脂肪肉瘤的靶向治疗研究进展

脂肪肉瘤是起源于间叶组织的恶性肿瘤,是较为常见的软组织肉瘤亚型之一,由于其对放疗和化疗不敏感,早期根治性手术是其首选的治疗方式.尽管进行了彻底的手术切除,但术后局部复发率仍然很高,患者的预后较差.因此,寻找驱动脂肪肉瘤发生发展的潜在分子机制及治疗靶点的相关转化研究和临床试验已成为近年来临床研究的热点.本文总结脂肪肉瘤潜...     

脂肪肉瘤16例临床分析

脂肪肉瘤１６例临床分析高平，赵宇柱上海市徐汇区日晖医院外科（上海２０００３２）我院自１９７０年６月～１９９２年９月共收治脂肪肉瘤１６例，均经手术及病理检查证实，现将１６例临床作一简要分析。临床资料本组１６例脂肪肉瘤，男９例，女７例。年龄最小为２８岁，...     

纵隔脂肪肉瘤4例报告

纵隔脂肪肉瘤临床上较少见.现将我院收治4例报告如下. 1 临床资料 收集1967年6月至2001年6月经我科手术病理确诊纵隔脂肪肉瘤4例进行报道. 病例1,男性,36岁,胸闷20天伴胸背部不适,咳嗽.     

腹膜后脂肪肉瘤的诊断与治疗策略的研究进展

腹膜后脂肪肉瘤是一种罕见的后腹膜间隙内的间质来源肿瘤,典型恶性肿瘤的表现并且通常预后不良。由于该肿瘤较罕见并且解剖位置隐蔽,因此诊断比较困难,在治疗方法上也存在诸多挑战。对肿瘤进行彻底切除后其复发率依然较高,因此需要长时间不定期随访。同时,除外科手术切除外,还有放疗、化疗、免疫治疗以及综合治疗等方式。     

原发性椎管内外脂肪肉瘤一例

患者男,67岁。因双下肢麻木、疼痛1个月伴瘫痪3d,于2006年10月入院。神经系统检查:双上肢肌力、肌张力正常,双下肢肌力0级,肌张力降低,病理征(-),乳头平面以下皮肤痛觉、温觉消失,触觉减退。胸椎MRI检查显示:胸     

大网膜脂肪肉瘤1例

患者男,45岁.因剑突下肿块1年入院.1年前无意中发现剑突下有一鸡蛋大肿块,进食可上下移动,无恶心、呕吐、腹痛、腹泻和黑粪.查体:腹软,剑突下扪及6cm×7cm大肿快,质中、表面光滑、边界清楚、活动度较大、无触痛,无移动性浊音,肠鸣音正常.腹部B超检查:左上腹有一7.4cm×4.1cm实性肿块,边界清楚,形态欠规则,内部回声强弱不等,分布不均匀.CT示:肝左叶下方见一约8.0cm×6.1cm×4.0cm类椭圆形实性肿块,边界清楚,密度均匀,肿块前缘接近右前腹壁,后紧贴胃窦部,肿块周围脂肪间隙清晰,腹内、腹膜后均未发现肿大淋巴结.初步诊断:大网膜良性肿癌.在硬膜外麻醉下行肿瘤切除术,术中见肿块位于大网膜内,约10cm×5cm×4cm大小,呈分叶状,无完整包膜,肿瘤组织血管丰富,状如血管瘤,与胃窦部疏松粘连,完整切除肿瘤.病理诊断:多形性脂肪肉瘤.     

脂肪肉瘤42例临床病理分析

脂肪肉瘤是较常见的软组织肿瘤,我科从1979年1月～1992年12月,14年间对有随访记录的脂肪肉瘤42例。进行病理类型,治疗和预后分析。 资料与方法 复习了42例脂肪肉瘤的病史及组织切片,切除标本以10％福尔马林液固定,每例取组织2～6块,常规石蜡切片,HE染色,大部分病例作苏丹Ⅲ染色。参考Hajdu标准,分为分化良好型、粘液样型、纤维母细胞型、圆型细胞型和多形性5型。     

原发性肾脏粘液性脂肪肉瘤1例

脂肪肉瘤发生于肾脏很少见 ,我院 1 999年诊治 1例。病例报道　患者男性 ,6 3岁。 2月前偶尔发现右腰部包块 ,约“鹅蛋”大小 ,不痛。 1月前患者感到局部胀痛 ,并涉及腹部 ,2周前涉及右下肢 ,但不影响行走。自发现包块以来 ,逐渐增大至“拳头”大小。病程中患者神清 ,无发热、体重减轻 ,也无气促、尿频、尿急、血尿 ,偶有心悸。体检 :右腰部触及 8ｃｍ× 6ｃｍ包块 ,质中 ,边缘不清 ,固定 ,表面光滑 ,皮肤无红肿热 ,有触痛。Ｂ超 :右肾实质占位性病变 (多属肾癌 )。肾盂静脉造影 :右肾占位病变。ＣＴ :右肾巨大囊实性占位病变。考虑为肾恶性…     

脂肪肉瘤及其治疗

脂肪肉瘤是最多见的软组织肿瘤,其临床表现、病理和分子特征多种多样.手术治疗仍然是治疗的基础,是惟一提供治愈可能的方法.分子生物学的发展,相关分子通路、癌基因及抑癌基因等的发现,使靶向药物治疗脂肪肉瘤在将来变为可能.     

