liposarcoma

脂肪肉瘤
  • 文章类型: Case Reports
    精索恶性肿瘤是一种罕见的形式,精索脂肪肉瘤甚至是一种罕见的疾病。脂肪肉瘤通常是缓慢进展的,非招标,由于与筋膜室相符,形状可变的界限良好。我们报告一例65岁男性,阴囊有两个月的最初招标和后来的非招标肿块,在睾丸上方。超声检查显示右侧睾丸上极有6x3x3厘米的右侧腹股沟中段肿块,右侧睾丸周围有少量液体。右腹股沟肿块的细针抽吸细胞学(FNAC)显示梭形细胞肿瘤。该患者接受了右腹股沟根治性睾丸切除术,并局部广泛切除了精索起源的肉瘤。最终组织病理学证实去分化脂肪肉瘤。没有提供辅助治疗,对患者进行了监测。随访10个月以上未发现局部复发,区域或非区域淋巴结,或全身转移。
    Spermatic cord malignancies are a scarce modality and liposarcoma of spermatic cord is even a rarer condition encountered. Liposarcoma is usually a slowly progressive, non-tender, well circumscribed mass of variable shapes owing to conformity to fascial compartments. We are reporting a case of 65-year-old male, with a two-month history of initially tender and later non-tender mass in the scrotum, above the testis. Ultrasonography showed a right mid inguinal mass measuring 6x3x3 cm at the superior pole of the right testis and small fluid around the right testis. Fine needle aspiration cytology (FNAC) of the right inguinal mass revealed a spindle cell neoplasm. The patient underwent right inguinal radical orchiectomy with local wide excision of the sarcoma of the spermatic cord origin. Final histopathology confirmed dedifferentiated liposarcoma. No adjuvant treatment was offered and the patient was put on surveillance. Follow-up of more than 10 months has not revealed any local recurrence, regional or non-regional lymph nodes, or systemic metastasis.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

    求助全文

  • 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.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Pubmed)

  • 文章类型: Journal Article
    背景:黏液性脂肪肉瘤黏液样脂肪肉瘤是一种来源于血管周围未分化基质细胞的恶性黏液样软组织肿瘤,下体腔和肌肉间隙,由前脂肪细胞分化为成熟细胞的不同阶段的细胞组成。在极少数情况下,它可能会从脂肪瘤恶性肿瘤改变。主要表现为无痛肿块,相对缓慢的增长,课程可以持续几十年,脂肪肉瘤在人群中的患病率为14%至18%,主要是成年人,男性患病率高于女性,但不重要。主要的好头发部分是大腿,有粘液性,高分化型,去分化类型,多态类型。临床诊断困难,早期没有明显的症状,所以诊断要结合B超,MRI,CT,以及其他辅助考试。金标准是病理检查。2023年12月,我们部门收治了一名腹部黏液性肿块患者。报告如下。
    方法:脂肪肉瘤会转移吗?手术后需要化疗吗?将来会复发吗?手术后的生存期是多少?
    方法:粘液性脂肪肉瘤。
    方法:手术切除肉瘤。
    结果:结节样本为33*28*13厘米,有完整的胶囊,灰色和黄色部分,质地细腻,软,灰色,红色,灰色,和黄色的粘液结节在某些地区,结节最大直径为21cm。免疫组化为:CD34(+),CDK4(+),CK(-),Desmin(弱+),Ki67(指数5%),MDM2(-),p16(弱+),S-100P(+),波形蛋白(+),BCL-2(+)。他还被送到北京协和医院病理科与陆朝晖教授会诊,其咨询意见与黏液脂肪肉瘤一致。
    结论:腹膜后脂肪肉瘤是一种常见的腹膜后肿瘤,但在临床实践中相对罕见;总体发病率较低,主要表现为腹痛和腹胀,腹胀,病程长;对放疗和化疗不敏感,并应密切随访CT检查以了解复发和转移。
    BACKGROUND: Mucinous liposarcoma myxoid liposarcoma is a malignant mucoid soft tissue tumor derived from undifferentiated stromal cells in perivascular, subbody cavity and intermuscular space, and composed of cells at different stages of differentiation from preadipocytes to mature cells. In rare cases, it may change from lipoma malignancy. The main manifestations is painless mass, relatively slow growth, the course can last decades, the prevalence of liposarcoma in the population is 14% to 18%, mainly in adults, male prevalence is higher than women, but not significant. The main good hair part is the thigh, have mucinous sex, high differentiation type, dedifferentiation type, polymorphic type. Clinical diagnosis is difficult, and there are no obvious symptoms in the early stage, so the diagnosis should be combined with B ultrasound, MRI, CT, and other auxiliary examinations. The gold standard is pathological examination. In December 2023, our department admitted a patient with a mucinous abdominal mass. The report is as follows.
    METHODS: Does liposarcoma metastasize? Is any chemotherapy required after surgery? Will it ever relapse in the future? What is the survival period after surgery?
    METHODS: Mucinous liposarcoma.
    METHODS: Surgical resection of the sarcoma.
    RESULTS: The nodule sample was 33 * 28 * 13 cm, with complete capsule, gray and yellow sections, fine texture, soft, gray, red, grayish, and yellow mucoid nodules in some areas, and the maximum diameter of the nodules was 21cm. Immunohistochemistry was: CD34 (+), CDK 4 (+), CK (-), Desmin (weak +), Ki67 (index 5%), MDM 2 (-), p16 (weak +), S-100P (+), Vimentin (+), BCL-2 (+). He was also sent to the Department of Pathology of Peking Union Medical College Hospital for consultation with Professor Lu Zhaohui, whose consultation opinion was in line with myxoliposarcoma.
    CONCLUSIONS: Retroperitoneal liposarcoma is a common retroperitoneal tumor, but it is relatively rare in clinical practice; the overall morbidity is low, mainly manifested as abdominal pain and abdominal distension, abdominal distension, and a long course of disease; it is not sensitive to radiotherapy and chemotherapy, and should be closely follow up by CT examination to understand the recurrence and metastasis.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

