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1.
Bone Joint J ; 103-B(9): 1541-1549, 2021 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-34465152

RESUMO

AIMS: While a centralized system for the care of patients with a sarcoma has been advocated for decades, regional variations in survival remain unclear. The aim of this study was to investigate regional variations in survival and the impact of national policies in patients with a soft-tissue sarcoma (STS) in the UK. METHODS: The study included 1,775 patients with a STS who were referred to a tertiary sarcoma centre. The geographical variations in survival were evaluated according to the periods before and after the issue of guidance by the National Institute for Health and Care Excellence (NICE) in 2006 and the relevant evolution of regional management. RESULTS: There had been a significant difference in survival between patients referred from the North East, North West, East Midlands, West Midlands, South West, and Wales in the pre-NICE era (five-year disease-specific survival (DSS); South West, 74% vs North East, 47% (p = 0.045) or West Midlands, 54% (p = 0.049)), which was most evident for patients with a high-grade STS. However, this variation disappeared in the post-NICE era, in which the overall DSS for high-grade STS improved from 47% to 68% at five years (p < 0.001). Variation in the size of the tumour closely correlated with the variation in DSS, and the overall size of the tumour and incidence of metastasis at the time of diagnosis also decreased after the national policies were issued. CONCLUSION: The survival of patients with a STS improved and regional variation corrected after the introduction of national policies, as a result of a decreasing size of tumour and incidence of metastasis at the time of diagnosis, particularly in patients with a high-grade STS. This highlights the positive impact of national guidelines on regional variation in the presentation, management, and outcome in patients with a STS. Cite this article: Bone Joint J 2021;103-B(9):1541-1549.


Assuntos
Extremidades , Política de Saúde , Sarcoma/mortalidade , Neoplasias de Tecidos Moles/mortalidade , Neoplasias Torácicas/mortalidade , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Pré-Escolar , Feminino , Humanos , Incidência , Lactente , Recém-Nascido , Masculino , Pessoa de Meia-Idade , Gradação de Tumores , Metástase Neoplásica , Características de Residência , Estudos Retrospectivos , Medicina Estatal , Taxa de Sobrevida , Reino Unido/epidemiologia
2.
Anticancer Res ; 41(8): 4027-4032, 2021 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-34281869

RESUMO

BACKGROUND/AIM: We aimed to investigate the effectiveness of postoperative radiotherapy (RT) on local recurrence-free survival (LRFS) in high-grade infiltrative soft tissue sarcomas (STSs) and determine its prognostic factors. PATIENTS AND METHODS: This was a retrospective cohort study and included 132 patients with high-grade STSs. Patients were divided into two groups: Group RT (n=48) who underwent postoperative RT and Group No-RT (n=84) who underwent only surgery. We analysed 5-year LRFS and its prognostic factors between these groups. Furthermore, 5-year LRFS in infiltrative and non-infiltrative STSs were evaluated. RESULTS: Five-year LRFS was not significantly different in Group RT (83.6%) and Group No-RT (79.6%) (p=0.698). Overall, significant prognostic factors influencing LRFS were age at diagnosis (p=0.02) and tumour growth pattern (p=0.04). Postoperative RT was less effective in the infiltrative than in non-infiltrative pattern of STSs. CONCLUSION: Postoperative RT does not influence local recurrence outcomes in infiltrative STSs.


Assuntos
Neoplasias de Tecidos Moles/radioterapia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Intervalo Livre de Doença , Feminino , Humanos , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Recidiva Local de Neoplasia/mortalidade , Recidiva Local de Neoplasia/patologia , Período Pós-Operatório , Estudos Retrospectivos , Neoplasias de Tecidos Moles/mortalidade , Neoplasias de Tecidos Moles/patologia , Adulto Jovem
3.
Am J Clin Oncol ; 44(8): 419-422, 2021 08 01.
Artigo em Inglês | MEDLINE | ID: mdl-34028371

RESUMO

BACKGROUND: Epithelioid hemangioendothelioma (EHE) is a malignant vascular neoplasm representing ∼1% of sarcomas. Due to its rarity, its clinical course is not well characterized and optimal treatment remains unknown. MATERIALS AND METHODS: This was a retrospective review of patients with EHE treated at Stanford University between 1998 and 2020. Demographic characteristics, pathology results, treatment modalities, and clinical outcomes were collected from the electronic medical records. RESULTS: A total of 58 patients had a mean age of 50.6 years and a slight female predominance (52%). Primary disease sites were liver (33%), soft tissue (29%), lung (14%), bone (9%), and mediastinum (9%). A majority (55%) had advanced or metastatic disease. Median overall survival (OS) was 16.9 years, with OS 89% at 1 year, 68% at 5 years, and 64% at 10 years. The longest median OS was associated with soft tissue sites and shortest with lung and mediastinal disease (P=0.03). The localized disease had improved median OS compared with metastatic disease (P=0.02). There was no OS difference between tumors >3 cm and those equal or smaller (P=0.85). Surgery was a common treatment (71%), while radiation and ablation were sometimes used (28% and 9%, respectively). The median time to initiating therapy of any kind was 68 days. The median time to systemic therapy was 114 days. CONCLUSIONS: We report on the clinical characteristics and outcomes of patients with EHE at a large academic center. Treatment options included surgical excision, liver transplant, ablation, radiation, and systemic therapy. A subset of patients had indolent disease not requiring treatment upfront.


