liposarcoma

脂肪肉瘤
  • 文章类型: Journal Article
    脂肪肉瘤是一种恶性肿瘤,起源于脂肪组织,可发生在身体的任何部位。目前对于不同解剖位置的脂肪肉瘤之间的预后是否存在显著差异尚无明确的结论。特别是腹膜后脂肪肉瘤(RLPS)和非腹膜后脂肪肉瘤(NRLPS)。这项研究的目的是揭示这两个位置的脂肪肉瘤之间的预后是否存在差异,并进一步探讨这些差异背后的根本原因。
    我们通过分析来自监测的数据,对影响脂肪肉瘤患者预后的因素进行了深入调查,流行病学,和最终结果计划(SEER)数据库。然后,我们使用倾向评分匹配(PSM)来平衡这些预后因素,以比较RLPS和NRLPS之间的生存率.此外,通过分析TCGA和日本基因型表型档案(JGA)的转录组和全外显子组数据,我们鉴定了具有显著表达差异的基因,并探索了免疫微环境的变化。
    通过对SEER数据库中RLPS和NRLPS患者的分析,我们观察到两组之间的显着预后差异,RLPS的预后较差(p<0.001)。即使在通过PSM调整了混杂因素之后,这些生存率差异仍然很大,RLPS仍显示较差的预后(p=0.017)。此外,我们对转录组数据的分析导致了467个差异表达基因的鉴定.此外,我们注意到两组之间在免疫微环境和整个外显子组测序数据方面存在显著差异.
    RLPS和NRLPS患者之间存在显着差异。因此,从临床研究到治疗策略,RLPS和NRLPS应被视为两种不同类型的肿瘤,他们的研究和治疗需要差异化的方法。
    UNASSIGNED: Liposarcoma is a malignant tumor that originates from adipose tissue and can occur in any part of the body. There is currently no clear conclusion on whether there are significant differences in prognosis between liposarcoma at different anatomical locations, especially retroperitoneal liposarcoma (RLPS) and non retroperitoneal liposarcoma (NRLPS). The aim of this study is to reveal whether there are differences in prognosis between these two locations of liposarcoma, and further explore the fundamental reasons behind these differences.
    UNASSIGNED: We conducted an in-depth investigation into the factors affecting the prognosis of patients with liposarcoma by analyzing the data from the Surveillance, Epidemiology, and End Results Program (SEER) database. Then, we used propensity score matching (PSM) to balance these prognostic factors for comparative analysis of survival between RLPS and NRLPS. In addition, by analyzing transcriptome and whole exome data from TCGA and the Japan Genotypic Phenotype Archive (JGA), we identified genes with significant expression differences and explored changes in the immune microenvironment.
    UNASSIGNED: Through analysis of RLPS and NRLPS patients in the SEER database, we observed significant prognostic differences between the two groups, with RLPS exhibiting worse prognosis (p < 0.001). Even after adjusting for confounding factors through PSM, these survival rate differences remained significant, with RLPS still showing worse prognosis (p = 0.017). Furthermore, our analysis of transcriptomic data led to the identification of 467 differentially expressed genes. Additionally, we noted significant differences in the immune microenvironment and whole exome sequencing data between the two groups.
    UNASSIGNED: There are significant differences between patients with RLPS and NRLPS. Therefore, from clinical research to treatment strategies, RLPS and NRLPS should be considered as two distinct types of tumors, necessitating differentiated approaches for their study and treatment.
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  • 文章类型: Case Reports
    怀孕期间的腹膜后脂肪肉瘤是罕见的,并提出了重大的诊断挑战。即使是经验丰富的专家。我们提供了一名27岁女性患者的病例报告,怀孕15周,他因巨大的腹膜后脂肪肉瘤入院。患者接受了肿瘤的手术切除。术后病理证实诊断为高分化脂肪肉瘤。虽然怀孕期间的脂肪肉瘤是罕见的和具有挑战性的诊断,CT或MRI在其检测中起着至关重要的作用。复发率取决于病理阶段,组织学分级,以及切除肿瘤的能力.
    Retroperitoneal liposarcoma during pregnancy is rare and poses significant diagnostic challenges, even for experienced specialists. We present a case report of a 27-year-old female patient, 15 weeks pregnant, who was admitted to the hospital due to a massive retroperitoneal liposarcoma. The patient underwent surgical resection of the tumor. Postoperative pathology confirmed a diagnosis of well-differentiated liposarcoma. Although liposarcoma during pregnancy is rare and challenging to diagnose, CT or MRI plays a crucial role in its detection. The recurrence rate depends on the pathological stage, histological grade, and ability to resect the tumor.
