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1.
Anticancer Res ; 39(10): 5605-5610, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31570456

RESUMO

BACKGROUND/AIM: We analyzed the process of healing at osteotomy sites and timing of achievement of full weight-bearing in sarcoma patients who underwent hemicortical or intercalary reconstruction using the extracorporeal irradiated autologous bone graft technique. PATIENTS AND METHODS: We studied 10 patients who had undergone tumor resection and reconstruction with hemicortical extracorporeal irradiated autologous bone graft at mid-shaft femur or tibia. The control group consisted of 30 patients who received the reconstruction using intercalary bone graft. RESULTS: Full weight-bearing was achieved in all 10 patients at a median time of 4.8 months. Function was excellent in all patients. When comparing the clinical outcome among the patients who received intercalary and hemicortical grafts, the duration to full weight-bearing achievement in patients who received hemicortical graft was shorter than that in those with intercalary graft. CONCLUSION: Early full weight-bearing may be achieved in patients undergoing hemicortical resection and reconstruction using extracorporeal irradiated autologous bone graft.


Assuntos
Neoplasias Ósseas/patologia , Neoplasias Ósseas/cirurgia , Sarcoma/patologia , Sarcoma/cirurgia , Neoplasias de Tecidos Moles/patologia , Neoplasias de Tecidos Moles/cirurgia , Adulto , Idoso , Idoso de 80 Anos ou mais , Transplante Ósseo/métodos , Osso e Ossos/patologia , Osso e Ossos/cirurgia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Osteotomia , Transplante Autólogo/métodos , Resultado do Tratamento
2.
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
3.
Anticancer Res ; 39(8): 3981-3989, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31366479

RESUMO

Uterine sarcomas are rare but very aggressive. Uterine myomas, on the other hand, are the most common benign tumors of the uterus. Currently there is no diagnostic technique available to distinguish them with certainty. This study aimed to summarize the published literature concerning protein-based biomarkers in the peripheral blood that can assist in this difficult differential diagnosis. In total, 48 articles, published between 1990 and 2017, were included. Most studies (n=37) concerned soft tissue sarcomas, while 11 discussed uterine sarcomas specifically. Vascular endothelial growth factor (VEGF), basic fibroblast growth factor (bFGF), interleukins (IL), cancer antigen 125 (CA 125), lactate dehydrogenase, gangliosides (LDH) and growth differentiation factor 15 (GDF-15) are the most studied proteins in soft tissue sarcomas, including uterine sarcomas. Future research on improving sarcoma diagnosis should include these proteins.


Assuntos
Leiomioma/sangue , Neoplasias/sangue , Sarcoma/sangue , Neoplasias Uterinas/sangue , Biomarcadores Tumorais/sangue , Diferenciação Celular/genética , Feminino , Regulação Neoplásica da Expressão Gênica , Humanos , Leiomioma/patologia , Neoplasias Musculares/sangue , Neoplasias Musculares/patologia , Neoplasias/patologia , Sarcoma/patologia , Neoplasias Uterinas/patologia
4.
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
5.
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
6.
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
7.
Pathologe ; 40(4): 353-365, 2019 Jul.
Artigo em Alemão | MEDLINE | ID: mdl-31168638

RESUMO

Soft tissue neoplasms with myxoid features are collectively not uncommon. Their often complex differential diagnosis makes them significantly over-represented among consultation cases. This applies not only to sarcomas but in particular to benign lesions as well. Generally, myxoid soft tissue lesions are divided into two major groups: (1) myxoid lesions by definition (which can however rarely be non-myxoid) and (2) rare myxoid variants of otherwise non-myxoid entities.Four major diagnostic challenges are responsible for the complexity of myxoid soft tissue neoplasms: (1) Diagnosis of malignancy in many cases is not based on conventional malignancy criteria but is defined by the entity itself, making under-diagnosis of malignancy likely in entities such as low-grade fibromyxoid sarcoma. (2) On the other hand, harmless myxoid lesions with features of high proliferation, e.g. nodular and proliferative fasciitis, tend to be over-diagnosed as malignant by the unworried. (3) The necessity to assess not only cellular morphology/differentiation, but also the stromal, vascular and architectural characteristics adds to the complexity of the differential diagnostic algorithm. (4) Last but not least, recognition of unexpected myxoid variants of non-myxoid entities is basically impossible if focal conventional areas are absent, underlining the need for high suspicion index and sufficient sampling.This review illuminates the various aspects related to the differential diagnostic workup of these challenging entities.