Assuntos
Hemangioendotelioma Epitelioide/mortalidade , Hemangioendotelioma Epitelioide/terapia , Antineoplásicos/uso terapêutico , Neoplasias Ósseas/mortalidade , Neoplasias Ósseas/patologia , Neoplasias Ósseas/terapia , Feminino , Hemangioendotelioma Epitelioide/patologia , Humanos , Estimativa de Kaplan-Meier , Neoplasias Hepáticas/mortalidade , Neoplasias Hepáticas/patologia , Neoplasias Hepáticas/terapia , Transplante de Fígado , Neoplasias Pulmonares/mortalidade , Neoplasias Pulmonares/patologia , Neoplasias Pulmonares/terapia , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos , Neoplasias de Tecidos Moles/mortalidade , Neoplasias de Tecidos Moles/patologia , Neoplasias de Tecidos Moles/terapia , Resultado do Tratamento
4.
Medicine (Baltimore) ; 100(20): e26040, 2021 May 21.
Artigo em Inglês | MEDLINE | ID: mdl-34011115

RESUMO

BACKGROUND: Tumor-specific DNA methylation can potentially be a useful indicator in cancer diagnostics and monitoring. Sarcomas comprise a heterogeneous group of mesenchymal neoplasms which cause life-threatening tumors occurring throughout the body. Therefore, potential molecular detection and prognostic evaluation is very important for early diagnosis and treatment. METHODS: We performed a retrospective study analyzing DNA methylation of 261 patients with sarcoma from The Cancer Genome Atlas (TCGA) database. Cox regression analyses were conducted to identify a signature associated with the overall survival (OS) of patients with sarcoma, which was validated in a validation dataset. RESULTS: Three DNA methylation signatures were identified to be significantly associated with OS. Kaplan-Meier analysis showed that the 3-DNA methylation signature could significantly distinguish the high- and low-risk patients in both training (first two-thirds) and validation datasets (remaining one-third). Receiver operating characteristic (ROC) analysis confirmed that the 3-DNA methylation signature exhibited high sensitivity and specificity in predicting OS of patients. Also, the Kaplan-Meier analysis and the area under curve (AUC) values indicated that the 3-DNA methylation signature was independent of clinical characteristics, including age at diagnosis, sex, anatomic location, tumor residual classification, and histological subtypes. CONCLUSIONS: The current study showed that the 3-DNA methylation model could efficiently function as a novel and independent prognostic biomarker and therapeutic target for patients with sarcoma.


Assuntos
Metilação de DNA/fisiologia , Sarcoma/diagnóstico , Sarcoma/metabolismo , Neoplasias de Tecidos Moles/diagnóstico , Neoplasias de Tecidos Moles/metabolismo , Adulto , Idoso , Idoso de 80 Anos ou mais , Biomarcadores , Feminino , Humanos , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Prognóstico , Curva ROC , Estudos Retrospectivos , Sarcoma/mortalidade , Neoplasias de Tecidos Moles/mortalidade , Adulto Jovem
5.
Am J Clin Oncol ; 44(7): 361-368, 2021 07 01.
Artigo em Inglês | MEDLINE | ID: mdl-33927134

RESUMO

OBJECTIVES: Synovial sarcomas (SS) arising in distal extremities are rare and have been studied using mostly case reports and small series. We aimed to evaluate clinical presentation and survival outcomes for patients with hand or foot SS. MATERIALS AND METHODS: We conducted a retrospective review of 84 patients diagnosed with primary hand (n=20) and foot (n=64) SS between 1979 and 2019. Progression-free survival (PFS), overall survival (OS), local recurrence-free survival and metastasis-free survival were estimated using the Kaplan-Meier method and log-rank test. Cox-proportional hazards regression was used to estimate the hazard ratios. RESULTS: Of 84 patients, 63 (75%) presented with localized disease with 36 years median age at diagnosis (range: 4 to 76) and 21 (25%) with metastasis with 30 years median age at diagnosis (range: 15 to 64). Among patients presenting with localized disease, (1) 5 years-PFS, OS, local recurrence-free survival, and metastasis-free survival rates were 82%, 88%, 100%, and 86%, respectively. (2) Tumor size <3.0 cm corresponded to 95% 5 years-PFS (vs. 84% for 3.0 to 4.9 cm, 53% for ≥5.0 cm; P=0.007) and 100% 5 years-OS (vs. 77% for ≥3.0 cm; P=0.04). (3) Patients with ≥5.0 cm (vs. <3.0 cm) tumor size had 7.99 (95% confidence interval: 1.68, 37.91) times higher hazard of progression. Remarkably, patients presenting with metastasis had 50% 5 years-OS rate. Also, younger age (15 to 39 vs. 40 y and above) predicted better OS among patients presenting with localized disease (P=0.04) and with metastasis (P=0.03). CONCLUSIONS: Survival outcomes are favorable for younger patients with <3.0 cm hand or foot SS. Local control is excellent, but we observed larger tumor size to be associated with poorer outcomes. Therefore, we recommend consideration of systemic therapy for patients with ≥3.0 cm hand or foot SS.