脂肪肉瘤的临床病理和基因改变的研究进展

脂肪肉瘤是一种起源于原始间叶细胞的恶性肿瘤,具有显著的组织多样性,由分化和异型程度不同的细胞组成。脂肪肉瘤主要分为四个亚型:以MDM2-HMGA2为关键基因的非典型脂肪源性肿瘤(Atypical lipogenic tumors,ALT)/高分化脂肪肉瘤(Well-differentiated liposarcoma,WDLPS)和去分化脂肪肉瘤(Dedifferentiated liposarcoma,DDLPS),以FUS-CHOP基因融合为主要发生机制的黏液性脂肪肉瘤(Myxoid liposarcoma,MLPS)/圆形细胞脂肪肉瘤(Round cell liposarcoma,RCLPS),以及发病机制复杂且多变的多形性脂肪肉瘤(Pleomorphic liposarcoma,PLPS)。ALT/WDLPS的主要病理变化为多形性成熟脂肪细胞增殖,DDLPS为高级别肉瘤,MLPS/RCLPS主要由非脂质间质细胞组成,而PLPS以异型多空泡脂肪母细胞为特点。这四个亚型所包含的肿瘤基因及临床病理学改变,导致了这四种亚型具有不同的临床行为、治疗敏感性和生物学特性。本文将对这四个亚型的病理及基因改变进行总结和探讨。     

Differences in recurrence and survival of extremity liposarcoma subtypes

Background

Liposarcomas can be divided into four subtypes and are most frequently located in the extremities. There are currently no studies comparing the clinical outcomes, such as local recurrence and distant metastasis, between the distinct subtypes of primary LPS of the extremity specifically.

复发性软组织脂肪肉瘤术前化疗的临床观察

目的观察复发性脂肪肉瘤术前灌注化疗的疗效。方法21例复发性脂肪肉瘤患者,术前采用皮下埋植式动脉介入化疗系统,用阿霉素和顺铂为主,联合咖啡因治疗。结果病变影像改变有效18例,病理中重度以上化疗反应15例,平均随访39个月,术后肿瘤局部复发2例,肺转移1例,3例死亡。结论复发性脂肪肉瘤的治疗可以选择动脉灌注化疗,肿瘤组织学分类恶性程度高的化疗效果更好,以化疗后手术切除的肿瘤病理改变作为重要的预后评价标准。     

成人脂肪肉瘤临床化疗疗效分析

目的 探讨晚期或复发脂肪肉瘤患者化疗有效率及临床预后的相关因素.方法 选取2014年7月至2019年5月间中国医学科学院北京协和医学院肿瘤医院收治的20例局部复发或晚期脂肪肉瘤患者,回顾性分析表柔比星联合异环磷酰胺方案化疗的有效率及其安全性.结果 20例患者中,完全缓解0例,部分缓解4例(20.0％),稳定9例(45....     

A review of retroperitoneal liposarcoma genomics

Retroperitoneal liposarcomas are rare tumours that carry a poorer prognosis than their extremity counterparts. Within their subtypes – well differentiated (WDL), dedifferentiated (DDL), myxoid (MLS) and pleomorphic (PLS) - they exhibit a diverse genomic landscape. With recent advances in next generation sequencing, the number of studies exploring this have greatly increased. The recent literature has deepened our understanding of the hallmark MDM2/CDK4 amplification in WDL/DDL and addressed concerns about toxicity and resistance when targeting this. The FUS-DDIT3 fusion gene remains the primary focus of interest in MLS with additional potential targets described. Whole genome sequencing has driven identification of novel genes and pathways implicated in WDL/DDL outside of the classic 12q13-15 amplicon. Due to their rarity; anatomical location and histologic subtype are infrequently mentioned when reporting the results of these studies. Reports can include non-adipogenic or extremity tumours, making it difficult to draw specific retroperitoneal conclusions. This narrative review aims to provide a summary of retroperitoneal liposarcoma genomics and the implications for therapeutic targeting.     

Primary mediastinal large B-cell lymphoma mimicking liposarcoma

A 78-year-old man consulted for acute exacerbation of chronic obstructive pulmonary disease (COPD) and an incidental finding of an anterior mediastinal tumor on chest radiograph was noted on admission. Chest computed tomography (CT) revealed a fat-containing mediastinal mass with solid component. Mediastinal liposarcoma was the initial diagnosis based on image characteristics but histopathologic examination of the excised tumor revealed lymphoma infiltration of the mediastinal adipose tissue. To our knowledge, this is the first case report of lymphomatous growth in mediastinal lipomatosis.     