Assuntos
Fibrossarcoma , Sarcoma , Neoplasias de Tecidos Moles , Algoritmos , Diagnóstico Diferencial , Fibrossarcoma/diagnóstico , Humanos , Sarcoma/diagnóstico , Sarcoma/patologia , Neoplasias de Tecidos Moles/diagnóstico , Neoplasias de Tecidos Moles/patologia
8.
Pathologe ; 40(4): 339-352, 2019 Jul.
Artigo em Alemão | MEDLINE | ID: mdl-31240452

RESUMO

More than 20% of soft-tissue tumors belong to the group of adipocytic neoplasms. Difficulties may occur in the differential diagnosis of lipomas versus atypical lipomatous tumors/well-differentiated liposarcomas, in the distinction of dedifferentiated liposarcomas from other soft-tissue sarcoma entities and in the detailed subtyping of liposarcomas. Especially in biopsies, the correct diagnosis and grading may be hampered due to limited tissue. Because of the ever-increasing molecular-pathological knowledge of soft-tissue tumors and the rising distribution of molecular diagnostic assays in institutes of pathology, differential diagnosis has been facilitated, as more than 90% of adipocytic tumors carry more or less specific genomic alterations. In the following, the most important subtypes of adipocytic tumors are described morphologically and genomically.


Assuntos
Lipoma , Lipossarcoma , Neoplasias Lipomatosas , Sarcoma , Neoplasias de Tecidos Moles , Humanos , Lipoma/diagnóstico , Lipoma/patologia , Lipossarcoma/diagnóstico , Lipossarcoma/patologia , Neoplasias Lipomatosas/diagnóstico , Neoplasias Lipomatosas/patologia , Sarcoma/diagnóstico , Sarcoma/patologia , Neoplasias de Tecidos Moles/genética , Neoplasias de Tecidos Moles/patologia
9.
Pathologe ; 40(4): 393-411, 2019 Jul.
Artigo em Alemão | MEDLINE | ID: mdl-31243551

RESUMO

Epithelioid soft tissue tumors consist exclusively of epithelioid tumor cells. Biphasic tumors are composed of both a spindle-cell and an epithelioid component. The rare mixed tumors of soft tissue show a broader variety of cellular and stromal differentiation but also include at least one, possibly several, epithelioid portions.The close morphological similarity of some of these entities with each other, as well as with the more frequent soft tissue metastases of carcinomas, carcinosarcomas, and melanomas, to malignant mesothelioma and certain lymphomas, can often make the correct diagnosis extremely difficult. Recent advances in the detection of certain molecular alterations (mostly chromosomal translocations) have contributed to changes in tumor classification but also to improved pathological diagnostics (e.g. through the development of potent diagnostic antibodies) and biological understanding.The present overview should help the pathologist in the diagnosis of these rare tumors through the classical approach of morphological pattern recognition. The most important entities are discussed and illustrated in more detail, with the incorporation of the latest immunohistochemical and molecular aspects and the differential diagnosis of similar tumors.


Assuntos
Mesotelioma , Sarcoma , Neoplasias de Tecidos Moles , Biomarcadores Tumorais , Diagnóstico Diferencial , Humanos , Imuno-Histoquímica , Mesotelioma/diagnóstico , Mesotelioma/patologia , Sarcoma/patologia , Neoplasias de Tecidos Moles/diagnóstico , Neoplasias de Tecidos Moles/patologia
10.
Ann R Coll Surg Engl ; 101(6): 405-410, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-31155889

RESUMO

INTRODUCTION: The aims of this study were to report the presenting characteristics and identify how best to distinguish bone and soft-tissue infections that mimic sarcomas. MATERIALS AND METHODS: A total of 238 (211 osteomyelitis and 27 soft-tissue infections) patients referred to a tertiary sarcoma multidisciplinary team with suspected sarcoma who were ultimately diagnosed with a bone or soft tissue infection were included. Data from a prospectively collated database was analysed retrospectively. RESULTS: Of all possible bone and soft-tissue sarcoma referrals, a diagnosis of infection was made in 2.1% and 0.7%, respectively. Median age was 18 years in the osteomyelitis group and 46 years in the soft-tissue infection group. In the osteomyelitis group, the most common presenting features were pain (85.8%) and swelling (32.7%). In the soft-tissue infection group, the most common clinical features were swelling (96.3%) and pain (70.4%). Those in the soft-tissue group were more likely to have raised inflammatory markers. Radiological investigations were unable to discern between tumour or infection in 59.7% of osteomyelitis and 81.5% of soft-tissue infection cases. No organism was identified in 64.9% of those who had a percutaneous biopsy culture. CONCLUSIONS: This study has highlighted that infection is frequently clinically indistinguishable from sarcoma and remains a principle non-neoplastic differential diagnosis. When patients are investigated for suspected sarcoma, infections can be missed due to falsely negative radiological investigations and percutaneous biopsy. As no single clinical, biochemical or radiological feature or investigation can be relied upon for diagnosis, clinicians should have a low threshold for tissue biopsy and discussion in a sarcoma multidisciplinary team meeting.


