Liposarcoma

脂肪肉瘤
  • 文章类型: Case Reports
    在间质肿瘤中,脂肪肉瘤约占所有肉瘤的20%。粘液瘤脂肪肉瘤是一种罕见的纵隔肿瘤,似乎与其他肺部疾病相同。最常见的症状是胸痛,呼吸困难,和吞咽困难.大多数诊断结果是通过放射学或术后组织病理学检查提供的。手术和化疗,在某些情况下,是治疗的基础。患有这种疾病的人如果接受早期诊断和治疗,则获得有利结果的可能性更高。我们介绍了一例48岁男性原发性纵隔脂肪肉瘤。患者主诉胸痛和呼吸急促伴咳嗽。计算机断层扫描(CT)显示右下胸腔有一个大的右囊性肿块。手术完成了,手术标本的组织病理学检查证实了诊断。
    Liposarcomas account for about 20% of all sarcomas among mesenchymal neoplasms. Myxomatous liposarcoma is a rare mediastinal tumor that seems the same as other lung disorders. The most common presenting symptoms are chest pain, dyspnea, and dysphagia. Most of the diagnostic findings are provided by radiological or postoperative histopathological tests. Surgery and chemotherapy, in some cases, are the basis of treatment. People with this condition have a higher probability of a favorable outcome if they receive an early diagnosis and treatment. We present a case of a 48-year-old male with primary mediastinal liposarcoma. The patient complained of chest pain and shortness of breath with a productive cough. Computed tomography (CT) showed a large right cystic mass on the right lower thoracic cavity. Surgery was done, and a histopathological examination of the surgical specimen confirmed the diagnosis.
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  • 文章类型: Case Reports
    这项研究检查了一个独特的案例,一个61岁的男性,有5年的历史,他的右肩上方的肿块逐渐增加,诊断为去分化多形性脂肪肉瘤。使用计算机断层扫描引导的核心针活检,肿瘤被鉴定为中高级。手术切除需要术前放疗以减小肿瘤的大小。几个独特的特征区分了这个特殊的脂肪肉瘤病例:它的巨大尺寸,其不可预测的增长模式,它没有转移,尤其是,长期得不到治疗。此病例报告概述了临床背景,诊断程序,以及用于控制这种情况的治疗方式,强调结合放疗和手术的局部双重治疗方法。重点放在区分脂肪肉瘤和脂肪母细胞瘤,良性脂肪细胞肿瘤,便于准确诊断和选择合适的治疗方法。在这种情况下取得的积极结果可以为类似大小的侵袭性肿瘤的未来治疗和管理提供有价值的见解。
    This study examines a unique case of a 61-year-old male with a 5-year history of a progressively growing mass above his right shoulder, diagnosed as a dedifferentiated pleomorphic liposarcoma. Using computerized tomography-guided core needle biopsy, the tumour was identified as intermediate to high grade. Surgical removal required preoperative radiotherapy to reduce the size of the tumour. Several unique characteristics set apart this particular case of liposarcoma: its substantial size, its unpredictable growth pattern, its absence of metastasis, and notably, its prolonged period of being untreated. This case report outlines the clinical background, diagnostic procedures, and treatment modalities employed in managing this condition, emphasizing a localized dual therapy approach combining radiotherapy and surgery. Emphasis is placed on distinguishing liposarcoma from lipoblastoma, a benign adipocyte tumour, to facilitate accurate diagnosis and appropriate treatment selection. The positive result achieved in this case could provide valuable insights for the future treatment and management of similarly sized aggressive tumours.