Assuntos
Sarcoma Sinovial/mortalidade , Sarcoma Sinovial/patologia , Neoplasias de Tecidos Moles/mortalidade , Neoplasias de Tecidos Moles/patologia , Adolescente , Adulto , Idoso , Antineoplásicos/uso terapêutico , Criança , Pré-Escolar , Feminino , Pé/patologia , Mãos/patologia , Humanos , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Intervalo Livre de Progressão , Estudos Retrospectivos , Sarcoma Sinovial/tratamento farmacológico , Sarcoma Sinovial/cirurgia , Neoplasias de Tecidos Moles/tratamento farmacológico , Neoplasias de Tecidos Moles/cirurgia , Adulto Jovem
6.
Jpn J Clin Oncol ; 51(6): 927-931, 2021 May 28.
Artigo em Inglês | MEDLINE | ID: mdl-33822979

RESUMO

OBJECTIVE: Synovial sarcoma is the most common soft tissue sarcomas among childhood and adolescents, accounting for 8-10% of all soft tissue sarcoma. Synovial sarcoma is considered a relatively chemosensitive tumor compared with other soft tissue sarcomas. However, the role of perioperative chemotherapy in synovial sarcoma remains controversial. The purpose of this systematic review is to evaluate the role of perioperative chemotherapy in childhood and adolescent patients with synovial sarcoma. METHODS: We evaluated studies published between 1 January 1990 and 31 December 2017. The following databases were searched: MEDLINE, Cochrane database (via PubMed) and Ichushi (in Japanese). RESULTS: The search yielded 216 articles in English and Japanese. After the initial screening, based on the title and abstract, 160 articles were excluded. As a second screening, we then assessed the full text of the remaining 56 articles for eligibility. Finally, 10 articles were included in the systematic review. Surgical resection with R0 margin alone was recommended because of the excellent results of two prospective studies. Meta-analysis was performed using data from two retrospective studies of 261 patients. Perioperative chemotherapy did not have a significant effect on survival and event-free survival. CONCLUSIONS: We weakly do not recommend perioperative chemotherapy in patients with non-metastatic synovial sarcoma ≤ 5 cm when R0 resection is acquired. There was no consensus concerning the role of perioperative chemotherapy in patients with synovial sarcoma > 5 cm or those with ≤5 cm who undergo R1 or R2 resection.


Assuntos
Sarcoma Sinovial/tratamento farmacológico , Sarcoma/tratamento farmacológico , Neoplasias de Tecidos Moles/tratamento farmacológico , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Estudos Prospectivos , Estudos Retrospectivos , Sarcoma/mortalidade , Sarcoma Sinovial/mortalidade , Neoplasias de Tecidos Moles/mortalidade
7.
Int J Surg Oncol ; 2021: 8871557, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33833875

RESUMO

Soft-tissue sarcomas are malignant tumors that require good management within specialized centers. Our study aims to assess the benefit of handling these kinds of tumors using the Multidisciplinary Meeting (MDM) approach. The current paper details this approach through a prospective study that has lasted for 42 months in the HASSAN II University Hospital Center, Fez, Morocco. During this research work, 116 cases were selected with an average age of 53 years. In 95.7% of the cases, it was found that the lower limb was the most frequent tumor type (78.4%). Also, ninety-two (92) patients (79.3%) have had a prior biopsy. Ninety-nine (99) patients (85.3%) have received a magnetic resonance imaging scan (MRI) before surgery. Sixty-three (63) patients were operated on, including R0 resection used for 37 patients, R1 used for 21 patients, and R2 used for five patients. As a result, liposarcomas were the most frequent type (30.1%), followed by synovial sarcomas (14.6%), leiomyosarcomas (9.5%), ewing sarcoma (8.6), and undifferentiated pleomorphic sarcomas (7.7%). In addition, neoadjuvant chemotherapy was used for 36 patients. The other 22 patients received adjuvant chemotherapy and/or radiotherapy. The overall survival rate was 60.56 months, which proves a significant improvement, thanks to the multidisciplinary meeting approach. Conclusion. The conducted investigation has shown that using MDM for managing soft-tissue sarcomas of extremities improves the patients' survival rate. Moreover, results have proven MDM might allow optimal treatment regarding less local recurrence and metastasis.


Assuntos
Comunicação Interdisciplinar , Equipe de Assistência ao Paciente , Sarcoma/diagnóstico , Sarcoma/terapia , Neoplasias de Tecidos Moles/diagnóstico , Neoplasias de Tecidos Moles/terapia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Extremidades/patologia , Extremidades/cirurgia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Prospectivos , Sarcoma/mortalidade , Sarcoma/patologia , Neoplasias de Tecidos Moles/mortalidade , Neoplasias de Tecidos Moles/patologia , Adulto Jovem
8.
Anticancer Res ; 41(4): 1927-1935, 2021 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-33813398

RESUMO

BACKGROUND/AIM: To identify prognostic imaging biomarkers from staging chest computed tomography (CT) in patients with sarcomas. PATIENTS AND METHODS: CT scans for baseline staging, and surveillance 1-year CT scans in patients newly diagnosed with sarcoma were evaluated. Pectoralis muscle area (PMA), pectoralis muscle index (PMI) and pectoralis CT attenuation density (PMT) were measured. Cox proportional-hazard models were used to determine the association with progression-free survival (PFS) and overall survival (OS). RESULTS: There were 147 patients (53.1% male) who were followed for a median 1,414 days (range=219-4851 days). Approximately 47.6% (70/147) of patients progressed and 29.9% (44/147) died. Multivariable Cox-proportional hazards models adjusting for gender, tumor grade and chemotherapy treatment showed that a higher baseline PMT and baseline PMI were associated with increased OS. CONCLUSION: Higher baseline PMI and PMT are associated with increased overall survival in patients with sarcoma.


