis is a potentially powerful complementary tool to predict the clinical behavior of ES and may be utilized for clinical trial stratification strategies and personalized oncology. Certain gene signatures, for example, IGF-related pathways, are coupled to biological functions that could be of clinical importance. Finally, our results indicate that IGF inhibition may be successful as a first-line therapy in conjunction with conventional radiochemotherapy for a subset of patients.


Assuntos
Neoplasias Ósseas/metabolismo , Resistencia a Medicamentos Antineoplásicos/genética , Regulação Neoplásica da Expressão Gênica/genética , Fator de Crescimento Insulin-Like II/metabolismo , Sarcoma de Ewing/metabolismo , Transdução de Sinais/genética , Adolescente , Apoptose/efeitos dos fármacos , Apoptose/genética , Apoptose/efeitos da radiação , Neoplasias Ósseas/tratamento farmacológico , Neoplasias Ósseas/genética , Neoplasias Ósseas/patologia , Proteínas de Ciclo Celular/genética , Proteínas de Ciclo Celular/metabolismo , Linhagem Celular Tumoral , Proliferação de Células/efeitos dos fármacos , Proliferação de Células/genética , Proliferação de Células/efeitos da radiação , Estudos de Coortes , Inibidor p16 de Quinase Dependente de Ciclina/genética , Inibidor p16 de Quinase Dependente de Ciclina/metabolismo , Progressão da Doença , Resistencia a Medicamentos Antineoplásicos/efeitos da radiação , MAP Quinases Reguladas por Sinal Extracelular/metabolismo , Feminino , Regulação Neoplásica da Expressão Gênica/efeitos dos fármacos , Glicólise/genética , Glicólise/fisiologia , Proteínas de Choque Térmico/genética , Proteínas de Choque Térmico/metabolismo , Humanos , Imuno-Histoquímica , Fator de Crescimento Insulin-Like II/genética , Masculino , Proteínas Proto-Oncogênicas c-akt/metabolismo , RNA-Seq , Proteínas Ribossômicas/genética , Proteínas Ribossômicas/metabolismo , Sarcoma de Ewing/tratamento farmacológico , Sarcoma de Ewing/genética , Sarcoma de Ewing/patologia , Transdução de Sinais/efeitos dos fármacos , Adulto Jovem
7.
Br J Cancer ; 122(8): 1242-1249, 2020 04.
Artigo em Inglês | MEDLINE | ID: mdl-32066914

RESUMO

BACKGROUND: The long-term risks and time trends of subsequent primary neoplasms (SPNs) among Ewing (ES) and osteosarcoma (OS) survivors are not fully understood. METHODS: We performed a nationwide study of all ES and OS patients identified in the Swedish Cancer Registry from 1958 to 2015 with up to 58 years of follow-up. The risk of SPN was compared with that of the general population using standardised incidence ratios (SIRs) and absolute excess risks (AERs). RESULTS: One hundred and fifteen SPNs were diagnosed among 1779 patients with ES or OS, yielding an overall SIR of 2.3 (95% confidence interval (CI), 1.9-2.7). The risk remained significantly increased in the latest treatment era (SIR2000-2015 2.0; 95% CI, 1.1-3.5). The highest absolute excess risks (AER) was due to breast cancer (AER 15.2/10,000 person-years; 95% CI, 5.0-29.8) followed by female genital malignancies (AER 9.5/10,000 person-years; 95% CI, 2.4-21.5). The excess breast cancer risk among ES survivors was noted also after 30 years of follow-up with 127 extra breast cancers/10,000 person-years (95% CI, 6.6-419). CONCLUSIONS: Breast- and female genital malignancies contribute most to the excess risk of SPN among ES and OS survivors. Importantly, excess risks did not decline over calendar time or long-term follow-up.