Assuntos
Neoplasias Ósseas/diagnóstico , Comunicação Interdisciplinar , Osteomielite/diagnóstico , Equipe de Assistência ao Paciente , Sarcoma/diagnóstico , Infecções dos Tecidos Moles/diagnóstico , Neoplasias de Tecidos Moles/diagnóstico , Biópsia , Neoplasias Ósseas/patologia , Osso e Ossos/patologia , Diagnóstico Diferencial , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Osteomielite/patologia , Estudos Retrospectivos , Sarcoma/patologia , Infecções dos Tecidos Moles/patologia , Neoplasias de Tecidos Moles/patologia
11.
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
12.
J Surg Oncol ; 120(3): 325-331, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31172531

RESUMO

BACKGROUND AND OBJECTIVES: Soft-tissue sarcomas (STSs) are often treated with resection and radiation (RT)±chemotherapy. The role of RT in decreasing resection width to achieve local control is unclear. We evaluated RT on margin width to achieve local control and local recurrence (LR). METHODS: From 2000 to 2016, 514 patients with localized STS were identified from the US Sarcoma Collaborative database. Patients were stratified by a margin and local control was compared amongst treatment groups. RESULTS: LR was 9% with positive, 4.2% with ≤1 mm, and 9.3% with >1 mm margins (P = .315). In the ≤1 mm group, LR was 5.7% without RT, 0% with preoperative RT, and 0% with postoperative RT (P < .0001). In the >1 mm group, LR was 10.2%, 0%, and 3.7% in the no preoperative and postoperative RT groups, respectively (P = .005). RT did not influence LR in patients with positive margins. In stage I-III and II-III patients, local recurrence-free survival was higher following RT (P = .008 and P = .05, respectively). CONCLUSIONS: RT may play a larger role in minimizing LR than margin status. In patients with positive margins, RT may decrease LR to similar rates as a negative margin without RT and may be considered to decrease the risk of LR with anticipated close/positive margins.


Assuntos
Sarcoma/radioterapia , Sarcoma/cirurgia , Adulto , Idoso , Idoso de 80 Anos ou mais , Quimioterapia Adjuvante , Estudos de Coortes , Feminino , Humanos , Masculino , Margens de Excisão , Pessoa de Meia-Idade , Terapia Neoadjuvante , Estadiamento de Neoplasias , Estudos Retrospectivos , Sarcoma/tratamento farmacológico , Sarcoma/patologia , Adulto Jovem
13.
J Surg Oncol ; 120(3): 332-339, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31172536

RESUMO

OBJECTIVE: Our aim was to compare outcomes in patients who underwent unplanned excisions (UE) of soft-tissue sarcomas (STS) against patients with planned excisions (PE). METHODS: The retrospective 7-institution US Sarcoma Collaborative database was used. Patients with curative-intent resection of truncal/extremity STS between 2000 and 2016 were included. Propensity score weighting analysis (PSWA) was performed. Endpoints were locoregional recurrence-free survival (LRFS), distant metastasis-free survival (DMFS), and disease-specific survival (DSS). RESULTS: One thousand five hundred and ninety-six patients were included. Eighty-two percent (n = 1315) underwent PE and 18% (n = 281) underwent UE. Compared with PE, patients with UE were younger with smaller tumors with similar tumor grade. Unmatched analysis revealed PE was associated with worse DMFS (hazard ratio [HR] 1.95, P = .009) and DSS (HR 1.78, P = .039), but not LRFS compared with UE. On PSWA, UE had earlier LRFS (3-year LRFS: 80.5% vs 89.8%, P = .039), but not DMFS or DSS. By grade, patients with high-grade tumors and UE had worse LRFS (1-year LRFS: 90% vs 94%, P = .015), but similar DMFS and DSS compared with PE. In low-grade patients, UE and PE had similar LRFS, DMFS, or DSS. CONCLUSIONS: UE of STS is not associated with worse prognosis compared to PE, though UE is associated with earlier locoregional recurrence in patients with high-grade tumors. Multimodality therapy is needed to achieve improved outcomes in these patients.