Assuntos
Neoplasias Ósseas/diagnóstico , Músculos Peitorais/diagnóstico por imagem , Sarcoma/diagnóstico , Neoplasias de Tecidos Moles/diagnóstico , Tomografia Computadorizada por Raios X/métodos , Adulto , Idoso , Anatomia Transversal/métodos , Neoplasias Ósseas/tratamento farmacológico , Neoplasias Ósseas/mortalidade , Neoplasias Ósseas/patologia , Estudos de Coortes , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Músculos Peitorais/patologia , Prognóstico , Radiografia Torácica/métodos , Estudos Retrospectivos , Sarcoma/tratamento farmacológico , Sarcoma/mortalidade , Sarcoma/patologia , Neoplasias de Tecidos Moles/tratamento farmacológico , Neoplasias de Tecidos Moles/mortalidade , Neoplasias de Tecidos Moles/patologia , Análise de Sobrevida
9.
Eur J Cancer ; 147: 164-169, 2021 04.
Artigo em Inglês | MEDLINE | ID: mdl-33684876

RESUMO

A group of patients with adult-type soft tissue sarcoma is at high risk of local recurrence and distant metastases. Age, tumour site, histological subtype, tumour size and grade have been identified as the most important independent adverse prognostic factors. Macroscopically complete tumour resection is considered as the mainstay of treatment with the addition of preoperative or postoperative radiotherapy for extremity or trunk localisation. Retroperitoneal localisation requires compartmental resection and is associated with a worse prognosis. Here, radiotherapy is of no proven value. Perioperative chemotherapy is considered to treat micrometastatic disease not detectable at the time of diagnosis. The neoadjuvant application gives the risk of distant metastasis the greatest importance as therapy is carried out at the earliest possible time, whereas adjuvant chemotherapy is delayed by surgery and the necessary wound healing. With reported response rates up to 30%, both the operability may be improved and the risk of intraoperative tumour cell dissemination may be reduced, resulting also in reduced local relapse rates. However, the potential risk of early tumour progression may counteract this benefit. Optimised strategies with multimodality approaches including chemotherapy, regional hyperthermia (RHT) and immunotherapeutic agents have been shown to improve survival in high-risk patients. Here, we focus on the data from available randomised studies investigating the use of perioperative chemotherapy in patients with high-risk adult-type soft tissue sarcoma, including the use of RHT for local enhancement of chemotherapy effect and immune induction.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Hipertermia Induzida , Terapia Neoadjuvante , Sarcoma/terapia , Neoplasias de Tecidos Moles/terapia , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Quimioterapia Adjuvante , Ensaios Clínicos Fase III como Assunto , Progressão da Doença , Humanos , Hipertermia Induzida/efeitos adversos , Terapia Neoadjuvante/efeitos adversos , Ensaios Clínicos Controlados Aleatórios como Assunto , Sarcoma/imunologia , Sarcoma/mortalidade , Sarcoma/patologia , Neoplasias de Tecidos Moles/imunologia , Neoplasias de Tecidos Moles/mortalidade , Neoplasias de Tecidos Moles/patologia , Fatores de Tempo , Resultado do Tratamento
10.
J Surg Oncol ; 123(8): 1792-1800, 2021 May.
Artigo em Inglês | MEDLINE | ID: mdl-33751586

RESUMO

BACKGROUND AND OBJECTIVES: Synovial, clear cell, angiosarcoma, rhabdomyosarcoma, and epithelioid (SCARE) soft tissue sarcoma are at risk for nodal involvement, although the nodal positivity rates and impact on prognostication in clinically node negative patients are not well described. METHODS: Patients with extremity SCARE sarcoma without clinical nodal involvement undergoing surgical resection in the National Cancer Database (2004-2017) were included. Logistic regression was used to evaluate the likelihood of nodal surgery and nodal positivity. Kaplan-Meier method and Cox regression were used to assess associations of nodal status to overall survival. RESULTS: We included 4158 patients, and 669 patients (16%) underwent regional lymph node surgery (RLNS). On multivariable logistic analysis, patients with epithelioid (odds ratio [OR]: 3.77; p < .001) and clear cell (OR: 6.38; p < .001) were most likely to undergo RLNS. Forty-five patients (7%) had positive nodes. Clear cell sarcoma (14%) and angiosarcoma (13%) had the highest rates of nodal positivity. Patients with positive nodes had reduced 5-year overall survival, and the stratification was largest in clear cell and angiosarcoma. CONCLUSION: Discordance exists between selection for pathologic nodal evaluation and factors associated with nodal positivity. Clinically node negative patients with clear cell and angiosarcoma should be considered for pathologic nodal evaluation.


