去分化脂肪肉瘤28例临床病理分析

目的 探讨去分化脂肪肉瘤(dedifferentiated liposarcoma,DL)中去分化成分的形态学特征.方法 用常规HE染色和免疫组织化学方法,对28例DL进行观察分析.结果 在28例DL中,25例由非典型脂肪瘤样肿瘤/高分化脂肪肉瘤和非脂肪性梭形细胞肉瘤组成;1例为黏液样脂肪肉瘤和非脂肪性梭形细胞肉瘤组成;2例复发性病例未见到高分化脂肪肉瘤成分,均为非脂肪性梭形细胞肉瘤成分.免疫组化:28例中有14例行免疫组化染色,脂肪肉瘤区域脂母细胞S-100蛋白(+),部分脂肪肉瘤中的梭形细胞CD34(+).14例DL中去分化成分3例SMA和HHF35(+),1例desmin和HHF35(+),CD34、CD117、S-100、CD99、AACT、HMB-45、CK、CR均(-),CD68部分病例散在(+).通过对DL的形态学观察发现,去分化区域可以单独或混合呈现以下形态结构:(1)多形性恶性纤维组织细胞瘤样,(2)纤维肉瘤样,(3)低度恶性黏液纤维肉瘤样,(4)纤维瘤病样,(5)平滑肌肉瘤样,(6)脑膜瘤样漩涡结构,(7)横纹肌肉瘤分化,(8)骨/软骨分化,(9)炎性肌纤维母细胞瘤样,(10)血管外皮瘤样等.其中炎性肌纤维母细胞瘤样和血管外皮瘤样结构文献中尚未见报道.结论 DL中去分化成分最常见的结构是高级别肉瘤形态,但也可以是低度恶性黏液纤维肉瘤样、纤维瘤病样、炎性肌纤维母细胞瘤样、血管外皮瘤样等低级别肉瘤形态.可以是单一分化,也可以向平滑肌、横纹肌、骨/软骨等异源性分化.     

幼女双乳脂肪肉瘤1例

幼女双乳脂肪肉瘤１例马振斌孙华患者女，１１岁，发现两侧乳腺无痛性肿块呈进行性肿大１个月。查体：双乳开始发育，对称，乳头正常。在右乳内下象限扪及约枣粒大小肿块，左乳外下象限扪及约核桃大小肿块，肿块质均较硬，可活动，境界清楚，呈结节状，表面肤色正常。全身...     

87例脂肪肉瘤的临床病理学研究：—附17例去分化脂肪肉瘤

87例脂肪肉瘤,40岁以上占70%,发生于深部软组织,以下肢和腹膜后多见。组织学类型分为粘液型、圆细胞型、多形性和分化良好型(包括脂肪瘤样型和硬化型),以分化良好型最多见(54%)。局部切除常有复发,但很少转移。其中17例发生去分化,占总数的20%。向恶性纤维组织细胞瘤分化者最多(47%)。本文还对本病的诊断、鉴别诊断和组织发生进行了探讨。     

阴囊高分化脂肪肉瘤误诊为黏液性脂肪肉瘤1例

患者男性,63岁,因右下腹痛2天余,以急性阑尾炎收治入院。阑尾炎术后发现患者左侧腹股沟区有一肿块,患者自述肿块存在2年余。查体：站立位,左侧腹股沟区可见一局限性隆起型肿块,进入阴囊,12 cm ×9 cm大小,质地中等偏硬,无压痛,平卧后肿块不消失,不能回纳,透光试验阴性。遂行左侧腹股沟斜疝无张力修补术+左侧阴囊内肿块切除术,术中见阴囊内肿块呈实性,质偏硬,从左侧阴囊底部向内上方将肿块推出左侧腹股沟区切口,完整切除肿块。术后行核磁共振检察,腹膜后未见占位性改变。     

5例子宫Mullerian腺肉瘤形态学观察

通过光镜、电镜、组化及免疫组化等方法观察5例子宫Müllerian腺肉瘤之形态学特点。其腺体衬以Müllerian管多型良性上皮,而其肉瘤部分既可是高分化,亦可为低分化之多形性。特染及免疫组化染色证明其肉瘤部分是内膜间质肉瘤、纤维肉瘤、少量平滑肌及粘液肉瘤,未见确切横纹肌及软骨成分。据光、电镜特点支持本瘤起源于多潜能的内膜间充质细胞,对瘤巨细胞和透明球的性质进行简要讨论。     

38例脂肪肉瘤病理分析

本文报告资料完整的脂肪肉瘤38例,男性23倒(60%),女性15例(40%),男女之比为1.5:1。年龄1～73岁,就诊时的平均年龄为43.6岁,较文献报告偏低;41～60岁者多见。下肢和腹膜后占60%。根据Enzinger的标准进行组织学分型,其中分化良好型14例(36.8%)、粘液样型13例(34.2%),圆形细胞型4例(10.4%),多形细胞型7例(18.6%)。讨论了分型、部位、年龄与预后之间的关系以及鉴别诊断问题。     