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  • 文章类型: Case Reports
    胰腺的脂肪性假性肥大(LPH)是一种罕见的疾病,其中胰腺实质被成熟的脂肪组织取代。这是一种特发性疾病,其诊断基于组织病理学分析。在这里,我们报道了一例50岁的男性患者,该患者的胰头有脂肪性肿块,在计算机断层扫描中进行了肾脏肿瘤的仔细检查。我们怀疑是脂肪肉瘤,并进行了剖腹手术。然而,组织学分析显示LPH。LPH的一些影像学发现可以进行无创诊断并有助于其临床方法。
    Lipomatous pseudohypertrophy of the pancreas (LPH) is a rare disease in which the pancreatic parenchyma is replaced with mature adipose tissue. It is an idiopathic condition whose diagnosis is made based on histopathological analyses. Herein, we report the case of a 50-year-old male patient with a lipomatous mass in the head of the pancreas on computed tomography for close examination of a renal tumor. We suspected liposarcoma, and laparotomy was performed. However, histological analyses revealed LPH. Several imaging findings of LPH can enable a noninvasive diagnosis and help its clinical approach.
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  • 文章类型: Journal Article
    背景:作为一种常见的软组织肉瘤,脂肪肉瘤(LPS)是一种来源于脂肪组织的异质性恶性肿瘤。由于转移和复发的风险很高,LPS的预后仍然不利。提高临床治疗水平,稳健的风险预测模型对于评估LPS患者的预后至关重要.
    方法:通过对来自GEO数据集的数据的综合分析,获得差异表达基因(DEGs)。随后采用单变量和LassoCox回归来揭示与远处无复发生存(DRFS)相关的DEGs并开发预后基因标签。通过Kaplan-Meier生存率和ROC曲线进行评估。进行GSEA和免疫浸润分析以阐明该模型在LPS进展中的分子机制和免疫相关性。此外,相关分析用于破译该模型对LPS的治疗意义.
    结果:开发了一种六基因标签来预测LPS患者的DRFS,并在更具侵略性的LPS亚型中显示出更高的精度性能。然后,基于该风险模型,进一步建立了临床应用的列线图.通过GSEA,高危人群的细胞周期相关通路显著富集.在LPS微环境中,中性粒细胞,记忆B细胞和静息肥大细胞在高危和低危患者的细胞丰度上表现出显著差异.此外,该模型与治疗靶点显著相关.
    结论:建立了预后性的六基因标签,并与细胞周期通路和治疗靶基因显著相关,这可能为LPS进展的风险评估和LPS患者改善预后的治疗策略提供新的见解。
    BACKGROUND: As a common soft tissue sarcoma, liposarcoma (LPS) is a heterogeneous malignant tumor derived from adipose tissue. Due to the high risk of metastasis and recurrence, the prognosis of LPS remains unfavorable. To improve clinical treatment, a robust risk prediction model is essential to evaluate the prognosis of LPS patients.
    METHODS: By comprehensive analysis of data derived from GEO datasets, differentially expressed genes (DEGs) were obtained. Univariate and Lasso Cox regressions were subsequently employed to reveal distant recurrence-free survival (DRFS)-associated DEGs and develop a prognostic gene signature, which was assessed by Kaplan-Meier survival and ROC curve. GSEA and immune infiltration analyses were conducted to illuminate molecular mechanisms and immune correlations of this model in LPS progression. Furthermore, a correlation analysis was involved to decipher the therapeutic significance of this model for LPS.
    RESULTS: A six-gene signature was developed to predict DRFS of LPS patients and showed higher precision performance in more aggressive LPS subtypes. Then, a nomogram was further established for clinical application based on this risk model. Via GSEA, the high-risk group was significantly enriched in cell cycle-related pathways. In the LPS microenvironment, neutrophils, memory B cells and resting mast cells exhibited significant differences in cell abundance between high-risk and low-risk patients. Moreover, this model was significantly correlated with therapeutic targets.
    CONCLUSIONS: A prognostic six-gene signature was developed and significantly associated with cell cycle pathways and therapeutic target genes, which could provide new insights into risk assessment of LPS progression and therapeutic strategies for LPS patients to improve their prognosis.