Assuntos
Extremidades , Sarcoma/patologia , Neoplasias de Tecidos Moles/patologia , Adolescente , Adulto , Idoso , Bases de Dados Factuais , Humanos , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Estudos Retrospectivos , Sarcoma/mortalidade , Sarcoma/terapia , Neoplasias de Tecidos Moles/mortalidade , Neoplasias de Tecidos Moles/terapia , Taxa de Sobrevida , Adulto Jovem
12.
Jpn J Clin Oncol ; 51(6): 918-926, 2021 May 28.
Artigo em Inglês | MEDLINE | ID: mdl-33774673

RESUMO

BACKGROUND: Approximately 10% of the patients with soft tissue sarcoma show metastasis at initial diagnosis, and hence, poorer prognosis. However, the prognostic factors and whether definitive surgery for the primary lesion improves overall survival, especially when complete resection of metastasis is difficult, remain unclear. METHODS: This retrospective analysis was based on the Bone and Soft Tissue Tumor Registry in Japan. Patients with soft tissue sarcoma having metastasis at diagnosis were enrolled, excluding those with Ewing's sarcoma, rhabdomyosarcoma and several other sarcomas with unique behavior and treatment strategies. Overall survival was estimated using the Kaplan-Meier method and compared among the common histologic subtypes. Multivariate analysis with the Cox regression model was used to identify the prognostic factors. RESULTS: In total, 1184 patients were included, with a median follow-up duration of 10 months (range: 1-83). The median overall survival was 21 months (95% confidence interval: 18.2-23.8). The multivariate analyses indicated that tumor size, grade and histologic subtypes significantly correlated with overall survival. Moreover, surgery for the primary lesion, in addition to surgery for metastases and chemotherapy, showed significant association with better survival. CONCLUSIONS: The prognostic factors in patients with metastatic soft tissue sarcoma at diagnosis are generally similar to those in patients with localized disease. The overall survival in patients differed significantly according to histologic subtype. Surgical resection of primary lesions, especially those with a wide margin, may be an independent prognostic factor. Further studies are needed identify which subgroup of patients would benefit the most from primary lesion surgery.


Assuntos
Sarcoma/diagnóstico , Sarcoma/cirurgia , Neoplasias de Tecidos Moles/diagnóstico , Neoplasias de Tecidos Moles/cirurgia , Adolescente , Adulto , Idoso , Estudos de Coortes , Humanos , Japão/epidemiologia , Masculino , Margens de Excisão , Pessoa de Meia-Idade , Metástase Neoplásica , Prognóstico , Estudos Retrospectivos , Sarcoma/mortalidade , Sarcoma/patologia , Neoplasias de Tecidos Moles/mortalidade , Neoplasias de Tecidos Moles/patologia , Fatores de Tempo
13.
JAMA Netw Open ; 4(3): e210845, 2021 03 01.
Artigo em Inglês | MEDLINE | ID: mdl-33666664

RESUMO

Importance: Host-related immune factors have been implicated in the development and progression of diverse malignant neoplasms. Identifying associations between immunologic laboratory parameters and overall survival may inform novel prognostic biomarkers and mechanisms of antitumor immunity in localized bone and soft tissue sarcoma. Objective: To assess whether lymphopenia at diagnosis is associated with overall survival among patients with localized bone and soft tissue sarcoma. Design, Setting, and Participants: This retrospective cohort study analyzed patients from the Stanford Cancer Institute with localized bone and soft tissue sarcoma between September 1, 1998, and November 1, 2018. Patients were included if laboratory values were available within 60 days of diagnosis and, if applicable, prior to the initiation of chemotherapy and/or radiotherapy. Statistical analysis was performed from January 1, 2019, to November 1, 2020. Exposures: Absolute lymphocyte count within 60 days of diagnosis and antimicrobial exposure, defined by the number of antimicrobial agent prescriptions and the cumulative duration of antimicrobial administration within 60 days of diagnosis. Main Outcomes and Measures: The association between minimum absolute lymphocyte count at diagnosis and 5-year overall survival probability was characterized with the Kaplan-Meier method and multivariate Cox proportional hazards regression models. Multivariable logistic regressions were fitted to evaluate whether patients with lymphopenia were at greater risk of increased antimicrobial exposure. Results: Among 634 patients, the median age at diagnosis was 53.7 years (interquartile range, 37.5-66.8 years), and 290 patients (45.7%) were women, with a 5-year survival probability of 67.9%. There was a significant inverse association between lymphopenia at diagnosis and overall survival (hazard ratio [HR], 1.82; 95% CI, 1.39-1.40), resulting in a 13.5% 5-year survival probability difference compared with patients who did not have lymphopenia at diagnosis (60.2% vs 73.7% for those who never had lymphopenia). In addition, poorer survival was observed with higher-grade lymphopenia (grades 3 and 4: HR, 2.44; 95% CI, 1.68-3.55; grades 1 and 2: HR, 1.60; 95% CI, 1.18-2.18). In an exploratory analysis, patients with increased antibiotic exposure were more likely to have lymphopenia (odds ratio, 1.96; 95% CI, 1.26-3.07 for total number of antimicrobial agents; odds ratio, 1.70; 95% CI, 1.10-2.57 for antimicrobial duration) than antimicrobial-naive patients. Conclusions and Relevance: This study suggests that an abnormally low absolute lymphocyte count at diagnosis is associated with higher mortality among patients with localized bone and soft tissue sarcoma; therefore, lymphopenia may serve as a reliable prognostic biomarker. Potential mechanisms associated with host immunity and overall survival include a suppressed antitumor response and increased infectious complications, which merit future investigation.