    求助全文

  • 文章类型: Case Reports
    在脂肪肉瘤中,高分化脂肪肉瘤和去分化脂肪肉瘤是最常见的。这些肿瘤中的大多数在深腹膜后或四肢中发现。当在腹膜外发现时,这些脂肪源性肿瘤被称为非典型脂肪瘤(ALT)。浅表ALT特别罕见;因此,对他们的临床表现知之甚少,基因组状态,和管理。这里,我们介绍了一个54岁的男子的情况,在他的左上背部逐渐长大了两年多,偶然诊断为ALT。这个病人的ALT,然而,显示与MDM2和对照着丝粒12(CEP12)共扩增和阴性CD34和S100和RB1表达的高度多态性,与文献中描述的大多数其他ALT不同。该病例报告详细介绍了脂肪组织的诊断检查和组织病理学发现,并总结了不同的亚型。包括非典型梭形细胞/多形性脂肪瘤,多形性脂肪肉瘤,梭形细胞/多形性脂肪瘤,简要讨论管理。
    UNASSIGNED: Among liposarcomas, well-differentiated liposarcoma and dedifferentiated liposarcoma are the most common. The majority of these tumors are found in deep retroperitoneum or extremities. When found outside the retroperitoneum, these adipose-derived tumors are known as atypical lipomatous tumors (ALT). Superficial ALT are particularly rare; thus, little is known about their clinical presentation, genomic status, and management. Here, we present the case of a 54-year-old man with an intermittently bothersome, slowly growing mass on his left upper back for over 2 years, which was incidentally diagnosed as ALT. This patient\'s ALT, however, showed a profound degree of pleomorphism with MDM2 and control centromere 12 (CEP12) coamplification and negative CD34 and S100 and RB1 expression, unlike most other ALT described in the literature. This case report details the diagnostic workup and histopathological findings for adipose tumors and summarizes the different subtypes, including atypical spindle cell/pleomorphic lipomatous tumor, pleomorphic liposarcoma, and spindle cell/pleomorphic lipoma, with brief discussion on management.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