Assuntos
Neoplasias Ósseas/complicações , Sobreviventes de Câncer , Neoplasias/etiologia , Osteossarcoma/complicações , Sarcoma de Ewing/complicações , Adolescente , Adulto , Idoso , Neoplasias Ósseas/mortalidade , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Lactente , Recém-Nascido , Masculino , Pessoa de Meia-Idade , Osteossarcoma/mortalidade , Risco , Sarcoma de Ewing/mortalidade , Adulto Jovem
8.
Cancer Immunol Immunother ; 68(6): 927-936, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30879106

RESUMO

BACKGROUND: Immune cells can regulate disease progression and response to treatment in multiple tumor types, but their activities in human soft tissue sarcoma are poorly characterized. METHODS: Marker-defined immune cell subsets were characterized from a tumor microenvironmental perspective in two independent cohorts of human soft tissue sarcoma by multiplex IHC, quantitative PCR and/or bioinformatics. RESULTS: B cell profiling revealed a prognostic role for CD20 protein (cohort 1, 33 patients) and MS4A1 gene expression (cohort 2, 265 patients). Multiplex IHC and gene correlation analysis supported a role in antigen presentation, immune cell differentiation and T cell activation. The prognostic role of MS4A1 expressing B cells was only observed in an IL10low, PTGS2low or CD163low tumor microenvironment according to the transcriptomic data. IL10 levels consistently correlated with the M2-like macrophage marker CD163, which also defined the majority of macrophages. A polarization of these cells toward a pro-tumoral phenotype was further supported by lack of correlation between CD163 and M1 markers like NOS2, as well as by low abundance of CD80 positive cells in tissue. CONCLUSIONS: Analysis of CD20/MS4A1 expression in soft tissue sarcoma merits further attention as a promising candidate prognostic tool for survival, but not in patients with a pronounced immunosuppressive tumor microenvironment. Macrophages are ubiquitous and polarized toward a protumoral phenotype. This provides a rationale for further studies on B cell function and immunotherapy targeting M2-polarized macrophages.


Assuntos
Antígenos CD20/imunologia , Linfócitos B/imunologia , Macrófagos/imunologia , Sarcoma/imunologia , Neoplasias de Tecidos Moles/imunologia , Transcriptoma/imunologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Antígenos CD20/genética , Antígenos CD20/metabolismo , Linfócitos B/metabolismo , Biomarcadores Tumorais/genética , Biomarcadores Tumorais/imunologia , Biomarcadores Tumorais/metabolismo , Estudos de Coortes , Feminino , Humanos , Estimativa de Kaplan-Meier , Macrófagos/metabolismo , Masculino , Pessoa de Meia-Idade , Prognóstico , Sarcoma/genética , Sarcoma/metabolismo , Neoplasias de Tecidos Moles/genética , Neoplasias de Tecidos Moles/metabolismo , Microambiente Tumoral/genética , Microambiente Tumoral/imunologia , Adulto Jovem
9.
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
10.
J Bone Oncol ; 14: 100216, 2019 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-30666289

RESUMO

Object: Many patients with Ewing sarcoma (ES) of the mobile spine present with neurologic symptoms leading to emergency decompressive surgery. Only rarely is optimal treatment involving neo-adjuvant chemotherapy followed by en bloc excision possible. The purpose of this study was to study treatment, neurologic and oncologic outcome in patients with ES of the mobile spine. Methods: Twenty-four patients diagnosed between 1986 and 2012 were identified through the Scandinavian Sarcoma Group registry. Charts were reviewed in order to assess details in patient characteristics, neurologic status, treatment and outcome. Prognostic factors were analyzed with respect to local control, disease-free survival and overall survival. Results: Neurologic symptoms were frequently observed at presentation, being present in 19/23 patients with documented neurologic status. Most (13/19) patients had a complete neurologic recovery regardless of whether or not emergency decompressive surgery was performed. The majority (18/24) of patients were treated with definitive radiotherapy. However, only 9/17 received the recommended dose of  ≥  50.4 Gy. The disease-free and overall survival rates were 48% and 57% at 5 and 10 years, respectively. The local recurrence rates were 19% and 27% at 5 and 10 years, respectively. Only year of diagnosis, categorized into periods with significant changes in chemotherapy protocols, was a significant factor for local recurrence, but there was a trend (p = 0.06) for an increased risk of a local recurrence if emergency decompressive surgery was performed. Conclusion: Patients with ES of the mobile have a relatively favorable prognosis. Nonetheless, local recurrence rate is high for this group of patients for which local treatment mainly relies on definitive radiotherapy. Emergency decompressive surgery may increase the risk for local recurrence.

