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1.
Anticancer Res ; 39(10): 5747-5753, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31570477

RESUMO

BACKGROUND/AIM: Liposarcomas (LS) are one of the most common entities within the heterogenous group of soft tissue sarcomas. The aim of this study was to identify prognostic indicators in patients with LS of the extremities and truncal wall. PATIENTS AND METHODS: We analysed the influence of potential prognostic factors on local recurrence-free survival (LRFS) and overall survival (OS) in 181 patients who were suitable for surgical treatment with curative intent. RESULTS: The median follow-up period was 7.1 years. The 5-year LRFS and OS rates were 79.1 and 93.3%. The 5-year OS rate was 94.7% in patients with R0-resected primary tumors and 72.7% in patients with R1/R2-status (p=0.023). In multivariate analysis, only histologic grade was found to be an independent prognostic factor of OS. CONCLUSION: Negative margins were not an independent prognostic factor in our series. Tumor biology reflected by histologic grade dictated the outcome.


Assuntos
Extremidades/patologia , Lipossarcoma/patologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Intervalo Livre de Doença , Feminino , Seguimentos , Humanos , Lipossarcoma/mortalidade , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Recidiva Local de Neoplasia/mortalidade , Recidiva Local de Neoplasia/patologia , Prognóstico , Estudos Retrospectivos , Sarcoma/mortalidade , Sarcoma/patologia , Adulto Jovem
2.
J Surg Oncol ; 120(4): 753-760, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31355444

RESUMO

BACKGROUND: Calls for multivisceral resection (MVR) of retroperitoneal sarcoma (RPS) are increasing, although the risks and benefits remain controversial. We sought to analyze current 30-day morbidity and mortality rates, and trends in utilization of MVR in a national database. METHODS: Overall morbidity, severe morbidity, mortality rates, and temporal trends were analyzed utilizing the American College of Surgeons National Surgical Quality Improvement Program (ACS-NSQIP). RESULTS: From 2012 to 2015, a total of 564 patients underwent RPS resection with 233 patients (41%) undergoing MVR. The MVR group had a higher rate of preoperative weight loss and larger tumors overall. When comparing MVR to non-MVR, there was no significant difference in overall morbidity (22% vs 17%, P = .13), severe morbidity (11% vs 8%, P = .18), or mortality (<1% vs 2%, P = .25). On multivariate analysis, MVR was not associated with increased overall morbidity or severe morbidity. Mortality rates were too low for meaningful statistical analysis. Annual rates of MVR ranged from 37% to 46% with no significant change over time (P = .47). RESULTS: Short-term morbidity and mortality rates after MVR for RPS remain acceptable, but rates of MVR show little change over time in NSQIP hospitals. Concerns about increased morbidity and mortality should not be viewed as a contraindication to wider implementation of MVR for RPS.


Assuntos
Mortalidade/tendências , Complicações Pós-Operatórias/mortalidade , Neoplasias Retroperitoneais/mortalidade , Sarcoma/mortalidade , Procedimentos Cirúrgicos Operatórios/mortalidade , Bases de Dados Factuais , Gerenciamento Clínico , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Morbidade , Prognóstico , Melhoria de Qualidade , Neoplasias Retroperitoneais/patologia , Neoplasias Retroperitoneais/cirurgia , Sarcoma/patologia , Sarcoma/cirurgia , Taxa de Sobrevida
3.
Br J Radiol ; 92(1102): 20190252, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31322910

RESUMO

OBJECTIVE: To assess the impact of adjuvant volumetric modulated arc therapy (VMAT) compared with three-dimensional conformal radiation therapy (3DCRT) in terms of toxicity and local control (LC) in patients with soft tissue sarcoma of the extremities. METHODS: From 2004 to 2016, 109 patients were treated, initially using 3DCRT and subsequently with VMAT. Clinical outcome was evaluated by contrast-enhanced MRI, thoracic and abdominal CT 3 months after treatments and then every 6 months. Toxicity was evaluated with Common Terminology Criteria for Adverse Events scale v. 4.3. RESULTS: Patients presented Stage III soft tissue sarcoma disease (77%), localized tumor (95%) at the lower extremity (87%), adipocytic histotype (46%). Surgical resection was performed in all patients, followed by adjuvant 3DCRT in 38, and VMAT in 71. The median total dose was 66 Gy/33 fractions (range 60-70 Gy;25-35 fractions). More successful bone sparing was recorded using VMAT (p < 0.001). Median follow-up was 61 months, 93 and 58 months for 3DCRT and VMAT group, respectively. The 2- and 5 year LC were 95.3±2.1%, and 87.4±3.4% for the whole cohort, 92.0±4.5%, 82.9±6.4% for 3DCRT, 97.1±2.0%, 89.6±4.1% for VMAT (p = 0.150). On univariate and multivariate analysis the factors recorded as conditioning LC were the status of the surgical resection margins (p = 0.028) and the total dose delivered (p = 0.013). CONCLUSION: The availability of modern radiotherapy technique permit a better conformity on the target with maximum sparing of normal tissue and acceptable side-effects. VMAT is a safe and feasible treatment with limited rate of toxicity, compared to 3DCRT. Results on LC of VMAT are encouraging. ADVANCES IN KNOWLEDGE: Soft tissue sarcoma of the extremities can benefit from the use of VMAT, with a reduction of the high dose to bones to avoid radiation osteonecrosis. An adequate total dose of at least 66 Gy and a radical surgical margin allow a good local control.