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  • 文章类型: Case Reports
    带有壁结节的卵巢粘液性肿瘤是一种罕见且特殊类型的卵巢表面上皮间质肿瘤。壁结节在形态上分为三种类型:肉瘤样,间变性癌,和真正的肉瘤结节.卵巢粘液性肿瘤与真正的肉瘤壁结节是罕见的,具有挑战性的诊断,全球仅报告10例。目前,脂肪肉瘤壁结节仍未报告。
    一名91岁女性因绝经后阴道出血住院3周。影像提示右侧卵巢见较大囊性肿块(20.0cm×17.7cm×12.8cm)。肿块是多房性囊性的,局灶性囊壁有壁结节(1.4cm×1.2cm×1.0cm)。根据组织学形态,免疫组织化学染色,和MDM2/CDK4荧光原位杂交测试,诊断为卵巢粘液性囊腺瘤,附壁结节为高分化脂肪肉瘤。据我们所知,以前从未报道过。高通量测序鉴定了卵巢粘液性囊腺瘤中的KRAS突变。然而,脂肪肉瘤壁结节未显示KRAS突变,但显示CDK4和DDR2的拷贝数扩增,以及ASXL1第13外显子的移码突变(p.A627Gfs*8)。
    该病例扩大了卵巢粘液性肿瘤中的壁结节的形态学范围,加深我们对这种罕见形态的认识。同时,通过高通量测序,我们发现脂肪肉瘤壁结节和相关卵巢粘液性囊腺瘤之间没有重叠的遗传证据.
    UNASSIGNED: Ovarian mucinous tumor with a mural nodule is a rare and special type of ovarian surface epithelial-stromal tumor. Mural nodules are morphologically classified into three types: sarcoma-like, anaplastic carcinomatous, and true sarcomatous nodules. Ovarian mucinous tumors with true sarcomatous mural nodules are rare and challenging to diagnose, with only 10 cases reported worldwide. Currently, liposarcoma mural nodules remain unreported.
    UNASSIGNED: A 91-year-old woman was hospitalized for postmenopausal vaginal bleeding for 3 weeks. Imaging revealed a large cystic mass (20.0 cm × 17.7 cm × 12.8 cm) on the right ovary. The mass was multilocular cystic, with a mural nodule (1.4 cm × 1.2 cm × 1.0 cm) in the focal cyst wall. Based on histological morphology, immunohistochemical staining, and MDM2/CDK4 fluorescence in situ hybridization testing, the diagnosis was ovarian mucinous cystadenoma with a mural nodule of well-differentiated liposarcoma. To the best of our knowledge, this has never been reported before. High-throughput sequencing identified KRAS mutations in the ovarian mucinous cystadenoma. However, the liposarcoma mural nodule did not exhibit KRAS mutations but displayed copy number amplifications of CDK4 and DDR2, as well as a frameshift mutation in exon 13 of ASXL1 (p. A627Gfs*8).
    UNASSIGNED: This case broadens the morphological spectrum of mural nodules in ovarian mucinous tumors, deepening our knowledge of this rare morphology. Meanwhile, through high-throughput sequencing, we found no overlapping genetic evidence between the liposarcoma mural nodule and associated ovarian mucinous cystadenoma.
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  • 文章类型: Case Reports
    背景:腹膜后脂肪肉瘤(RPLPS)是一种相对罕见的疾病。脂肪肉瘤大小不同,但是直径大于30厘米的相当大的RPLPS非常罕见,他们的诊断和治疗提出了重大挑战。
    方法:我们报告了一名58岁的男性患者,因腹围增大入院,后来被诊断为巨大的RPLPS。发现脂肪肉瘤粘附在右肾和整个输尿管上,侵入升结肠.患者接受了完整的联合手术切除。肿瘤完整切除,55.0厘米×30.0厘米×18.0厘米,重19.8kg。组织病理学分析显示高分化脂肪肉瘤(WDLPS)。患者顺利出院,随访6个月,无复发迹象。
    结论:RPLPS是一种罕见的肿瘤,临床表现不典型。手术仍然是治疗腹膜后肉瘤的最有效方法。如果有当地入侵,则完全删除。术前检查,包括三维(3D)重建,是手术成功的关键.辅助放疗或化疗的作用仍存在争议。然而,临床医生不应排除他们是可行的选择.