腹膜后巨大脂肪肉瘤1例并文献复习

目的 探讨腹膜后脂肪肉瘤的诊断和治疗,提高少见病例的诊疗水平。方法 回顾江汉大学附属医院收治的1例腹膜后巨大脂肪肉瘤患者的临床资料,结合文献讨论腹膜后脂肪肉瘤的临床特点以及诊断、治疗方法。结果 患者经影像学诊断后行左侧腹膜后肿瘤切除+左肾部分切除术,术后病理提示腹膜后脂肪肉瘤,手术顺利,术后随访20个月,未见肿瘤复发及转移。结论 腹膜后脂肪肉瘤临床少见,无特异性临床表现,CT有助于该病的诊断,手术是首选的治疗方法。     

Impact of primary tumor location on outcome of liposarcoma patients,a retrospective cohort study

BackgroundTumor location as a prognostic factor for patients with liposarcoma (LPS) has been studied modestly with varying outcomes. The aim was to establish the impact of tumor location on recurrence and survival of LPS patients.MethodsA retrospective database of patients treated for LPS until December 2017 was used to assess 5-year local recurrence-free survival (LRFS), distant metastasis-free survival (DMFS) and disease-specific survival (DSS) per tumor location using the Kaplan-Meier method and log-rank test. A multivariable Cox regression analysis was performed to adjust for other prognostic factors.ResultsIn total, 518 patients were identified with a median follow-up of 68 months (interquartile range 31–138). Patients with retroperitoneal/intrathoracic WDLPS or DDLPS (p = 0.014), or testicular WDLPS (p = 0.026) developed a local recurrence more often than patients with other tumor locations. No differences between LPS subtypes and tumor location in the development of metastases (p = 0.600) was observed. Five-year LRFS differed significantly between tumor locations (p < 0.001) as well as 5y-DSS (p < 0.001), but 5y-DMFS did not (p = 0.241), with retroperitoneal/intrathoracic LPS having a worse prognosis. Patients with WDLPS in the extremity, trunk or testicular region did not die of disease, except for the rare occasion of dedifferentiation upon recurrence. After adjustment for other prognostic factors, tumor location was only of prognostic value for DSS (retroperitoneal/intrathoracic vs. extremity: HR 5.08, 95% CI 2.41–10.71, p < 0.001).ConclusionFor all tumor locations, DSS mimicked DMFS except for retroperitoneal/intrathoracic LPS, where DSS mimicked LRFS and where DSS was worse than DMFS. This implies that these patients die of local disease instead of metastatic disease.     

Oncological outcomes after major vascular resections for primary retroperitoneal liposarcoma

BackgroundThe surgical management of retroperitoneal sarcomas frequently involves complex multivisceral resections, however retroperitoneal liposarcoma (LPS) rarely invade major abdominal vessels. The aim of the study was to assess association of major vascular resections with outcome of primary LPS.MethodsAll consecutive patients who underwent resection at our institutions for primary LPS between 2002 and 2019 were included. A propensity matched analysis was performed, adjusting the groups for the variables of Sarculator, to assess the effect of vascular resection on oncological outcomes.ResultsOverall 425 patients were identified. Twenty-four (5%) patients had vascular resection. At final pathology 18 patients had vascular infiltration, 2 vascular encasement and 4 involvement without infiltration. Vascular resection was associated with longer operative time (480′ vs. 330’; p < 0.001) and greater need for transfusions (4 vs. 0 units; p < 0.001), and was burdened by a higher rate of major complications (54% vs. 25%; p = 0.002). After propensity matched analysis, patients undergoing vascular resection had a lower 5-year OS (60% vs. 81%; p = 0.05), and a higher incidence of local and distant recurrence at 5 years (local: 45% vs. 24%, p = 0.05; distant: 20% vs. 0%, p = 0.04).ConclusionsVascular resection is feasible and safe even in the context of multivisceral resection for primary retroperitoneal liposarcomas, although associated to a higher complication rate. However, the independent association between vascular involvement and a higher risk of local recurrence, distant metastases and death may imply a more aggressive biology, which should be factored in the initial management of this complex disease.     

Differential sensitivity of liposarcoma subtypes to chemotherapy

Liposarcoma is one of the most common soft tissue sarcomas and has a number of different subtypes: well-differentiated; dedifferentiated; myxoid/round cell; and pleomorphic. However, the response of these subgroups to chemotherapy is not well documented. In this study, we have conducted a retrospective analysis of a prospectively maintained database of soft tissue sarcoma patients treated at the Royal Marsden Hospital. Eighty-eight liposarcoma patients who received chemotherapy between August 1989 and June 2004 were identified. The response rates to chemotherapy of the different histological subtypes and overall and progression free survival were investigated. Survival according to histological grade was also assessed. A statistically significant higher response rate to first-line chemotherapy was observed in patients with myxoid liposarcoma compared to de- and well-differentiated tumours, 48% (95%CI; 28–69) and 11% (95%CI; 2–29), P = 0.005. Similarly, those with myxoid liposarcoma had a significantly higher response rate compared to all other liposarcoma patients, 48% (95%CI; 28–69) and 18% (95%CI; 8–31). Patients with lower grade tumours had better overall survival. This retrospective analysis suggests that myxoid liposarcoma is relatively chemosensitive in comparison to a combination of other liposarcomas, and in particular de- and well-differentiated tumours. Further confirmation of these results should be sought by similar analyses of other databases.