Assuntos
Neoplasias Ósseas/sangue , Neoplasias Ósseas/complicações , Linfopenia/etiologia , Sarcoma/sangue , Sarcoma/complicações , Neoplasias de Tecidos Moles/sangue , Neoplasias de Tecidos Moles/complicações , Adulto , Idoso , Neoplasias Ósseas/diagnóstico , Neoplasias Ósseas/mortalidade , Estudos de Coortes , Feminino , Humanos , Contagem de Linfócitos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Sarcoma/diagnóstico , Sarcoma/mortalidade , Neoplasias de Tecidos Moles/diagnóstico , Neoplasias de Tecidos Moles/mortalidade , Taxa de Sobrevida
14.
Bone Joint J ; 103-B(3): 569-577, 2021 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-33641420

RESUMO

AIMS: Urgent referral to a specialist centre for patients with a soft-tissue sarcoma (STS) has been recommended by the National Institute for Health and Care Excellence (NICE) in the UK since 2006. However, the impact of this recommendation on the prognosis for these patients remains unclear. We aimed to determine the impact of the NICE guidelines on the disease-specific survival (DSS) of patients with an STS. METHODS: A total of 2,427 patients with an STS referred to a supraregional centre in the ten-year periods before (n = 1,386) and after (n = 1,041) the issue of the NICE guidelines were evaluated. RESULTS: The mean size of the tumour was significantly smaller at the time of diagnosis (10.3 cm (SD 6.5) vs 9.1 cm (SD 6.2); p < 0.001) and the number of patients who had undergone an inadvertent excision significantly decreased (28% (n = 389) vs 20% (n = 204); p < 0.001) following the introduction of the NICE guidelines. The five-year DSS was 63% in the pre-NICE and 71% in post-NICE groups (p < 0.001). The improved survival was more significant for those with a high-grade tumour (pre-NICE, 48%; post-NICE, 68%; p < 0.001). In those with a high-grade tumour, the mean size of the tumour (11.6 cm (SD 6.2) vs 9.6 cm (SD 5.8); p < 0.001) and the number of patients with metastasis at the time of diagnosis (15% (n = 124 vs 10% (n = 80); p = 0.007) significantly decreased in the post-NICE group. CONCLUSION: An improvement in survival was seen after the introduction of the NICE guidelines, especially in patients with a high-grade STS. More patients were referred at an earlier stage, indicating a clearer pathway after the issue of national policy for the management of STSs in the UK. Cite this article: Bone Joint J 2021;103-B(3):569-577.


Assuntos
Guias de Prática Clínica como Assunto , Encaminhamento e Consulta/estatística & dados numéricos , Sarcoma/mortalidade , Sarcoma/cirurgia , Neoplasias de Tecidos Moles/mortalidade , Neoplasias de Tecidos Moles/cirurgia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Gradação de Tumores , Prognóstico , Estudos Retrospectivos , Sarcoma/patologia , Neoplasias de Tecidos Moles/patologia , Taxa de Sobrevida , Reino Unido/epidemiologia
15.
Medicine (Baltimore) ; 100(11): e25008, 2021 Mar 19.
Artigo em Inglês | MEDLINE | ID: mdl-33725976

RESUMO

ABSTRACT: The tumor microenvironment (TME) plays an important role in the occurrence and development of soft tissue sarcoma (STS). A number of studies have shown that to inhibit tumor growth, the TME can be remodeled into an environment unsuitable for tumor proliferation. However, a lack of understanding exists regarding the dynamic regulation of TME.In this study, we used CIBERSORT and ESTIMATE calculation methods from the Cancer Genome Atlas (TCGA) database to calculate the proportion of tumor infiltrating immune cells (TICs) and the number of immune and stromal components in 263 STS samples. Differential expression genes (DEGs) shared by Immune Score and Stromal Score were obtained via difference analysis. Univariate Cox regression analysis and construction of protein-protein interaction (PPI) networks were applied to the DEGs.Through intersection analysis of univariate COX and PPI, PLCG2 was determined as the indicator. Further analysis showed that PLCG2 expression was positively correlated with the survival of STS patients. Gene set enrichment analysis (GSEA) showed that genes in the highly expressed PLCG2 group were enriched in immune-related activities. In the low-expression PLCG2 group, genes were enriched in the E2F, G2M, and MYC pathways. Difference analysis and correlation analysis showed that CD8+ T cells, gamma delta T cells, monocytes, and M1 macrophages were positively correlated with PLCG2 expression, indicating that PLCG2 may represent the immune status of TME.Therefore, the level of PLCG2 may aid in determining the prognosis of STS patients, especially the status of TME. These data provide additional insights into the remodeling of TME.


Assuntos
Fosfolipase C gama/metabolismo , Sarcoma/genética , Neoplasias de Tecidos Moles/genética , Microambiente Tumoral/genética , Biomarcadores Tumorais , Bases de Dados Genéticas , Feminino , Humanos , Estimativa de Kaplan-Meier , Linfócitos do Interstício Tumoral/metabolismo , Ativação de Macrófagos/genética , Masculino , Modelos de Riscos Proporcionais , Mapas de Interação de Proteínas , Sarcoma/mortalidade , Neoplasias de Tecidos Moles/mortalidade , Células Estromais/metabolismo , Transcriptoma
16.
Medicine (Baltimore) ; 100(5): e23787, 2021 Feb 05.
Artigo em Inglês | MEDLINE | ID: mdl-33592836