Assuntos
Radioterapia Conformacional/métodos , Radioterapia de Intensidade Modulada/métodos , Sarcoma/radioterapia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Análise de Variância , Meios de Contraste , Extremidades , Feminino , Humanos , Estimativa de Kaplan-Meier , Imagem por Ressonância Magnética/métodos , Masculino , Pessoa de Meia-Idade , Lesões por Radiação/etiologia , Dosagem Radioterapêutica , Radioterapia Adjuvante/efeitos adversos , Radioterapia Adjuvante/métodos , Radioterapia Conformacional/efeitos adversos , Radioterapia Conformacional/mortalidade , Radioterapia de Intensidade Modulada/efeitos adversos , Radioterapia de Intensidade Modulada/mortalidade , Estudos Retrospectivos , Sarcoma/diagnóstico por imagem , Sarcoma/mortalidade , Sarcoma/patologia , Adulto Jovem
4.
Cancer Sci ; 110(9): 2894-2904, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31348579

RESUMO

Nivolumab is a human monoclonal antibody against the immune checkpoint receptor programmed death-1, inhibiting binding to programmed death-ligand 1 or 2 (PD-L1 or PD-L2). This phase 2 study evaluated the efficacy and safety of nivolumab in patients with advanced/recurrent uterine cervical cancer, uterine corpus cancer, or soft tissue sarcoma (STS). Patients received nivolumab 240 mg at 2-week intervals. Primary endpoint was objective response rate; secondary endpoints included overall survival, progression-free survival, and safety. PD-L1 expression and microsatellite-instability (MSI) status were analyzed as potential efficacy biomarkers. Objective response rate was 25%, 23%, and 0% in patients with cervical cancer (n = 20), corpus cancer (n = 22), and STS (n = 21), respectively. The lower 80% confidence intervals of objective response rates in patients with cervical or corpus cancer exceeded the threshold rate (5%); the primary endpoint was met in cervical and corpus cancer, but not in STS. Median progression-free survival was 5.6, 3.4, and 1.4 months, and 6-month overall survival was 84%, 73%, and 86% in cervical cancer, corpus cancer, and STS, respectively. The objective response rate was higher in patients with cervical cancer with PD-L1-positive (n = 5/15; 33%) versus PD-L1-negative (n = 0/5; 0%) tumors. The two patients with corpus cancer classified as MSI-high responded; the six patients classified as microsatellite stable did not respond. Overall, nivolumab showed acceptable toxicity in all cohorts, with evidence of clinical activity in uterine cervical or corpus cancer, but not in STS. PD-L1 expression in cervical cancer and MSI-high in corpus cancer may predict clinical activity of nivolumab in these cancers.


Assuntos
Antineoplásicos/administração & dosagem , Recidiva Local de Neoplasia/tratamento farmacológico , Nivolumabe/administração & dosagem , Sarcoma/tratamento farmacológico , Neoplasias Uterinas/tratamento farmacológico , Adulto , Idoso , Antineoplásicos/efeitos adversos , Antígeno B7-H1/metabolismo , Esquema de Medicação , Feminino , Humanos , Infusões Intravenosas , Japão/epidemiologia , Instabilidade de Microssatélites , Pessoa de Meia-Idade , Recidiva Local de Neoplasia/genética , Recidiva Local de Neoplasia/mortalidade , Recidiva Local de Neoplasia/patologia , Nivolumabe/efeitos adversos , Receptor de Morte Celular Programada 1/antagonistas & inibidores , Receptor de Morte Celular Programada 1/metabolismo , Intervalo Livre de Progressão , Estudos Prospectivos , Sarcoma/genética , Sarcoma/mortalidade , Sarcoma/patologia , Análise de Sobrevida , Neoplasias Uterinas/genética , Neoplasias Uterinas/mortalidade , Neoplasias Uterinas/patologia , Útero/patologia
5.
Virchows Arch ; 475(1): 59-66, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-31177317

RESUMO

Sarcomatoid carcinomas recently came into the spotlight through genetic profiling studies and also as a distinct model of epithelial-mesenchymal transition. The literature on sarcomatoid carcinomas of gallbladder is limited. In this study, 656 gallbladder carcinomas (GBC) were reviewed. Eleven (1.7%) with a sarcomatoid component were identified and analyzed in comparison with ordinary GBC (O-GBC). Patients included 9 females and 2 males (F/M = 4.5 vs. 3.9) with a mean age-at-diagnosis of 71 (vs. 64). The median tumor size was 4.6 cm (vs. 2.5; P = 0.01). Nine patients (84%) presented with advanced stage (pT3/4) tumor (vs. 48%). An adenocarcinoma component constituting 1-75% of the tumor was present in nine, and eight had surface dysplasia/CIS; either in situ or invasive carcinoma was present in all cases. An intracholecystic papillary-tubular neoplasm was identified in one. Seven showed pleomorphic-sarcomatoid pattern, and four showed subtle/bland elongated spindle cells. Three had an angiosarcomatoid pattern. Two had heterologous elements. One showed few osteoclast-like giant cells, only adjacent to osteoid. Immunohistochemically, vimentin, was positive in six of six; P53 expression was > 60% in six of six, keratins in six of seven, and p63 in two of six. Actin, desmin, and S100 were negative. The median Ki67 index was 40%. In the follow-up, one died peri-operatively, eight died of disease within 3 to 8 months (vs. 26 months median survival for O-GBC), and two were alive at 9 and 15 months. The behavior overall was worse than ordinary adenocarcinomas in general but was not different when grade and stage were matched. In summary, sarcomatoid component is identified in < 2% of GBC. Unlike sarcomatoid carcinomas in the remainder of pancreatobiliary tract, these are seldom of the "osteoclastic" type and patients present with large/advanced stage tumors. Limited data suggests that these tumors are aggressive with rapid mortality unlike pancreatic osteoclastic ones which often have indolent behavior.