9例脂肪肉瘤的超微结构分析

用光镜、电镜及免疫组织化学方法观察９例脂肪肉瘤，提示组成脂肪肉瘤的细胞在超微结构上与发育中的脂肪组织细胞十分相似，包括原始间叶细胞，纤维母细胞，早、中、晚三期指母细胞及成熟的脂肪细胞，但也有差别。不同类型的脂肪肉瘤主要由不同分化程期的脂母细胞组成。此外，在电镜观察下发现有由原始间叶细胞及纤母细胞向脂母细胞的过渡形态，说明脂母细胞可能起源于这两种细胞。我们还讨论了瘤细胞内脂质形成及脂肪肉瘤与其他肿瘤     

乳腺脂肪肉瘤2例

乳腺脂肪肉瘤２例１雷承宝２王万德例１女性，４３岁。出现左乳肿物１个月。左乳肿块约蚕豆大，生长迅速，伴疼痛，与月经无关。检查：双乳对称，乳头无凹陷及溢液。左乳内外上象限近乳晕处可触及一３ｃｍ×３．０ｃｍ圆形肿物，质硬，压痛明显，边界清楚，活动良好，右乳...     

子宫伴有脂肪肉瘤及巨细胞分化的腺肉瘤一例

患者女，５２岁。５０岁闭经，白带增多并带血性水样物流出１年余。近日加重流出息肉样物２小时于１９９７年７月１日入院。妇科检查：阴道畅通、宫颈口有１．５ｃｍ息肉状物突出。宫体前位，如妊娠７周大小，压痛明显，活动度好，质地中等，未触及附件包块。经抗类治疗息...     

骨内脂肪肉瘤1例

患者,男,19岁,1个月前无明显诱因出现左胫骨近端内侧疼痛肿胀,轻微局部隆起,运动时加重,休息后可缓解,于当地医院按软组织发炎,给于口服药物、外用药等治疗,症状未见明显好转,后在当地医院行X线检查发现左胫骨近端内侧骨质破坏,转入我院治疗.查体:左下肢无畸形,运动感觉未见明显异常,左侧股部肌肉萎缩,左侧周经较右侧周经少约2 cm,左胫骨近端内侧局部隆起,可触及一个大小约4 cm×3 cm×3 cm肿物,质硬,不动,边界不清,与周围组织粘连,局部皮温较高,未见明显静脉怒张,压痛,叩击痛明显.于2008年10月24日行段切人工关节假体置换术.     

Myxoid Liposarcoma: Study of Ten Cases

The ultrastructure of 10 myxoid liposarcomas from 9 patients is presented. Features closely resembling those of developing fetal adipose tissue were seen in all tumors. The various cell types identified ranged from undifferentiated cells to typical multivacuolated and univacuolated lipoblasts. In addition, a secretory-type mesenchymal cell with distended cisternae of rough endoplasmic reticulum was found in every case as well as intermediate cells with secretory product and lipid accumulation. Relative proportions of these types varied from case to case.

A prominent feature in all cases was the particular spatial relationship of the abundant capillaries to the various cell types. The undifferentiated cells had perivascular processes with a tendency to envelop the primitive capillaries. These cells progressively enlarged and acquired features of the differentiated cell types as they separated from the lamina. Mitotic activity, when detectable, was prominent in the capillary wall and its vicinity. Lipoblasts resembling those of brown adipose tissue frequently coexisted in the same tumor with others analogous to those of white adipose tissue.

Our findings suggest that the tumor cells in lipo-sarcoma originate from a dividing population of perivascular mesenchymal cells, in a manner akin to that of developing white adipose tissue.     

Myxoid Liposarcoma: Study of Ten Cases

The ultrastructure of 10 myxoid liposarcomas from 9 patients is presented. Features closely resembling those of developing fetal adipose tissue were seen in all tumors. The various cell types identified ranged from undifferentiated cells to typical multivacuolated and univacuolated lipoblasts. In addition, a secretory-type mesenchymal cell with distended cisternae of rough endoplasmic reticulum was found in every case as well as intermediate cells with secretory product and lipid accumulation. Relative proportions of these types varied from case to case.

A prominent feature in all cases was the particular spatial relationship of the abundant capillaries to the various cell types. The undifferentiated cells had perivascular processes with a tendency to envelop the primitive capillaries. These cells progressively enlarged and acquired features of the differentiated cell types as they separated from the lamina. Mitotic activity, when detectable, was prominent in the capillary wall and its vicinity. Lipoblasts resembling those of brown adipose tissue frequently coexisted in the same tumor with others analogous to those of white adipose tissue.