Assuntos
Sarcoma/cirurgia , Estudos de Coortes , Procedimentos Cirúrgicos de Citorredução/métodos , Extremidades/patologia , Extremidades/cirurgia , Feminino , Humanos , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Pontuação de Propensão , Estudos Retrospectivos , Sarcoma/patologia , Tronco/patologia , Tronco/cirurgia , Resultado do Tratamento
14.
J Surg Oncol ; 120(3): 340-347, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31246290

RESUMO

BACKGROUND AND OBJECTIVES: Resection of primary retroperitoneal sarcomas (RPS) has a high incidence of recurrence. This study aims to identify patterns of recurrence and its impact on overall survival. METHODS: Adult patients with primary retroperitoneal soft tissue sarcomas who underwent resection in 2000-2016 at eight institutions of the US Sarcoma Collaborative were evaluated. RESULTS: Four hundred and ninety-eight patients were analyzed, with 56.2% (280 of 498) having recurrences. There were 433 recurrences (1-8) in 280 patients with 126 (25.3%) being locoregional, 82 (16.5%) distant, and 72 (14.5%) both locoregional and distant. Multivariate analyses revealed the following: Patient age P = .0002), tumor grade (P = .02), local recurrence (P = .0003) and distant recurrence (P < .0001) were predictors of disease-specific survival. The 1-, 3-, and 5-year survival rate for patients who recurred vs not was 89.6% (standard error [SE] 1.9) vs 93.5% (1.8), 66.0% (3.2) vs 88.4% (2.6), and 51.8% (3.6) vs 83.9% (3.3), respectively, P < .0001. Median survival was 5.3 years for the recurrence vs 11.3+ years for the no recurrence group (P < .0001). Median survival from the time of recurrence was 2.5 years. CONCLUSIONS: Recurrence after resection of RPS occurs in more than half of patients independently of resection status or perioperative chemotherapy and is equally distributed between locoregional and distant sites. Recurrence is primarily related to tumor biology and is associated with a significant decrease in overall survival.


Assuntos
Recidiva Local de Neoplasia/epidemiologia , Neoplasias Retroperitoneais/epidemiologia , Neoplasias Retroperitoneais/cirurgia , Sarcoma/epidemiologia , Sarcoma/cirurgia , Procedimentos Cirúrgicos de Citorredução/estatística & dados numéricos , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Recidiva Local de Neoplasia/patologia , Estadiamento de Neoplasias , Neoplasias Retroperitoneais/patologia , Estudos Retrospectivos , Sarcoma/patologia , Estados Unidos/epidemiologia
16.
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
17.
Medicine (Baltimore) ; 98(23): e15874, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-31169692

RESUMO

RATIONALE: Pulmonary artery sarcomas (PAS) are easily misdiagnosed as thromboembolic disease of pulmonary arteries, because of rarity and presenting with nonspecific signs, symptoms, or imaging findings. PATIENT CONCERNS: A 26-year-old man was admitted to the department of invasive technology with fever and dyspnea. Blood tests showed inflammatory activity, a slight increase of D-dimer and Fibrin Degradation Product. A chest enhanced computed tomography (CT) scanning revealed multiple filling defects occurred in the main trunk of both pulmonary arteries and branches of the left pulmonary artery DIAGNOSES:: It was initially diagnosed with pulmonary embolism (PE) and deep vein thrombosis (DVT), but was eventually diagnosed with pulmonary artery sarcoma that was confirmed by biopsy. INTERVENTIONS: The transcatheter thrombolysis therapy, inferior vena cava filter implantation, and operation were performed. OUTCOMES: The Organized mass was removed by the operation and was pathologically diagnosed as pulmonary artery sarcoma, the patient received postoperative chemotherapy according to the recommendation of oncology department. LESSONS: Coagulation markers have been reported to differentiate PAS from PE, but this case suggested that PAS can be associated with DVT and abnormal coagulation-fibrinolysis system.


Assuntos
Artéria Pulmonar/patologia , Sarcoma/diagnóstico , Neoplasias Vasculares/diagnóstico , Trombose Venosa/diagnóstico , Adulto , Erros de Diagnóstico , Humanos , Masculino , Trombólise Mecânica , Artéria Pulmonar/cirurgia , Sarcoma/patologia , Sarcoma/cirurgia , Tomografia Computadorizada por Raios X , Neoplasias Vasculares/patologia , Neoplasias Vasculares/cirurgia , Filtros de Veia Cava , Trombose Venosa/terapia
18.
Eur J Radiol ; 115: 39-45, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-31084757