Assuntos
Adenocarcinoma/patologia , Biomarcadores Tumorais/análise , Carcinoma in Situ/patologia , Neoplasias da Vesícula Biliar/patologia , Neoplasias Complexas Mistas/patologia , Sarcoma/patologia , Adenocarcinoma/química , Adenocarcinoma/mortalidade , Adenocarcinoma/cirurgia , Idoso , Carcinoma in Situ/química , Carcinoma in Situ/mortalidade , Carcinoma in Situ/cirurgia , Feminino , Neoplasias da Vesícula Biliar/química , Neoplasias da Vesícula Biliar/mortalidade , Neoplasias da Vesícula Biliar/cirurgia , Humanos , Imuno-Histoquímica , Masculino , Pessoa de Meia-Idade , Invasividade Neoplásica , Neoplasias Complexas Mistas/química , Neoplasias Complexas Mistas/mortalidade , Neoplasias Complexas Mistas/cirurgia , Sarcoma/química , Sarcoma/mortalidade , Sarcoma/cirurgia , Fatores de Tempo , Resultado do Tratamento
6.
Int J Surg Oncol ; 2019: 2912361, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31186956

RESUMO

This study examines survival time in patients with small bowel tumors and determines its contributing factors. In this retrospective analytical study, the medical records of 106 patients with small bowel cancer (from 2006 to 2011) were investigated. The patients' data were extracted, including age, gender, clinical presentation, location of tumor, histological type, grade of tumor, site of metastasis, and type of treatment. The Kaplan-Meier test was used to estimate the overall survival time and the Log-rank test to compare the survival curves. The Cox regression was also used to evaluate the effect of the confounding variables on survival time. This study was conducted on 106 patients with a median age of 60 years (Min: 7, Max: 87). The tumor types included adenocarcinoma (n=78, 73.6%), MALToma (n=22, 20.8%), neuroendocrine tumors (n=4, 3.8%), and sarcoma (n=2. 1.8%). Grade 3 adenocarcinomas had a significantly lower survival time (HR: 1.48, 95% CI: 0.46-2.86; P=.001). Combined therapy (chemotherapy and surgery) vs. single-therapy (only surgery) had no significant effects on the survival of the patients with MALToma (5 vs. 3 months, 95% CI: 1.89-5.26; P=.06). There were no significant differences between the survival time in adenocarcinoma and MALToma (12 vs. 20 months, 95% CI: 6.24-24.76; P=.49). Tumor grade was the only independent prognostic factor that affected survival in adenocarcinoma. The patients diagnosed with MALToma in the study also had a poor prognosis, and the type of treatment had no significant effect on their survival.


Assuntos
Adenocarcinoma/mortalidade , Neoplasias Intestinais/mortalidade , Intestino Delgado , Linfoma de Zona Marginal Tipo Células B/mortalidade , Tumores Neuroendócrinos/mortalidade , Sarcoma/mortalidade , Adenocarcinoma/diagnóstico , Adenocarcinoma/patologia , Adenocarcinoma/terapia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Feminino , Seguimentos , Humanos , Neoplasias Intestinais/diagnóstico , Neoplasias Intestinais/patologia , Neoplasias Intestinais/terapia , Estimativa de Kaplan-Meier , Linfoma de Zona Marginal Tipo Células B/diagnóstico , Linfoma de Zona Marginal Tipo Células B/patologia , Linfoma de Zona Marginal Tipo Células B/terapia , Masculino , Pessoa de Meia-Idade , Tumores Neuroendócrinos/diagnóstico , Tumores Neuroendócrinos/patologia , Tumores Neuroendócrinos/terapia , Prognóstico , Estudos Retrospectivos , Sarcoma/diagnóstico , Sarcoma/patologia , Sarcoma/terapia , Análise de Sobrevida , Adulto Jovem
7.
J Surg Oncol ; 120(2): 183-192, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31042011

RESUMO

BACKGROUND AND OBJECTIVES: We investigated implant revision, implant failure, and amputation risk after limb-sparing bone tumor surgery using the Global Modular Replacement System (GMRS) tumor prosthesis in patients suffering from bone sarcomas (BS), giant cell tumors (GCT), or metastatic bone disease (MBD). MATERIAL AND METHODS: A retrospective study of a nationwide consecutive cohort (n = 119, 47 [12-81] years, M/F = 65/54) having limb-sparing surgery and reconstruction using the GMRS tumor prosthesis due to bone tumors (BS/GCT/MBD = 70/8/41) from 2005 to 2013. Anatomical locations were as followed: distal femur (n = 49), proximal femur (n = 41), proximal tibia (n = 26), or total femur (n = 3). Kaplan-Meier survival analysis and competing risk analysis with death as a competing risk were used for statistical analysis. RESULTS: For BS and GCT patients, 5-year patient survival was 72% (95% confidence interval [CI]: 59-85%) and for MBD 33% (95% CI: 19-48%). Thirty-two patients underwent revision surgery (5-year revision incidence 14%; 95% CI: 8-21%). Twelve patients had revision of bone-anchored parts (implant failure) with a 5-year revision incidence 6% (95% CI: 2-10%). Ten amputations were performed due to local relapse (n = 9) or recurrent infections (n = 1) with a 5-year incidence of amputation: 8% (95% CI: 3-13%). CONCLUSIONS: We identified a low risk of revision and amputation when using the GMRS tumor prosthesis for limb-sparing bone tumor.