    求助全文

  • 文章类型: Journal Article
    背景:这是一个多中心,单臂,II期研究旨在进一步探索trabectedin在腹膜后平滑肌肉瘤(LMS)和高分化/去分化脂肪肉瘤(LPS)中作为二线/进一步线治疗的活性。
    方法:主要终点是生长调节指数(GMI),定义为trabectedin下的PFS(PFS)与先前化疗期间的PFS之间的比率:进展时间(TTP-1)。次要终点是客观缓解率(ORR)和PFS。根据协议,如果GMI>1.33,则患者被认为是应答者,如果<0.75,则为无应答者,如果为0.76-1.32,则均为无应答者.
    结果:共有91例患者可评估主要终点(32例LMS患者和59例LPS患者):接受的周期中位数为6.0(Q1-Q33.0-12.0),治疗中断的主要原因是72%的患者出现疾病进展。中位PFS为6.0个月,而中位TTP1为7.5个月(LMS和LPS的8.1和6.4个月,分别)。33名患者[52%,95%置信区间(CI)36%至58%,P=0.674,反应几率1.1]的GMI>1.33(LMS46%,95%CI26%至67%,应答几率0.85;LPS56%,95%CI40%至72%,响应几率1.3)。总的来说,在LPS中,我们观察到15/47患者的GMI<0.5和15/47患者的GMI>2。在LMS患者中,9/26的GMI<0.5,10/26的GMI>2。总的来说,ORR(完全反应+部分反应)为16%(LMS为24%,LPS为12%)。
    结论:虽然未达到研究的主要终点,我们注意到,与以前的治疗相比,TTP与trabectedin显著差异的患者亚组(GMI<0.5或>2,后者包括一些TTP与trabectedin的患者).观察到PFS和总生存期不匹配,可能是由于两种不同组织学的自然史以及LMS中其他细胞系的可用性。
    BACKGROUND: This is a multicentre, single-arm, phase II study aimed at further exploring the activity of trabectedin as second-/further-line treatment in retroperitoneal leiomyosarcoma (LMS) and well-differentiated/dedifferentiated liposarcoma (LPS).
    METHODS: The primary endpoint was the growth modulation index (GMI) defined as the ratio between PFS under trabectedin (PFS) and during previous chemotherapy treatment: time to progression (TTP-1). Secondary endpoints were objective response rate (ORR) and PFS. As per protocol, patients were considered responders if the GMI was >1.33, non-responders if <0.75 and neither if 0.76-1.32.
    RESULTS: Overall 91 patients were assessable for the primary endpoint (32 patients with LMS and 59 patients with LPS): the median number of cycles received was 6.0 (Q1-Q3 3.0-12.0), and the main reason for treatment discontinuation was disease progression in 72% of patients. The median PFS was 6.0 months, while the median TTP1 was 7.5 months (8.1 and 6.4 months for LMS and LPS, respectively). Thirty-three patients [52%, 95% confidence interval (CI) 36% to 58%, P = 0.674, odds of response 1.1] had a GMI >1.33 (LMS 46%, 95% CI 26% to 67%, odds of response 0.85; LPS 56%, 95% CI 40% to 72%, odds of response 1.3). Overall, in LPS we observed 15/47 patients with a GMI <0.5 and 15/47 patients with a GMI >2. Among LMS patients, 9/26 had a GMI <0.5 and 10/26 had a GMI >2. Overall, ORR (complete response + partial response) was 16% (24% for LMS and 12% for LPS).
    CONCLUSIONS: While the primary endpoint of the study was not met, we noticed a subgroup of patients with a markedly discrepant TTP with trabectedin in comparison to previous therapy (GMI <0.5 or >2, the latter including some patients with a long TTP with trabectedin). A mismatch between PFS and overall survival was observed, possibly due to the natural history of the two different histologies and the availability of further lines in LMS.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

    求助全文

  • 文章类型: Case Reports
    非典型梭形细胞/多形性脂肪瘤被归类为良性脂肪瘤,但是各种MRI检查结果提出了准确的诊断挑战。在我们的案例中,MRI和PET/CT扫描均提示非典型脂肪瘤/高分化脂肪肉瘤或去分化脂肪肉瘤的可能性.穿刺活检提示良性至低度恶性;因此,我们选择了广泛切除.通过组织病理学分析,最终诊断为非典型梭形细胞/多形性脂肪瘤,包括免疫组织化学和荧光原位杂交。由于仅通过成像实现准确诊断可能具有挑战性,组织病理学仍然是必不可少的。
    UNASSIGNED: Atypical spindle cell/pleomorphic lipomatous tumor is categorized as a benign lipomatous tumor, but various MRI findings pose accurate diagnostic challenges. In our case, both MRI and PET/CT scans indicated the possibility of atypical lipomatous tumor/well-differentiated liposarcoma or dedifferentiated liposarcoma. Needle biopsy suggested benign to low-grade malignancy; hence, we opted for the wide resection. The final diagnosis of atypical spindle cell/pleomorphic lipomatous tumor was confirmed through histopathology analysis, including immunohistochemistry and fluorescence in situ hybridization. Since achieving an accurate diagnosis solely through imaging can be challenging, histopathology remains essential.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