RESUMO

ABSTRACT: Soft tissue sarcomas (STSs) are heterogeneous at the clinical with a variable tendency of aggressive behavior. In this study, we constructed a specific DNA methylation-based classification to identify the distinct prognosis-subtypes of STSs based on the DNA methylation spectrum from the TCGA database. Eventually, samples were clustered into 4 subgroups, and their survival curves were distinct from each other. Meanwhile, the samples in each subgroup reflected differentially in several clinical features. Gene Ontology (GO) and the Kyoto Encyclopedia of Genes and Genomes (KEGG) analysis was also conducted on the genes of the corresponding promoter regions of the above-described specific methylation sites, revealing that these genes were mainly concentrated in certain cancer-associated biological functions and pathways. In addition, we calculated the differences among clustered methylation sites and performed the specific methylation sites with LASSO algorithm. The selection operator algorithm was employed to derive a risk signature model, and a prognostic signature based on these methylation sites performed well for risk stratification in STSs patients. At last, a nomogram consisted of clinical features and risk score was developed for the survival prediction. This study declares that DNA methylation-based STSs subtype classification is highly relevant for future development of personalized therapy as it identifies the prediction value of patient prognosis.


Assuntos
Metilação de DNA/genética , Nomogramas , Sarcoma/classificação , Neoplasias de Tecidos Moles/classificação , Algoritmos , Biomarcadores Tumorais/genética , Bases de Dados Genéticas , Regulação Neoplásica da Expressão Gênica/genética , Ontologia Genética , Humanos , Valor Preditivo dos Testes , Prognóstico , Modelos de Riscos Proporcionais , Medição de Risco , Sarcoma/genética , Sarcoma/mortalidade , Neoplasias de Tecidos Moles/genética , Neoplasias de Tecidos Moles/mortalidade
17.
J Surg Oncol ; 123(5): 1274-1283, 2021 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-33524203

RESUMO

BACKGROUNDS AND OBJECTIVES: This investigation sought to describe the outcomes of primary leiomyosarcoma of bone (PLB) compared to soft tissue leiomyosarcoma (SLMS). METHODS: This was a review of the Surveillance, Epidemiology, and End Results database from 1975 to 2016. Kaplan-Meier methods were used to estimate disease-specific survival (DSS), and a Cox regression model was used to identify prognostic factors. RESULTS: Of the 7502 identifiable cases, 1% (n = 74) were PLB and 99% (n = 7428) were SLMS. Survival was the same between PLB and SLMS (p = .209). On multivariable analysis for high-grade SLMS, radiation (neoadjuvant: hazard ratio [HR], 0.56; 95% confidence interval [CI], 0.4-0.8; p = .003; adjuvant: HR, 0.75; 95% CI, 0.6-0.9; p = .008) and surgery (procedure specific) improved DSS. For PLB, wide resection/limb salvage (HR, 0.40; 95% CI, 0.3-0.5; p = .018) and amputation (HR, 0.69; 95% CI, 0.5-0.9; p < .001) were positive prognostic factors. Neither radiation nor chemotherapy were prognostic factors for survival in PLB. CONCLUSIONS: For SLMS, radiation portends a survival advantage. For PLB, however, neither chemotherapy nor radiation were significant prognostic factors, which suggests the optimal treatment for PLB, similar to other primary soft tissue sarcomas originating in bone, remains an unmet medical need.


Assuntos
Neoplasias Ósseas/mortalidade , Leiomiossarcoma/mortalidade , Neoplasias de Tecidos Moles/mortalidade , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Neoplasias Ósseas/patologia , Neoplasias Ósseas/terapia , Criança , Pré-Escolar , Estudos de Coortes , Terapia Combinada , Feminino , Seguimentos , Humanos , Lactente , Recém-Nascido , Leiomiossarcoma/patologia , Leiomiossarcoma/terapia , Pessoa de Meia-Idade , Prognóstico , Neoplasias de Tecidos Moles/patologia , Neoplasias de Tecidos Moles/terapia , Taxa de Sobrevida , Adulto Jovem
18.
Medicine (Baltimore) ; 100(3): e24124, 2021 Jan 22.
Artigo em Inglês | MEDLINE | ID: mdl-33546021

RESUMO

BACKGROUND: The impact of surgical margin status on the survival of patients with extremity soft tissue sarcoma (STS) remains to be clearly defined. The evidence regarding the impact of surgical margins on survival is limited by retrospective single-institution cohort studies. We conducted a systematic review and meta-analysis to examine the impact of surgical margin status on patient survival in extremity STS. METHODS: A literature search in the PubMed, EMBASE, and Cochrane Controlled Trials Register electronic databases, and a manual search of reference lists of original studies was performed. The following text words and/or Medical Subject Heading terms were searched: (neoplasm) or/and (sarcoma) and/or (connective tissue) and/or (soft tissue) and/or (extremity) and/or (extremity) and/or (surgical margin). RESULTS: Six selected studies that reported a total of 2917 cases of extremity STS were published between 1994 and 2013. All the eligible studies were observational cohort studies, and the sample size ranged from 95 to 1261 patients. A meta-analysis of 6 studies showed that a positive surgical margin predicted poor 5-year OS in a random-effects model (summary hazard ratio, 1.56; 95% confidence interval, 1.12-2.17). Moderate heterogeneity was observed among the studies (P < .075; heterogeneity, 45.6%). CONCLUSIONS: This meta-analysis supports the hypothesis that adequate surgical margins are associated with improved survival in extremity STS.