Assuntos
Neoplasias Ósseas/cirurgia , Prótese Ancorada no Osso , Tumor de Células Gigantes do Osso/cirurgia , Sarcoma/cirurgia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Amputação , Neoplasias Ósseas/mortalidade , Neoplasias Ósseas/patologia , Criança , Feminino , Tumor de Células Gigantes do Osso/mortalidade , Tumor de Células Gigantes do Osso/patologia , Humanos , Masculino , Pessoa de Meia-Idade , Desenho de Prótese , Falha de Prótese , Reoperação , Estudos Retrospectivos , Sarcoma/mortalidade , Sarcoma/patologia , Resultado do Tratamento , Adulto Jovem
8.
J Surg Oncol ; 120(2): 193-199, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31042013

RESUMO

BACKGROUND: While survival after surgical treatment of extremity soft tissue sarcoma (STS) is traditionally reported as actuarial survival, conditional survival (CS) may be more clinically relevant as it accounts for time already survived. We compared actuarial survival and CS of STS patients. MATERIALS AND METHODS: We analyzed 567 patients who underwent surgery for localized extremity STS. Actuarial survival was estimated using the Kaplan-Meier method. Cox proportional hazards model was used to evaluate factors associated with disease-specific survival. Five-year CS (CS5) estimates at "χ" year(s) after surgery were calculated as CS5 = S(χ + 5)/S(χ). RESULTS: Whereas actuarial survival decreased over time, CS5 increased. The postsurgical 1-, 3-, and 5-year CS5 values were 84.5%, 90.0%, and 93.8%, respectively, whereas the 6-, 8-, and 10-year actuarial survival rates were 82.0%, 79.4%, and 78.5%, respectively. The calculated CS5 exceeded actuarial survival especially in patients with risk factors such as large tumor size and Federation Nationale des Centers de Lutte Contre le Cancer (FNCLCC) grades 2 and 3 tumors. Patients with tumor size ≥5 cm had an actuarial survival of 73.9% at 10 years compared to a CS5 of 95.4% in patients alive at 5 years. Likewise, patients with FNCLCC grade 3 tumors had an actuarial survival of 71.1% at 10 years compared to a CS5 of 96.0% in patients alive at 5 years. CONCLUSIONS: Survival estimation by determination of CS can be dynamic and accurate especially in high-risk patients. CS can be useful for survival prediction and clinical decision making in extremity STS patients.


Assuntos
Extremidades , Sarcoma/mortalidade , Sarcoma/cirurgia , Neoplasias de Tecidos Moles/mortalidade , Neoplasias de Tecidos Moles/cirurgia , Análise Atuarial , Feminino , Humanos , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Modelos de Riscos Proporcionais , Estudos Retrospectivos , Taxa de Sobrevida
9.
J Surg Oncol ; 120(2): 168-175, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31134646

RESUMO

BACKGROUND: A single-institution experience of pulmonary metastasectomy in soft tissue sarcoma (STS) was retrospectively reviewed. Our specific aim was to examine, whether the resection of pulmonary metastases could be curative. We also compared overall survival (OS) of patients after complete or incomplete pulmonary resection and nonsurgical treatment. METHODS: Between 1987 and 2016, 1580 patients were treated for STS with curative intent by Soft Tissue Sarcoma Group at Helsinki University Hospital, Finland. Three hundred forty-seven patients (22%) developed advanced disease and 130 STS patients (9%) developed pulmonary metastases as first systemic relapse. Seventy four patients (5%) were operated for lung metastases. RESULTS: Fifty-five patients (42%) had a complete and 19 (15%) incomplete resection. Fifty-six (43%) were unoperated. Median OS after complete or incomplete metastasectomy, chemotherapy, or best supportive care was 22, 18, 8, and 5 months, respectively. Twelve patients (9%) developed no further metastases and are alive with no evidence of disease. Disease-free survival (DFS) for completely resected patients was 17% at 5 years. All long-term survivors had oligometastatic disease and they underwent one to three complete metastasectomies. CONCLUSIONS: Complete pulmonary metastasectomy in STS results in 5 years DFS in nearly one-fifth of patients. Most of these patients are probably cured.


Assuntos
Neoplasias Pulmonares/secundário , Neoplasias Pulmonares/cirurgia , Metastasectomia , Pneumonectomia , Sarcoma/secundário , Sarcoma/cirurgia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Neoplasias Pulmonares/mortalidade , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Sarcoma/mortalidade , Taxa de Sobrevida , Resultado do Tratamento , Adulto Jovem
10.
J Surg Oncol ; 119(8): 1087-1098, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30977916

RESUMO

BACKGROUND: As the U.S. population ages, differences in oncologic outcomes among the elderly have been recognized. Our objective was to analyze the clinical, pathologic, and treatment outcomes for elderly soft tissue sarcoma (STS) patients, hypothesizing significant differences in the management and response to therapy. METHODS: Using the National Cancer Database, we identified 33 859 patients with nonmetastatic extremity STS. We defined elderly as ≥74 years in age and compared patient and treatment variables between adult and elderly patients. Cox-proportional hazards analysis was used to determine predictors of overall survival (OS). RESULTS: We identified 8504 elderly patients. Significant differences in histologic subtype, grade, and facility type between elderly and nonelderly patients (P < 0.05) exist. Elderly patients were less likely to undergo R0 resection (P = 0.001) and had a higher 90-day mortality (P = 0.001). Surgical elderly patients experienced superior OS compared with nonsurgical patients (P = 0.001). Among elderly patients, younger age, and female sex, lower Charlson-Deyo score, lower grade, smaller tumors, surgical resection, negative surgical margins, and radiation therapy were associated with better OS. CONCLUSIONS: Key differences exist in elderly extremity STS patients, including a narrower benefit/risk ratio with surgical management. These data highlight that elderly patients represent a distinct cohort for whom more careful selection appears indicated.