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  • 文章类型: Case Reports
    源自软组织的肿瘤并不常见,在这些肿瘤中,脂肪肉瘤是最常见的。这些肿瘤长时间无症状,只有当它们达到一个重要的尺寸时才会暴露出来。在这种情况下,治疗困难,需要广泛的手术程序,可以切除几个相邻的结构,可能通过辅助放疗完成。尽管治疗成功,复发率仍然很高。我们报告了一个巨大的脂肪肉瘤的病例,需要进行涉及肿瘤切除的整体广泛切除,左肾,左肾上腺,和后腹壁的一部分。
    Tumors originating from soft tissues are uncommon, among these tumors, liposarcomas are the most frequent. These tumors remain asymptomatic for a long time, and only revealing themselves when they reach an important size. In such cases, treatment is difficult, requiring extensive surgery procedures that can excise several adjacent structures, potentially completed by adjuvant radiotherapy. Despite successful treatment, the recurrence rate remains very high. We report the case of a giant liposarcoma requiring a monobloc extensive resection involving the removal of the tumor, left kidney, left adrenal gland, and a portion of the posterior abdominal wall.
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  • 文章类型: Journal Article
    背景:腹膜后脂肪肉瘤(RLPS)占腹膜后肉瘤的大多数。虽然手术切除仍然是唯一的治疗方法,确定RLPS的最佳手术策略仍然难以捉摸.这项研究解决了围绕RLPS最佳手术策略的持续辩论。
    方法:我们招募了77例接受积极手术治疗的RLPS患者。患者分为三种手术亚型:胰腺上RLPS,胰腺RLPS,和胰腺下RLPS。我们的标准化手术策略涉及根据手术亚型切除宏观上未受累的相邻器官。我们收集了临床,用于分析的病理和预后数据。
    结果:中位随访时间为45.5个月。总生存期(OS)和无复发生存期(RFS)与多灶性RLPS显著相关,病理亚型,复发RLPS和组织学分级(OS分别为P=0.011,0.004,0.010和<0.001,RFS分别为P=0.004,0.001,<0.001和<0.001)。高分化脂肪肉瘤(WDLPS)的5年估计OS,G1RLPS,从头RLPS和单焦点RLPS为100%,89.4%,75.3%和69.1%,分别。远处转移率为1.4%。胰腺上的发病率(≥III级),胰腺,胰下RLPS为26.7%,15.6%,和13.3%,分别。围手术期死亡率为2.6%。
    结论:标准化的积极手术策略证明了RLPS的预后益处,特别是对于G1RLPS,WDLPS,单焦点RLPS,和从头RLPS。这种方法有效地平衡了充分暴露的考虑,手术安全,彻底去除所有脂肪组织。G1RLPS,WDLPS,单焦点RLPS,从头RLPS可能是积极手术政策的潜在指征。
    BACKGROUND: Retroperitoneal liposarcoma (RLPS) constitutes the majority of retroperitoneal sarcomas. While surgical resection remains the sole curative approach, determining the optimal surgical strategy for RLPS remains elusive. This study addresses the ongoing debate surrounding the optimal surgical strategy for RLPS.
    METHODS: We recruited 77 patients with RLPS who underwent aggressive surgical policies. Patients were categorized into three surgical subtypes: suprapancreatic RLPS, pancreatic RLPS, and subpancreatic RLPS. Our standardized surgical strategy involved resecting macroscopically uninvolved adjacent organs according to surgical subtypes. We collected clinical, pathological and prognostic data for analyses.
    RESULTS: The median follow-up was 45.5 months. Overall survival (OS) and recurrence-free survival (RFS) were significantly correlated with multifocal RLPS, pathological subtype, recurrent RLPS and histological grade (P for OS = 0.011, 0.004, 0.010, and < 0.001, P for RFS = 0.004, 0.001, < 0.001, and < 0.001, respectively). The 5-Year Estimate OS of well-differentiated liposarcoma (WDLPS), G1 RLPS, de novo RLPS and unifocal RLPS were 100%, 89.4%, 75.3% and 69.1%, respectively. The distant metastasis rate was 1.4%. The morbidity rates (≥ grade III) for suprapancreatic, pancreatic, and subpancreatic RLPS were 26.7%, 15.6%, and 13.3%, respectively. The perioperative mortality rate is 2.6%.
    CONCLUSIONS: Standardized aggressive surgical policies demonstrated prognostic benefits for RLPS, particularly for G1 RLPS, WDLPS, unifocal RLPS, and de novo RLPS. This approach effectively balanced considerations of adequate exposure, surgical safety, and thorough removal of all fat tissue. G1 RLPS, WDLPS, unifocal RLPS, and de novo RLPS could be potential indications for aggressive surgical policies.
