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
    一名70多岁的男子出现了左腹股沟大肿块。睾丸肿瘤标志物显示人绒毛膜促性腺激素(hCG)明显升高。切除24.5厘米的肿块,组织学证实罕见诊断为伴有横纹肌肉瘤分化的睾丸旁分化脂肪肉瘤(DDLPS)。患者在向全科医生就诊11个月后因远处转移而死亡。产生HCG的软组织肉瘤(STS)通常被报道为高级,分化差,预后差。hCG在肿瘤血管生成中的作用可能会影响这些特征。腹膜后STS的管理影响了睾丸旁STS治疗指南,相对更常见。对泌尿生殖系统STS的研究表明,积极的手术切缘对局部复发和无转移生存构成最大的风险。这个案例证明了产生DDLPS的hCG的快速增长,转移的倾向,预后不良,需要进一步研究DDLPS辅助放疗的益处。
    A man in his 70s presented with a left inguinoscrotal mass. Testicular tumour markers showed markedly elevated human chorionic gonadotropin (hCG). The 24.5 cm mass was resected, and histology confirmed a rare diagnosis of paratesticular dedifferentiated liposarcoma (DDLPS) with rhabdomyosarcomatous differentiation. The patient expired with distant metastasis 11 months after presenting to his general practitioner.HCG-producing soft tissue sarcomas (STS) are commonly reported as high-grade, poorly differentiated and with a poor prognosis. The role of hCG in tumour angiogenesis may influence these features.Paratesticular STS treatment guidelines have been influenced by the management of retroperitoneal STS, which are relatively more common. Studies of genitourinary STS demonstrate that positive surgical margins pose the greatest risk to local recurrence and metastasis-free survival.This case demonstrates the rapid growth of DDLPS-producing hCG, the propensity to metastasise, and poor prognosis, requiring further research into the benefit of adjuvant radiotherapy for DDLPS.
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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: Journal Article
    肉瘤浆液性积液并不常见,在诊断上具有挑战性。去分化和多形性脂肪肉瘤是胸腔积液中的罕见肿瘤,揭示了模仿癌的高度多形性肿瘤细胞,间皮瘤,黑色素瘤,和其他肉瘤。胸腔积液进一步使细胞学诊断复杂化。正确的细胞学识别很重要。我们报告了一名56岁的男性在积液液中通过细胞学检测到的多形性脂肪肉瘤,该男性表现出大量的单侧胸腔积液。ThinPrep显示出血性积液,其特征是红细胞溶解,泡沫巨噬细胞,和铁皮噬菌体与高度多形性,主要是裸露的单核和巨核混合。聚集的铁皮细胞和空泡巨噬细胞可能被误认为是肿瘤细胞,而裸露的细胞核可能作为非特异性退化变化而被遗漏。细胞块切片显示高度多形性的单核和多核巨大肿瘤细胞具有诊断性成纤维细胞,与泡沫巨噬细胞和铁皮混合。细胞块免疫细胞化学显示肿瘤细胞中波形蛋白和S-100蛋白的染色。其他谱系特异性免疫标记为阴性。CD68和钙视网膜素揭示了频繁的背景巨噬细胞和稀缺的间皮细胞。肿瘤细胞MDM2和CDK4阴性。接受的细胞病理学诊断为多形性脂肪肉瘤。从肿块中获取核心针活检。组织标本的组织病理学特征和免疫特征与细胞病理学和免疫细胞化学发现相匹配,证实了多形性脂肪肉瘤的细胞学诊断。多形性脂肪肉瘤是一种意外的细胞学上具有挑战性的发现,特别是当因含铁血黄素液引入的陷阱而加剧时。注意某些细胞学线索可减少陷阱。当与相关的阴性和阳性免疫细胞化学标志物整合时,细胞阻断是有价值的诊断工具。
    Sarcomatous serous effusions are uncommon and diagnostically challenging. Dedifferentiated and pleomorphic liposarcomas are rare tumors in pleural effusions revealing highly pleomorphic tumor cells mimicking carcinoma, mesothelioma, melanoma, and other sarcomas. Hematothoracic effusions further complicate the cytologic diagnosis. Correct cytologic recognition is important. We report pleomorphic liposarcoma cytologically detected in effusion fluid in a 56-year-old man who presented with a massive unilateral pleural effusion. ThinPrep showed hemorrhagic effusion fluid characterized by lysed red blood cells, foamy macrophages, and siderophages intermixed with highly pleomorphic predominantly naked mononuclear and giant nuclei. The aggregated siderophages and vacuolated macrophages could be mistaken for tumor cells, whereas the bare nuclei may be missed as nonspecific degenerate changes. Cellblock sections showed highly pleomorphic mononuclear and multinucleated giant tumor cells with diagnostic lipoblasts, intermixed with foamy macrophages and siderophages. Cellblock immunocytochemistry showed staining for vimentin and S-100 protein in the tumor cells. Other lineage-specific immunomarkers were negative. CD68 and calretinin revealed frequent background macrophages and scarce mesothelial cells. The tumor cells were negative for MDM2 and CDK4. The entertained cytopathologic diagnosis was pleomorphic liposarcoma. Core needle biopsy was procured from the mass. The histopathologic features and immunoprofile of the tissue specimen matched the cytopathologic and immunocytochemical findings confirming the cytologic diagnosis of pleomorphic liposarcoma. Pleomorphic liposarcoma is an unexpected cytologically challenging finding in effusions, particularly when compounded by pitfalls introduced by hemosiderotic fluid. Attention to certain cytologic clues mitigate pitfalls. Cellblock is a valuable diagnostic tool when integrated with relevant negative and positive immunocytochemical markers.
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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: 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.
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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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