Assuntos
Sarcoma/terapia , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Estudos de Coortes , Extremidades/patologia , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Sarcoma/mortalidade , Sarcoma/patologia , Estados Unidos/epidemiologia
11.
J Surg Oncol ; 120(2): 109-116, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-30993699

RESUMO

INTRODUCTION: Sarcomas of the mandible are extremely rare tumors, with osteosarcoma being the most common, followed by Ewing's sarcoma MATERIALS AND METHODS: A retrospective review of the clinical records, imaging studies, and pathology slides of patients with sarcoma of the mandible at a Tertiary Care Cancer Center from 1998 to 2014 was undertaken. The impact of neoadjuvant chemotherapy and postoperative radiotherapy with or without chemotherapy was studied, and factors impacting upon local control and disease-specific survival were analyzed. RESULTS: Twenty-two patients were treated over the study period, comprising of 15 males and seven females. External swelling, intraoral growth, or facial numbness were the presenting symptoms. Eighteen patients had osteosarcoma and four had the Ewing's sarcoma. Nine patients received neoadjuvant chemotherapy. All but one patient underwent surgery. Eleven had negative margins, with 90% recurrence-free survival at 3 years, compared to 10 with positive or close margins, leading to 67% recurrence-free survival. None of the patients receiving neoadjuvant chemotherapy developed recurrence and all were alive at 3 years. The impact of postoperative radiotherapy or adjuvant chemotherapy was not statistically significant. CONCLUSIONS: Wide surgical resection with negative margins remains the hallmark of surgical treatment.


Assuntos
Neoplasias Mandibulares/diagnóstico , Neoplasias Mandibulares/terapia , Sarcoma/diagnóstico , Sarcoma/terapia , Adolescente , Adulto , Idoso , Criança , Terapia Combinada , Feminino , Humanos , Masculino , Neoplasias Mandibulares/mortalidade , Margens de Excisão , Pessoa de Meia-Idade , Recidiva Local de Neoplasia/epidemiologia , Prognóstico , Estudos Retrospectivos , Sarcoma/mortalidade , Taxa de Sobrevida , Adulto Jovem
12.
Int J Surg Oncol ; 2019: 2350157, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30956820

RESUMO

Objectives: This study aims to assess the impact of surgical margin and malignancy grade on overall survival (OS) and local recurrence free rate (LRFR) for soft tissue sarcomas (STS) of the thoracic wall. Methods: This retrospective cohort study identified 88 patients, diagnosed and treated surgically for a nonmetastatic STS located in the thoracic wall between 1995 and 2013, using the population based and validated Aarhus Sarcoma Registry and Danish Sarcoma Registry. The Kaplan-Meier method was used to estimate OS and LRFR. Multivariate Cox analyses were used to determine prognostic factors for OS and LRFR. Results: The 5-year OS was 55% (95% confidence interval (CI): 0.44-0.65) and 5-year LRFR was 77% (95% CI: 0.67-0.85). High malignancy grade and intralesional/marginal resection were identified as negative predictors for OS. High grade was the only prognostic factor associated with a lower LRFR. Conclusions: In this large, single institution, study tumor grade was the key predictor for OS and LRFR. Surgical margin only statistically significantly influenced mortality, not local recurrence.


Assuntos
Sarcoma/cirurgia , Neoplasias de Tecidos Moles/cirurgia , Neoplasias Torácicas/cirurgia , Parede Torácica/cirurgia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Seguimentos , Humanos , Masculino , Margens de Excisão , Pessoa de Meia-Idade , Gradação de Tumores , Recidiva Local de Neoplasia/epidemiologia , Recidiva Local de Neoplasia/mortalidade , Recidiva Local de Neoplasia/patologia , Prognóstico , Sistema de Registros , Estudos Retrospectivos , Sarcoma/mortalidade , Sarcoma/patologia , Neoplasias de Tecidos Moles/mortalidade , Neoplasias de Tecidos Moles/patologia , Análise de Sobrevida , Neoplasias Torácicas/mortalidade , Neoplasias Torácicas/patologia , Parede Torácica/patologia , Adulto Jovem
13.
Anticancer Res ; 39(4): 2007-2014, 2019 04.
Artigo em Inglês | MEDLINE | ID: mdl-30952744

RESUMO

BACKGROUND: Retroperitoneal sarcomas (RPS) are rare, histologically heterogeneous, and anatomically complex tumors. National Comprehensive Cancer Network guidelines recommend evaluation and management by multidisciplinary teams with experience in sarcoma. Our aim was to determine an appropriate hospital volume threshold for the treatment of RPS. PATIENTS AND METHODS: Patients undergoing resection of RPS were identified from the National Cancer Data Base (1998-2012). Multivariable modeling with restricted cubic splines was employed to examine the association between hospital volume and survival and identify possible hospital volume threshold. RESULTS: The study included 5,340 patients who underwent surgery at 909 different hospitals. Median annual volume was two cases per year. After adjustment, hospital volume was associated with improved survival (p=0.01), without cutoff. The cohort was then grouped into: Low-volume (≤5 cases/year), intermediate-volume (6-10 cases/year), and high-volume (>10 cases/year). The majority of patients were treated in low-volume hospitals (86%), compared to 9% in intermediate- and 5% in high-volume centers; 44% of patients were treated in hospitals that performed one case per year. Compared to low-volume, high-volume hospitals more often had patients with high-grade and larger tumors. Adjusted 90-day mortality was significantly lower in high- vs. low-volume hospitals (odds ratio(OR)=0.25, p=0.02). With adjustment, treatment in high- vs. low-volume hospitals was associated with lower odds of margin positivity (OR=0.58, p=0.001), and improved overall survival (hazard ratio(HR)=0.61, p=0.002). CONCLUSION: Treatment of RPS in high-volume centers is associated with significant reduction in short-term mortality and improved long-term survival. Hospital volume may be a surrogate for the infrastructure and support necessary for the optimal management of these complex malignancies.