11.
Mod Pathol ; 31(12): 1834-1841, 2018 12.
Artigo em Inglês | MEDLINE | ID: mdl-30065261

RESUMO

Chondrosarcomas are malignant skeletal tumors with chondroid differentiation. Prognosis is largely dependent on histological grading, which suffer from significant interobserver variability. Telomerase activity and abundant telomerase reverse transcriptase (hTERT) expression has previously been associated with chondrosarcoma grade and metastasis. We therefore analyzed the hTERT promoter in clinicopathologically well-characterized chondrosarcomas (grade 1-3) from 87 patients. Using Sanger sequencing we identified an activating -124 C > T mutation in 23 cases (26%). Promoter mutations were significantly associated with increased histological grade (8% of grade 1, 32% of grade 2 and 46% of grade 3, P = 0.002), suggesting a role in tumor progression. In four chondrosarcomas where the histopathological grade was heterogenous, the hTERT mutation was only identified in the higher-grade areas. Additionally, hTERT promoter mutations were significantly associated with worse metastasis-free survival (P = 0.018), chondrosarcoma-specific survival (P = 0.022) and older patient age (P = 0.003). These data suggest that hTERT promoter mutations are common in high grade conventional chondrosarcomas. Granted that additional studies can confirm these findings; hTERT promoter analysis could potentially serve as an adjuvant prognostic marker in routine chondrosarcoma grading. This study reinforces the rationale of telomerase targeted therapy in a subset of chondrosarcomas.


Assuntos
Neoplasias Ósseas/genética , Neoplasias Ósseas/mortalidade , Condrossarcoma/genética , Condrossarcoma/mortalidade , Telomerase/genética , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Biomarcadores Tumorais/genética , Progressão da Doença , Feminino , Humanos , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Mutação , Prognóstico , Regiões Promotoras Genéticas/genética , Adulto Jovem
12.
J Clin Pathol ; 71(9): 832-839, 2018 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-29703757

RESUMO

AIMS: Solitary fibrous tumour (SFT) is an infrequently metastasising mesenchymal tumour defined by the NAB2-STAT6 fusion gene. Activating mutations in the telomerase reverse transcriptase (hTERT) gene promoter has been reported to associate with adverse patient outcome in SFTs. METHODS: We analysed the hTERT gene for promoter mutations and copy number alterations in 43 primary extrameningeal SFTs (9 malignant and 34 benign tumours according to WHO 2013 criteria), six local recurrences and three metastatic lesions. RESULTS: Activating -124 C>T (n=12) or -148 C>T (n=2) mutations were found in 33% of the tumours and associated with older age (P=0.006), necrosis (P=0.009), higher mitotic rate (P=0.003), nuclear atypia (P=0.002), malignant histological diagnosis (P=0.04) and worse progression-free survival (P=0.023). We also observed frequent (24%) hTERT promoter mutations in histologically benign tumours without metastasis (mean follow-up >9 years), and in 14%-18% of low-risk SFTs as determined by three risk-stratification models. Mutations were seen in 2/6 metastatic tumours and metastatic lesions. hTERT copy number gain was seen in 11/28 hTERT promoter wild-type cases. CONCLUSIONS: Activating hTERT promoter mutations associate with aggressive histopathological features, indicating a role in tumour progression. Given the comparatively high prevalence of hTERT promoter mutations in low-risk and non-metastasising lesions, further studies are required to clarify the prognostic value of hTERT promoter analysis before implementing the analysis in clinical diagnostics.


Assuntos
Variações do Número de Cópias de DNA , Dosagem de Genes , Mutação , Regiões Promotoras Genéticas , Tumores Fibrosos Solitários/genética , Telomerase/genética , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Biópsia , Análise Mutacional de DNA , Progressão da Doença , Intervalo Livre de Doença , Feminino , Predisposição Genética para Doença , Humanos , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Recidiva Local de Neoplasia , Fenótipo , Fatores de Risco , Tumores Fibrosos Solitários/enzimologia , Tumores Fibrosos Solitários/secundário , Tumores Fibrosos Solitários/cirurgia , Suécia , Fatores de Tempo , Resultado do Tratamento , Adulto Jovem
13.
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
14.
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
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