Assuntos
Margens de Excisão , Sarcoma/mortalidade , Sarcoma/cirurgia , Neoplasias de Tecidos Moles/mortalidade , Neoplasias de Tecidos Moles/cirurgia , Extremidades/cirurgia , Humanos
19.
Anticancer Res ; 41(1): 359-368, 2021 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-33419832

RESUMO

BACKGROUND/AIM: Surgical resection for soft tissue sarcomas (STSs) is the gold standard for a curative oncologic therapy in combination with neoadjuvant or adjuvant radiation therapy (NRT/ART). The aim of this study was to determine prognostic factors influencing the survival of patients with STS undergoing NRT or ART considering various parameters in a retrospective, single-centre analysis over 15 years. PATIENTS AND METHODS: We included 119 patients (male 59) and the median follow-up period was 69 months (4-197). The patients received NRT (n=64) or ART (n=55). We recorded the histopathologic subtype of STS, tumour grade, localization, tumour margins, complications, survival, local recurrence, and metastases. Survival analysis was performed using the Kaplan-Meier method. RESULTS: The overall survival rate was 68.9% at 5 years. The localization (epifascial/subfascial), resection margin and type of radiation therapy (NRT/ART) had no significant impact on survival. Tumour grade, tumour size, local recurrence and metastases were significantly correlated with patient survival (p<0.05). Local recurrence was significantly higher in patients with ART (p=0.044). CONCLUSION: Tumour grade and tumour size were independently associated with disease-specific survival, and patients with local recurrence and metastases had lower survival rates.


Assuntos
Sarcoma/mortalidade , Sarcoma/cirurgia , Neoplasias de Tecidos Moles/mortalidade , Neoplasias de Tecidos Moles/cirurgia , Adulto , Idoso , Terapia Combinada , Feminino , Humanos , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Terapia Neoadjuvante/efeitos adversos , Terapia Neoadjuvante/métodos , Gradação de Tumores , Estadiamento de Neoplasias , Prognóstico , Radioterapia Adjuvante/efeitos adversos , Radioterapia Adjuvante/métodos , Retratamento , Estudos Retrospectivos , Sarcoma/diagnóstico , Neoplasias de Tecidos Moles/diagnóstico , Resultado do Tratamento
20.
BMC Surg ; 21(1): 66, 2021 Jan 30.
Artigo em Inglês | MEDLINE | ID: mdl-33516222

RESUMO

BACKGROUND: Perianal/perineal rhabdomyosarcomas (PRMS) are easily misdiagnosed soft tissue tumours with a poor prognosis. This study was designed to analyze the clinical, diagnostic, pathological and prognostic features of PRMS, and to explore currently available therapeutic modalities. METHODS: Clinical data of PRMS patients admitted to the Sixth Affiliated Hospital and the Cancer Center of Sun Yat-sen University and from related Chinese literature published from 1987 to 2018 were collected and analyzed. The Chi-square test was used to evaluate the differences between each group. The Kaplan-Meier methods were applied to estimate and compare survival rates. RESULTS: A total of 35 patients were included in this study; 20 identified within related Chinese literatures and 15 from our center admitted during the period of 1997-2019. Out of these cases, 34 presented with perianal masses and the remaining one manifested as an inguinal mass. Moreover, 20 patients complained of pain and 16 of them were misdiagnosed as perianal abscesses, in which the presence of pain contributed to the misdiagnosis (p < 0.05). The average time interval between symptom onset and pathological diagnosis was 3.1 months. Next, 13 cases were classified into IRS group III/IV and 20 cases into stages 3/4. Additionally, 14 and 9 cases received the pathological diagnoses of embryonal rhabdomyosarcoma and alveolar rhabdomyosarcoma, respectively. Regarding the patients' survival rates, five patients survived for more than 2 years, and three of them survived for more than 5 years. The overall 2 years and 5 years survival rates were 32% and 24%, respectively. The symptom of pain and misdiagnosis both contributed to the poor prognosis in these patients (p < 0.05). MRI showed that the PRMS were closely related to external anal sphincter in 10 cases. CONCLUSION: PRMS are easily misdiagnosed lesions, which often leads to an unfavourable outcome in affected patients. Patients with painful perianal masses should be evaluated to exclude PRMS. MRI revealed that PRMS are closely related to the external anal sphincter. Multidisciplinary management is recommended in the treatment of PRMS.


Assuntos
Neoplasias do Ânus/patologia , Rabdomiossarcoma/patologia , Neoplasias de Tecidos Moles/patologia , Adolescente , Adulto , Canal Anal/diagnóstico por imagem , Neoplasias do Ânus/mortalidade , Neoplasias do Ânus/cirurgia , Criança , Pré-Escolar , Diagnóstico Diferencial , Erros de Diagnóstico , Humanos , Biópsia Guiada por Imagem , Lactente , Estimativa de Kaplan-Meier , Imageamento por Ressonância Magnética , Pessoa de Meia-Idade , Períneo/diagnóstico por imagem , Prognóstico , Reto/diagnóstico por imagem , Rabdomiossarcoma/mortalidade , Rabdomiossarcoma/cirurgia , Neoplasias de Tecidos Moles/mortalidade , Neoplasias de Tecidos Moles/cirurgia , Taxa de Sobrevida , Resultado do Tratamento , Ultrassonografia , Adulto Jovem
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