Assuntos
Hospitais com Alto Volume de Atendimentos , Hospitais com Baixo Volume de Atendimentos , Doenças Raras/mortalidade , Neoplasias Retroperitoneais/mortalidade , Sarcoma/mortalidade , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Doenças Raras/cirurgia , Neoplasias Retroperitoneais/cirurgia , Sarcoma/cirurgia
14.
Zhonghua Zhong Liu Za Zhi ; 41(2): 140-145, 2019 Feb 23.
Artigo em Chinês | MEDLINE | ID: mdl-30862145

RESUMO

Objective: To evaluate the efficacy and prognostic factors of comprehensive treatment of undifferentiated high grade pleomorphic sarcoma (UHGPS) in extremities and trunk, including surgery, radiotherapy and chemotherapy. Methods: A retrospective analysis and follow-up of 131 UHGPS cases with clinical stage Ⅱ or Ⅲ in extremities and trunk soft tissue was performed to analyze the prognostic factors. Survival data were collected through follow-up. The survival rate was calculated with life table method and Kaplan-Meier survival curves were drawn. Survival rate between the two groups was compared using Log rank test. The multivariate analysis was performed using Cox regression model. Results: The median survival time of 131 patients was 41.6 months. The 1-year, 3-year and 5-year survival rates were 95.0%, 82.0%, and 77.0%, respectively. The 5-year recurrence-free survival rate was 81.0%, and the 5-year metastasis-free survival rate was 72.0%. Univariate analysis showed that the tumor size, initial or recurrence, surgical margin, AJCC stage, and with/without standard treatment were associated with overall survival (all P<0.05). Stratification analysis according to the American Joint Committee of Cancer (AJCC) stage showed that 5-year survival rate of stage Ⅱ patients with radiotherapy was 100.0%, which was higher than that of patients without radiotherapy (79.6%) and the difference was statistically significant (P=0.010); but no statistical significance of radiotherapy for stage Ⅲ and chemotherapy for stage Ⅱ or Ⅲ patients (all P>0.05). The multivariate analysis showed surgical margin (HR=4.220, P=0.002), with/without standard treatment (HR=4.040, P=0.030) were independent risk factors associated with prognosis of UHGPS patients. Conclusions: For UHGPS with stage Ⅱ or stage Ⅲ in extremities and trunk soft tissue, patients with complete resection and standard treatment have improved prognosis. Therefore, standard treatment, including extensive resection for the first surgery, should be performed according to expert consensus in order to increase the long-term survival rate. Adjuvant radiotherapy should be performed for stage Ⅱ patients.


Assuntos
Extremidades , Sarcoma/terapia , Seguimentos , Humanos , Análise Multivariada , Estadiamento de Neoplasias , Prognóstico , Estudos Retrospectivos , Sarcoma/mortalidade , Sarcoma/patologia , Taxa de Sobrevida
15.
BMC Cancer ; 19(1): 232, 2019 Mar 14.
Artigo em Inglês | MEDLINE | ID: mdl-30871494

RESUMO

BACKGROUND: Alternative lengthening of telomeres (ALT) is a telomerase-independent mechanism used by a broad range of neoplasms to maintain telomere length, permitting uncontrolled replication during their progression. ALT has been described in different types of sarcoma, but a comprehensive analysis of its clinical significance is still lacking. Therefore, we provide here the first meta-analysis on this topic. METHODS: We searched SCOPUS and PubMed through July 2018 to identify all studies that investigated the prognostic role of ALT in sarcomas. We considered the risk of death (risk ratio, RR) calculated as the number of death vs. total participants during follow-up in ALT+ versus ALT- patients as the primary outcome. The secondary outcome was the hazard ratio (HR), adjusted for the maximum number of covariates available, using ALT- patients as reference. RESULTS: Eight articles comprising a total of 551 patients with sarcomas (226 ALT+ and 325 ALT-) were selected. The ALT+ group showed a higher mitotic count and a higher tumor grade compared with the ALT- group (p < 0.01). Furthermore, we demonstrate a strong impact of ALT on survival. In fact, ALT+ patients showed a statistically significant higher risk of death than ALT- patients, when also considering data from multivariate analyses (RR = 1.50; 95% CI: 1.15-1.96; p = 0.003; HR = 2.02; 95% CI: 1.22-3.38; p = 0.007). CONCLUSIONS: Our results indicate that ALT is associated with an increased risk of death in patients with sarcoma. In these neoplasms, ALT should be taken into account for a precise prognostic stratification and design of potential therapeutic strategies.