    BACKGROUND: Retroperitoneal liposarcoma (RPLPS) is a relatively rare disease. Liposarcomas vary in size, but sizeable RPLPS larger than 30 cm in diameter are very rare, and their diagnosis and treatment present significant challenges.
    METHODS: We report a 58-year-old male patient who was admitted to the hospital with an increased abdominal circumference and was later diagnosed with a giant RPLPS. The liposarcoma was found to adhere to the right kidney and the entire ureter, invading the ascending colon. The patient underwent complete combined surgical resection. The tumor was removed intact, measured 55.0 cm × 30.0 cm × 18.0 cm, and weighed 19.8 kg. Histopathologic analysis revealed well-differentiated liposarcoma (WDLPS). The patient was successfully discharged from the hospital and followed up for 6 months with no signs of recurrence.
    CONCLUSIONS: RPLPS is a rare tumor with atypical clinical presentation. Surgery remains the most effective method of treatment for retroperitoneal sarcomas, with complete removal if there is local invasion. Preoperative examination, including three-dimensional (3D) reconstruction, is essential for surgical success. The role of adjuvant radiotherapy or chemotherapy remains controversial. However, clinicians should not rule them out as viable options.
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  • 文章类型: Case Reports
    黏液样多形性脂肪肉瘤(MPLPS)是一种极为罕见的实体,最近已被文献认可并包括在内。脂肪肉瘤是脂肪细胞肿瘤,通常位于四肢和腹膜后。睾丸旁脂肪肉瘤是极为罕见的恶性肿瘤,起源于睾丸旁区域的脂肪组织。黏液样多形性脂肪肉瘤(MPLPS)是一种极其罕见的脂肪肉瘤变体,到目前为止,文献中报道的病例很少。纵隔是MPLPS最常见的部位,其次是四肢,头部,和脖子。我们报告了一例50岁的男性患者,其右侧腹股沟区出现肿胀,这在过去的一个月里引起了病人的注意。经过调查,进行了右高位睾丸切除术.进行了组织病理学检查和免疫组织化学检查,并诊断出涉及精索的粘液样多形性脂肪肉瘤(MPLPS)。因此,在这里,我们在文献中首次报道了一例涉及精索的粘液样多形性脂肪肉瘤。
    Myxoid pleomorphic liposarcoma (MPLPS) is an extremely rare entity that has been recognized and included in the literature recently. Liposarcomas are adipocytic tumors that are usually located in the extremities and retroperitoneum. Paratesticular liposarcomas are extremely rare malignant tumors originating from the adipose tissue of the paratesticular region. Myxoid pleomorphic liposarcoma (MPLPS) is an exceedingly rare variant of liposarcoma, with very few cases reported in the literature so far. Mediastinum is the most common site for MPLPS followed by the limbs, head, and neck. We report a case of a 50-year-old male patient who presented with a swelling in the right inguinal region, which came to the patient\'s attention in the past month. After investigations, a right-high orchidectomy was done. Histopathological examination and immunohistochemistry were performed and a diagnosis of myxoid pleomorphic liposarcoma (MPLPS) involving the spermatic cord was made. So here we report this case of myxoid pleomorphic liposarcoma involving the spermatic cord for the first time in the literature.
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  • 文章类型: Case Reports
    脂肪肉瘤是一种恶性软组织肿瘤,很少累及胃肠道。去分化型通常预后最差。这里,我们描述了1例罕见的直肠去分化脂肪肉瘤病例,该病例为年轻男性患者,接受手术切除治疗.治疗通常很困难,并且对放疗或化疗的益处没有明确的共识。
    Liposarcoma is a malignant soft tissue tumor that rarely involves the gastrointestinal tract. The dedifferentiated type typically carries the worst prognosis. Here, we describe a rare case of dedifferentiated liposarcoma of the rectum in a young male patient who was treated with surgical excision. Treatment is often difficult and there is no clear consensus on the benefits of radiotherapy or chemotherapy.
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  • 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: 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.
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  • 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.
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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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