    求助全文

  • 文章类型: Journal Article
    目的:当存在不会经历感兴趣事件的患者亚群时,治疗模型是肿瘤学研究中Cox比例风险模型的有用替代方法。虽然软件可用于治疗模型,评估的工具有限,报告,并将模型结果可视化。本文介绍了cureitR包,用于构建混合物固化模型的端到端管道,并证明了其在原发性四肢和躯干脂肪肉瘤患者数据集中的用途。
    方法:为了评估1982年7月至2017年9月在纪念斯隆·凯特琳癌症中心治疗的患者的脂肪肉瘤组织学亚型与疾病特异性死亡(DSD)之间的关联,使用治疗包适合并评估混合治疗模型。脂肪肉瘤组织学亚型被定义为高分化,去分化,粘液样,圆形细胞,多形性。
    结果:与分化良好的治疗模型相比,所有其他分析的脂肪肉瘤组织学亚型与较高的DSD显著相关。在多变量模型中,粘液样(比值比[OR],6.25[95%CI,1.32至29.6])和圆形单元格(OR,16.2[95%CI,2.80至93.2])脂肪肉瘤与高分化患者相比,DSD的发生率更高。相比之下,去分化脂肪肉瘤与DSD的潜伏期相关(风险比,10.6[95%CI,1.48至75.9])。多形性脂肪肉瘤在DSD的发病率和潜伏期均具有显著较高的风险(P<0.0001)。Brier评分表明治愈和Cox模型之间具有可比性的预测准确性。
    结论:我们开发了cureit管道以适应和评估混合治疗模型,并证明了其在脂肪肉瘤疾病环境中的临床实用性,关于亚型特异性关联与发病率和/或潜伏期的见解。
    OBJECTIVE: Cure models are a useful alternative to Cox proportional hazards models in oncology studies when there is a subpopulation of patients who will not experience the event of interest. Although software is available to fit cure models, there are limited tools to evaluate, report, and visualize model results. This article introduces the cureit R package, an end-to-end pipeline for building mixture cure models, and demonstrates its use in a data set of patients with primary extremity and truncal liposarcoma.
    METHODS: To assess associations between liposarcoma histologic subtypes and disease-specific death (DSD) in patients treated at Memorial Sloan Kettering Cancer Center between July 1982 and September 2017, mixture cure models were fit and evaluated using the cureit package. Liposarcoma histologic subtypes were defined as well-differentiated, dedifferentiated, myxoid, round cell, and pleomorphic.
    RESULTS: All other analyzed liposarcoma histologic subtypes were significantly associated with higher DSD in cure models compared with well-differentiated. In multivariable models, myxoid (odds ratio [OR], 6.25 [95% CI, 1.32 to 29.6]) and round cell (OR, 16.2 [95% CI, 2.80 to 93.2]) liposarcoma had higher incidences of DSD compared with well-differentiated patients. By contrast, dedifferentiated liposarcoma was associated with the latency of DSD (hazard ratio, 10.6 [95% CI, 1.48 to 75.9]). Pleomorphic liposarcomas had significantly higher risk in both incidence and the latency of DSD (P < .0001). Brier scores indicated comparable predictive accuracy between cure and Cox models.
    CONCLUSIONS: We developed the cureit pipeline to fit and evaluate mixture cure models and demonstrated its clinical utility in the liposarcoma disease setting, shedding insights on the subtype-specific associations with incidence and/or latency.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

    求助全文

  • 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.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Pubmed)

  • 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.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Pubmed)

  • 文章类型: Journal Article
    脂肪肉瘤(LPS)是一种罕见的软组织肉瘤,从脂肪细胞的分化发展,通常发生在下肢和腹膜后间隙。根据其组织学形态和分子变化,LPS可分为各种亚型,每个都表现出不同的生物学行为。治疗期间,特别是在腹膜后产生的LPS,初次手术的范围和质量至关重要.治疗策略必须针对LPS的特定类型进行调整。在过去的几十年里,LPS的治疗经历了许多进步,随着新的治疗方法,如靶向药物和免疫疗法不断涌现。本文综述了其生物学特性,分子改变,以及各种LPS亚型的手术和药物治疗,目的是增强临床医生的理解并强调个体化精准治疗的重要性。随着对LPS生物学特性和分子改变的深入了解,未来的治疗趋势可能更侧重于制定个性化治疗计划,以更好地解决各种类型的LPS.
    Liposarcoma (LPS) is a rare soft tissue sarcoma that develops from the differentiation of fat cells, typically occurring in the lower extremities and retroperitoneal space. Depending on its histological morphology and molecular changes, LPS can be divided into various subtypes, each exhibiting distinct biological behaviors. During treatment, especially for LPS arising in the retroperitoneum, the extent and quality of the initial surgery are critically important. Treatment strategies must be tailored to the specific type of LPS. Over the past few decades, the treatment of LPS has undergone numerous advancements, with new therapeutic approaches such as targeted drugs and immunotherapies continually emerging. This paper reviews the biological characteristics, molecular alterations, as well as surgical and pharmacological treatments of various LPS subtypes, with the aim of enhancing clinicians\' understanding and emphasizing the importance of individualized precision therapy. With a deeper understanding of the biological characteristics and molecular alterations of LPS, future treatment trends are likely to focus more on developing personalized treatment plans to better address the various types of LPS.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

    求助全文

公众号