Assuntos
Sarcoma/patologia , Homeostase do Telômero , Telômero/metabolismo , Humanos , Pessoa de Meia-Idade , Prognóstico , Modelos de Riscos Proporcionais , Medição de Risco , Sarcoma/metabolismo , Sarcoma/mortalidade , Análise de Sobrevida
16.
Surg Oncol ; 28: 96-102, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30851921

RESUMO

In the last decades, the deeper understanding of the biological basis of the disease, along with the advances of surgical techniques and oncologic multimodal treatments, have led to an overall increase of survival of cancer patients. However, significant amelioration of the prognosis of rare and under-investigated tumors such as soft tissue sarcoma is less evident. In this review, main changes in the surgical management of retroperitoneal sarcoma (RPS) are discussed in order to figure out whether actual improvement in RPS outcome has been occurred in the last years.


Assuntos
Neoplasias Retroperitoneais/mortalidade , Neoplasias Retroperitoneais/cirurgia , Sarcoma/mortalidade , Sarcoma/cirurgia , Humanos , Prognóstico , Neoplasias Retroperitoneais/patologia , Sarcoma/patologia , Taxa de Sobrevida
17.
Oncol Rep ; 41(4): 2241-2253, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30816547

RESUMO

Systemic treatment options for soft tissue sarcomas (STSs) have remained unchanged despite the need for novel drug candidates to improve STS outcomes. Drug repurposing involves the application of clinical drugs to different diseases, reducing development time, and cost. It has also become a fast and effective way to identify drug candidates. The present study used a computational method to screen three drug­gene interaction databases for novel drug candidates for the treatment of several common STS histologic subtypes through drug repurposing. STS survival­associated genes were generated by conducting a univariate cox regression analysis using The Cancer Genome Atlas survival data. These genes were then applied to three databases (the Connectivity Map, the Drug Gene Interaction Database and the L1000 Fireworks Display) to identify drug candidates for STS treatment. Additionally, pathway analysis and molecular docking were conducted to evaluate the molecular mechanisms of the candidate drug. Bepridil was identified as a potential candidate for several STS histologic subtype treatments by overlapping the screening results from three drug­gene interaction databases. The pathway analysis with the Kyoto Encyclopedia of Genes and Genomes predicted that Bepridil may target CRK, fibroblast growth factor receptor 4 (FGFR4), laminin subunit ß1 (LAMB1), phosphoinositide­3­kinase regulatory subunit 2 (PIK3R2), WNT5A, cluster of differentiation 47 (CD47), elastase, neutrophil expressed (ELANE), 15­hydroxyprostaglandin dehydrogenase (HPGD) and protein kinase cß (PRKCB) to suppress STS development. Further molecular docking simulation suggested a relatively stable binding selectivity between Bepridil and eight proteins (CRK, FGFR4, LAMB1, PIK3R2, CD47, ELANE, HPGD, and PRKCB). In conclusion, a computational method was used to identify Bepridil as a potential candidate for the treatment of several common STS histologic subtypes. Experimental validation of these in silico results is necessary before clinical translation can occur.


Assuntos
Antineoplásicos/farmacologia , Bepridil/farmacologia , Reposicionamento de Medicamentos/métodos , Redes Reguladoras de Genes/genética , Sarcoma/tratamento farmacológico , Antineoplásicos/química , Antineoplásicos/uso terapêutico , Bepridil/química , Bepridil/uso terapêutico , Biologia Computacional , Bases de Dados Genéticas/estatística & dados numéricos , Perfilação da Expressão Gênica , Regulação Neoplásica da Expressão Gênica/efeitos dos fármacos , Redes Reguladoras de Genes/efeitos dos fármacos , Humanos , Simulação de Acoplamento Molecular , Ligação Proteica , Sarcoma/genética , Sarcoma/mortalidade , Sarcoma/patologia , Análise de Sobrevida
18.
Oncol Res Treat ; 42(3): 95-100, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30820020

RESUMO

BACKGROUND: Germ cell tumors (GCTs) with malignant somatic transformation (MST) represent an uncommon variant of what is typically a curable malignancy. There is a paucity of data on time of somatic transformation, response to conventional therapy, and survival outcomes of different somatic subtypes. METHODS: After obtaining institutional review board (IRB) approval, we searched our institutional database from 1982 to 2017 and identified patients with GCTs with MST. Patient characteristics, pathologic description, treatment, and clinical outcomes were extracted from the medical records. RESULTS: We identified 24 cases of GCTs with MST; the MST was adenocarcinoma in 50% and sarcoma in 50%. Median age at diagnosis was 27. Alpha-fetoprotein and beta-human chorionic gonadotropin were undetectable in 44%, both were elevated in 54%. The majority were advanced stage (71% stage III), and International Germ Cell Cancer Collaborative Group (IGCCCG) risk was classified as 'good' in 60%. Median follow-up was 41 months (range 10-346 months). Median progression-free survival was 84 months (95% confidence interval (CI) 56-232), and median overall survival was 219 months (95% CI 165-not reported). CONCLUSION: Patients with GCTs with an MST appear to have poor responses to cisplatin-based chemotherapy, suggesting that somatic component-driven therapies should be considered. Furthermore, resection of residual disease when feasible is an essential component of management.