RESUMO

OBJECTIVES: To explore whether MRI and radiomic features can differentiate uterine sarcoma from atypical leiomyoma. And to compare diagnostic performance of radiomic model with radiologists. METHODS: 78 patients (29 sarcomas, 49 leiomyomas) imaged with pelvic MRI prior to surgery were included in this retrospective study. Certain clinical and MRI features were evaluated for one lesion per patient. Radiological diagnosis was made based on MRI features. A radiomic model using automated texture analysis based on ADC maps was built to predict pathological results. The association between MRI features and pathological results was determined by multivariable logistic regression after controlling for other variables in univariate analyses with P < 0.05. The diagnostic efficacy of radiologists and radiomic model were compared by area under the receiver-operating characteristic curve (AUC), sensitivity, specificity and accuracy. RESULTS: In univariate analyses, patient's age, menopausal state, intratumor hemorrhage, tumor margin and uterine endometrial cavity were associated with pathological results, P < 0.05. Patient's age, tumor margin and uterine endometrial cavity remained significant in a multivariable model, P < 0.05. Diagnosis efficacy of radiologists based on MRI reached an AUC of 0.752, sensitivity of 58.6%, specificity of 91.8%, and accuracy of 79.5%. The optimal radiomic model reached an AUC of 0.830, sensitivity of 76.0%, average specificity of 73.2%, and accuracy of 73.9%. CONCLUSIONS: Ill-defined tumor margin and interrupted uterine endometrial cavity of older women were predictors of uterine sarcoma. Radiomic analysis was feasible. Optimal radiomic model showed comparable diagnostic efficacy with experienced radiologists.


Assuntos
Leiomioma/patologia , Sarcoma/patologia , Neoplasias Uterinas/patologia , Adulto , Idoso , Diagnóstico Diferencial , Feminino , Humanos , Imagem por Ressonância Magnética/métodos , Pessoa de Meia-Idade , Curva ROC , Estudos Retrospectivos , Sensibilidade e Especificidade
19.
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
20.
Adv Exp Med Biol ; 1123: 95-118, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31016597

RESUMO

Sarcomas represent an extensive group of divergent malignant diseases, with the only common characteristic of being derived from mesenchymal cells. As such, sarcomas are by definition very heterogeneous, and this heterogeneity does not manifest only upon intertumoral comparison on a bulk tumor level but can be extended to intratumoral level. Whereas part of this intratumoral heterogeneity could be understood in terms of clonal genetic evolution, an essential part includes a hierarchical relationship between sarcoma cells, governed by both genetic and epigenetic influences, signals that sarcoma cells are exposed to, and intrinsic developmental programs derived from sarcoma cells of origin. The notion of this functional hierarchy operating within each tumor implies the existence of sarcoma stem cells, which may originate from mesenchymal stem cells, and indeed, mesenchymal stem cells have been used to establish several crucial experimental sarcoma models and to trace down their respective stem cell populations. Mesenchymal stem cells themselves are heterogeneous, and, moreover, there are alternative possibilities for sarcoma cells of origin, like neural crest-derived stem cells, or mesenchymal committed precursor cells, or - in rhabdomyosarcoma - muscle satellite cells. These various origins result in substantial heterogeneity in possible sarcoma initiation. Genetic and epigenetic changes associated with sarcomagenesis profoundly impact the biology of sarcoma stem cells. For pediatric sarcomas featuring discrete reciprocal translocations and largely stable karyotypes, the translocation-activated oncogenes could be crucial factors that confer stemness, principally by modifying transcriptome and interfering with normal epigenetic regulation; the most extensively studied examples of this process are myxoid/round cell liposarcoma, Ewing sarcoma, and synovial sarcoma. For adult sarcomas, which have typically complex and unstable karyotypes, stemness might be defined more operationally, as a reflection of actual assembly of genetically and epigenetically conditioned stemness factors, with dedifferentiated liposarcoma providing a most thoroughly studied example. Alternatively, stemness can be imposed by tumor microenvironment, as extensively documented in osteosarcoma. In spite of this heterogeneity in both sarcoma initiation and underlying stemness biology, some of the molecular mechanisms of stemness might be remarkably similar in diverse sarcoma types, like abrogation of classical tumor suppressors pRb and p53, activation of Sox-2, or inhibition of canonical Wnt/ß-catenin signaling. Moreover, even some stem cell markers initially characterized for their stem cell enrichment capacity in various carcinomas or leukemias seem to function quite similarly in various sarcomas. Understanding the biology of sarcoma stem cells could significantly improve sarcoma patient clinical care, leading to both better patient stratification and, hopefully, development of more effective therapeutic options.


Assuntos
Sarcoma/patologia , Células-Tronco/citologia , Epigênese Genética , Humanos , Sarcoma de Ewing , Sarcoma Sinovial
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