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  • 文章类型: Case Reports
    睾丸旁肿瘤是罕见的恶性肿瘤,常被误诊。我们介绍了一个有六个月无痛史的老年男性病例,逐渐增加的左腹股沟肿胀。在初步审查和调查中,肿胀被误诊为淋巴结肿物。随后,一项磁共振成像研究发现了一个与精索没有明显区别的病变。所述病变的活检测试提示低分化的梭形细胞肿瘤。然后,患者接受了腹股沟高位睾丸切除术。组织病理学检查证实了具有横纹肌母细胞分化的高级别睾丸旁分化脂肪肉瘤的诊断。由于这种肿瘤的罕见,对辅助化疗和放疗的需求存在争议。
    Paratesticular tumours are rare malignancies that are frequently misdiagnosed on presentation. We present a case of an elderly male with a six-month history of painless, progressively increasing left inguinal swelling. On preliminary examination and investigation, the swelling was misdiagnosed as a lymph nodal mass. Subsequently, a magnetic resonance imaging study detected a lesion that was not distinct from the spermatic cord. Biopsy testing of the said lesion was suggestive of poorly differentiated spindle cell neoplasm. The patient then underwent a high inguinal orchidectomy. Histopathological examination confirmed the diagnosis of a high-grade paratesticular dedifferentiated liposarcoma with rhabdomyoblastic differentiation. Due to the rarity of such tumours, the need for adjuvant chemotherapy and radiotherapy is debated.
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  • 文章类型: Journal Article
    目的:腹膜后脂肪肉瘤(RLPS)的根治性切除可能需要血管切除和重建。进行该研究以评估具有主要血管受累的RLPS的手术结果。
    方法:2011年4月至2022年12月在北京大学肿瘤医院肉瘤中心接受手术切除的RLPS患者从前瞻性维护的数据库中进行鉴定。患者分为两组:血管切除组和非血管切除组。进行倾向评分匹配分析以消除组间的基线差异。分析手术细节和术后结果。此外,评估了无局部复发生存期(LRFS)和总生存期(OS)的预后因素.
    结果:总体而言,199例患者被确定,中位随访期为48个月(四分位距[IQR]45-69)。42例(21%)患者行血管切除,其中25人血管浸润。共有39例患者进行了血管置换,3例患者进行了部分切除术(侧壁切除术)。血管切除的主要发病率较高(38%vs.14%,p<0.001)和30天死亡率(7.1%vs.1.3%,p=0.005)。经过倾向匹配分析,接受血管切除术的患者的5年LRFS和OS率与无血管受累的患者相当.大血管切除不是LRFS或OS的独立危险因素。
    结论:尽管伴随着重大发病率和死亡率的风险增加,大血管切除使晚期RLPS患者能够进行根治性切除,与没有提供的5年期LRFS和OS费率相比,提供可比的5年期LRFS和OS费率。
    OBJECTIVE: Radical resection of retroperitoneal liposarcoma (RLPS) may necessitate vascular resection and reconstruction. The study was conducted to assess surgical outcomes of surgery for RLPS with major vascular involvement.
    METHODS: Patients with RLPS who underwent surgical resection at the Sarcoma Center of Peking University Cancer Hospital between April 2011 and December 2022 were identified from a prospectively maintained database. Patients were classified into two groups: vascular resection and non-vascular resection groups. A propensity score matching analysis was performed to eliminate baseline differences between the groups. Surgical details and postoperative outcomes were analyzed. Furthermore, prognostic factors for local recurrence-free survival (LRFS) and overall survival (OS) were assessed.
    RESULTS: Overall, 199 patients were identified and the median follow-up period was 48 (interquartile range [IQR] 45-69) months. Vascular resection was performed in 42 (21%) patients, 25 of whom had vascular infiltration. A total of 39 patients had vascular replacement and 3 patients underwent partial resection (side-wall resection). Vascular resection was burdened by higher rates of major morbidity (38% vs. 14%, p < 0.001) and 30-day mortality (7.1% vs. 1.3%, p = 0.005). After propensity-matched analysis, patients who underwent vascular resection had 5-year LRFS and OS rates comparable to those without vascular involvement. Major vascular resection was not an independent risk factor for LRFS or OS.