Assuntos
Adenocarcinoma/patologia , Diferenciação Celular , Neoplasias Embrionárias de Células Germinativas/patologia , Sarcoma/patologia , Neoplasias Testiculares/patologia , Adenocarcinoma/mortalidade , Adenocarcinoma/terapia , Adolescente , Adulto , Protocolos de Quimioterapia Combinada Antineoplásica/farmacologia , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Cisplatino/farmacologia , Cisplatino/uso terapêutico , Resistencia a Medicamentos Antineoplásicos , Humanos , Masculino , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Neoplasias Embrionárias de Células Germinativas/mortalidade , Neoplasias Embrionárias de Células Germinativas/terapia , Orquiectomia , Intervalo Livre de Progressão , Estudos Retrospectivos , Sarcoma/mortalidade , Sarcoma/terapia , Neoplasias Testiculares/mortalidade , Testículo/patologia , Testículo/cirurgia , Adulto Jovem
19.
J Cancer Res Clin Oncol ; 145(5): 1321-1330, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-30847552

RESUMO

PURPOSE: Neoadjuvant radiotherapy ± chemotherapy and wide local excision is an accepted management of localized soft tissue sarcomas (STS). Necrosis is prognostic for survival in osteosarcomas, but the significance for STS is undetermined. This study aimed to determine if percent true necrosis, opposed to a combination of necrosis and fibrosis, leads to improved survival in extremity and trunk STS. METHODS: From 2000 to 2015, 162 patients with STS were treated with neoadjuvant therapy and resection. Patient and tumor variables were reviewed, and resected specimens underwent pathological assessment. Necrosis was ratiometrically determined. Overall (OS), distant metastasis-free (DMFS), and progression-free survival (PFS) were calculated using Kaplan-Meier estimator. Survival was determined using the Fisher's exact test for univariate analysis (UVA) and logistic regression for multivariate analysis (MVA). RESULTS: Median follow-up was 4.5 years and median necrosis was 24.97%. Necrosis predicted worse OS, DMFS, and PFS on UVA, and DMFS and PFS on MVA. Necrosis was positively correlated with size and grade. To mitigate the role of size, a sub-analysis of ≥ 10 cm tumors was performed revealing necrosis predicted decreased DMFS and PFS on UVA and MVA. In high-grade tumors, necrosis correlated with decreased DMFS and PFS on UVA. Necrosis did not predict OS in ≥ 10 cm or high-grade tumors. CONCLUSIONS: Our data suggests necrosis may be an additional independent, prognostic variable with increased necrosis predicting a worse prognosis. Necrosis may not be a measure of treatment response and instead suggests more aggressive tumor biology as high-grade, large STS were associated with increased necrosis.


Assuntos
Necrose/patologia , Sarcoma/mortalidade , Sarcoma/patologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Biomarcadores , Quimiorradioterapia , Terapia Combinada , Comorbidade , Feminino , Humanos , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Terapia Neoadjuvante , Gradação de Tumores , Estadiamento de Neoplasias , Curva ROC , Sarcoma/radioterapia , Adulto Jovem
20.
World J Gastroenterol ; 25(5): 608-621, 2019 Feb 07.
Artigo em Inglês | MEDLINE | ID: mdl-30774275

RESUMO

BACKGROUND: Intrahepatic sarcomatoid chonalgiocarcinoma (s-CCC) is an extremely rare disease, accounting for less than 1% of hepatobiliary system malignancies, and its pathophysiology is not well known. On the hypothesis that its clinical, serologic, or radiologic diagnosis are not fully understood and its prognosis is poor, we investigated the distinguishing features of s-CCC compared with those of intrahepatic bile duct adenocarcinoma [cholangiocellular carcinoma (CCC)] in patients from a single center. AIM: To analyze the clinical, serologic, imaging, and histopathologic characteristics of intrahepatic s-CCC patients diagnosed in a single center. METHODS: The clinical, serologic, imaging, and histopathologic features of 227 patients diagnosed with intrahepatic cholangiocarcinoma (IHCC) in a single medical center during the last 17 years were analyzed. The characteristics of 11 patients with s-CCC were compared with those of 216 patients with CCC. RESULTS: The number of patients with s-CCC who presented fever and abdominal pain and past history of chronic viral hepatitis or liver cirrhosis (LC) was higher than that of patients with CCC. In imaging studies, patients with s-CCC showed relatively aggressive features. However, no clear distinction was observed between s-CCC and CCC based on other clinical, serologic or radiologic examination results. An accurate diagnosis could be made only via a histopathologic examination through immunohistochemical staining. The clinical course of s-CCC was generally aggressive, and patients had a relatively poor prognosis. CONCLUSION: In patients with s-CCC, early diagnosis through biopsy and aggressive treatment, including surgical resection, are important.


Assuntos
Neoplasias dos Ductos Biliares/patologia , Ductos Biliares Intra-Hepáticos/patologia , Colangiocarcinoma/patologia , Doenças Raras/patologia , Sarcoma/patologia , Idoso , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Neoplasias dos Ductos Biliares/diagnóstico , Neoplasias dos Ductos Biliares/mortalidade , Neoplasias dos Ductos Biliares/terapia , Ductos Biliares Intra-Hepáticos/diagnóstico por imagem , Ductos Biliares Intra-Hepáticos/cirurgia , Biomarcadores Tumorais/sangue , Biópsia , Quimioterapia Adjuvante/métodos , Colangiocarcinoma/diagnóstico , Colangiocarcinoma/mortalidade , Colangiocarcinoma/terapia , Feminino , Seguimentos , Hepatectomia , Humanos , Estimativa de Kaplan-Meier , Testes de Função Hepática , Imagem por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Prognóstico , Doenças Raras/diagnóstico , Doenças Raras/mortalidade , Doenças Raras/terapia , República da Coreia/epidemiologia , Estudos Retrospectivos , Sarcoma/diagnóstico , Sarcoma/mortalidade , Sarcoma/terapia , Tomografia Computadorizada por Raios X , Resultado do Tratamento , Ultrassonografia
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