    CONCLUSIONS: Although accompanied by increased risks of major morbidity and mortality, the major vascular resection enabled radical resection in patients with advanced RLPS, affording comparable 5-year LRFS and OS rates compared to those who did not.
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  • 文章类型: Journal Article
    单个细胞外囊泡(EV)的分析有可能产生有关其形态结构的有价值的无标记信息,生物标志物和治疗靶点,尽管由于缺乏对这些顺应性纳米级颗粒的机械性能的可靠和定量测量而阻碍了这种分析。机械性能测量的技术挑战来自现有的工具和方法,这些工具和方法提供有限的吞吐量,和报告的弹性模量范围在几个数量级。这里,我们报告了一种基于流动的方法,辅以透射电子显微镜(TEM)成像,以提供高通量,全EV变形分析,用于估计脂肪肉瘤衍生的EV的机械性能与其大小的关系。我们的研究包括从432张TEM图像的大型数据集中提取电动汽车的形态数据,对于包含单个到多个EV的图像,并实现了薄壳变形理论。我们估计了弹性模量,对于小型电动汽车(sEV;30-150nm),E=0.16±0.02MPa(平均值±SE),对于大型电动汽车(lEV;>150nm),E=0.17±0.03MPa(平均值±SE)。据我们所知,这是关于LPS衍生的EV的机械性能估计的第一份报告,并且有可能在EV尺寸和EV机械性能之间建立关系。
    Analysis of single extracellular vesicles (EVs) has the potential to yield valuable label-free information on their morphological structure, biomarkers and therapeutic targets, though such analysis is hindered by the lack of reliable and quantitative measurements of the mechanical properties of these compliant nanoscale particles. The technical challenge in mechanical property measurements arises from the existing tools and methods that offer limited throughput, and the reported elastic moduli range over several orders of magnitude. Here, we report on a flow-based method complemented by transmission electron microscopy (TEM) imaging to provide a high throughput, whole EV deformation analysis for estimating the mechanical properties of liposarcoma-derived EVs as a function of their size. Our study includes extracting morphological data of EVs from a large dataset of 432 TEM images, with images containing single to multiple EVs, and implementing the thin-shell deformation theory. We estimated the elastic modulus, E = 0.16 ± 0.02 MPa (mean±SE) for small EVs (sEVs; 30-150 nm) and E = 0.17 ± 0.03 MPa (mean±SE) for large EVs (lEVs; >150 nm). To our knowledge, this is the first report on the mechanical property estimation of LPS-derived EVs and has the potential to establish a relationship between EV size and EV mechanical properties.
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  • 文章类型: Case Reports
    背景:多态变体是最不常见的,因此,最不为人所知的脂肪肉瘤亚型。它占所有脂肪肉瘤的<5%,并在成年后期发展。
    方法:我们报告了一例77岁男性患者,患者右侧髂窝和右侧腹股沟区疼痛。CT扫描显示盲肠水平有石质病变。进行了右半结肠切除术,病理报告为多形性脂肪肉瘤阳性。因此,他被转介接受辅助放疗。
    分子分析验证了脂肪肉瘤亚型的区别,尽管组织学亚型仍然是日常实践中可靠的预后参数,最近的证据表明,基因谱最终可能会影响个体患者的风险分层。
    结论:脂肪肉瘤的多形性亚型很少见,它们在盲肠中非常罕见,因此,目前建议由多学科团队对患者进行评估以做出治疗决定,因为这是一种非常攻击性行为的亚型,在开始任何类型的肿瘤治疗之前。
    BACKGROUND: The pleomorphic variant is the least common and consequently, the least known subtype of liposarcomas. It represents <5 % of all liposarcomas and develops during late adulthood.
    METHODS: We report the case of a 77-year-old male who presented with pain in the right iliac fossa and right inguinal region. A CT scan revealed a stony lesion at the level of the cecum. A right hemicolectomy was performed, and the pathology report was positive for pleomorphic liposarcoma. Consequently, he was referred for adjuvant radiotherapy.
    UNASSIGNED: Molecular analysis has validated the distinction of liposarcoma subtypes, although histological subtype remains the reliable prognostic parameter in daily practice, recent evidence suggests that genetic profiles may eventually influence risk stratification of individual patients.
    CONCLUSIONS: The pleomorphic subtype of liposarcomas is infrequent, and they are very rare in the cecum, so it is currently recommended that patients be evaluated by a multidisciplinary team to make a therapeutic decision as it is a subtype of very aggressive behavior, before initiating any type of oncologic treatment.
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