Our findings suggest that the tumor cells in lipo-sarcoma originate from a dividing population of perivascular mesenchymal cells, in a manner akin to that of developing white adipose tissue.     

电镜在脂肪肉瘤诊断中的意义

从2183例电镜诊断病例中收集最后电镜观察明确为脂肪肉瘤的16例进行回顾性分析,重点对比光镜和电镜的观察后发现,其中分化好的脂肪肉瘤2例及多形性脂肪肉瘤3例无论光镜及电镜诊断上都不困难。粘液型脂肪肉瘤5例,如果抓住丰富的分枝状小血管及找到脂肪母细胞,光镜诊断也不困难,但对于梭形细胞的3例和圆形细胞的3例,光镜下要肯定诊断较困难,但电镜下则可见到瘤细胞内脂滴、细胞膜外的基板及找到吞饮小泡的特点,有助于脂肪肉瘤的诊断。     

Surgical Treatment of Retroperitoneal Liposarcoma

Background

Retroperitoneal liposarcoma (RL) is a relatively rare tumor and is usually found at the advanced stage. Chemotherapy or radiotherapy for this tumor is not yet defined, and if operable, surgery is the treatment of choice. Complete resection of tumor with wide margins including excision of other organs has been recommended. However, many patients suffer from deterioration of the quality of a postoperative life. In the present study, we retrospectively analyzed the ideal surgical procedures for treating RL.

Methods

RL patients treated at our institute between 2003 and 2013 amounted to 10. RL was primary in 5 patients and recurrent in the rest 5. We analyzed cases of the 10 patients retrospectively.

Results

Tumor resection was performed for 9 patients, 7 of whom underwent complete tumor resection. RL was well-differentiated in 6 patients and dedifferentiated in 4. We analyzed the overall survival of 10 patients, and the relapse free survival of the operated 9 patients. Patients with well-differentiated RL showed better survival than those with dedifferentiated RL. Even the recurrent RL was huge, complete tumor resection could be performed in the well-differentiated type, but it was difficult in the dedifferentiated type.

Conclusion

In the recurrent huge RL, the chance of a margin-negative resection remains low, but surgery remains the treatment of choice. Tumor resection with preserving important organs may improve patients’ quality of postoperative life and survival.     

Liposarcoma of the larynx: report of a case and review of literature

Liposarcomas of the larynx are extremely rare tumors, with only 37 cases reported in the English or French literature to date. The first two cases of laryngeal liposarcomas were reported respectively by Kapur and Dockerty in 1968 [1, 2]. Liposarcoma of the larynx is at high risk of local recurrence and seldom has metastatic potential. Prognosis for this tumor is better than that of non laryngeal liposarcoma. The present case is the first patient of primary liposarcoma of the larynx reported from China. A review of the literature was performed, and the presentation, position, pathological diagnosis, treatment and prognosis of the patients with liposarcoma of the larynx of the reported cases before are analyzed.     

c‐Jun promotes cell migration and drives expression of the motility factor ENPP2 in soft tissue sarcomas

Genomic amplification of the c‐Jun proto‐oncogene has been identified in ～30% of dedifferentiated liposarcomas (DDLPS), but the functional contribution of c‐Jun to the progression of DDLPS remains poorly understood. In previous work we showed that knock‐down of c‐Jun by RNA interference impaired the in vitro proliferation and in vivo growth of a DDLPS cell line (LP6) with genomic amplification of the c‐Jun locus. Here, we used gene expression analysis and functional studies in a broad panel of cell lines to further define the role of c‐Jun in DDLPS and other soft tissue sarcomas. We show that c‐Jun knock‐down impairs transition through the G₁ phase of the cell cycle in multiple DDLPS cell lines. We also found that high levels of c‐Jun expression are both necessary and sufficient to promote DDLPS cell migration and invasion in vitro. Our data suggest that high levels of c‐Jun enhance motility in part by driving the expression of ENPP2/Autotaxin. c‐Jun over‐expression has minimal effects on in vitro proliferation but substantially enhances the in vivo growth of weakly tumourigenic DDLPS cell lines. Finally, we provide evidence that c‐Jun genomic amplification and over‐expression may have similar functional consequences in other types of soft tissue sarcoma. Our data suggest a model in which relatively low levels of c‐Jun are sufficient for in vitro proliferation, but high levels of c‐Jun enhance invasiveness and capacity for in vivo tumour growth. These observations provide an explanation for the selective advantage provided by c‐Jun genomic amplification in vivo and suggest that sarcomas with elevated c‐Jun levels are likely to have a particularly high malignant potential. Data from exon array and RNA‐Seq experiments have been deposited in the GEO database (Accession No. GSE57531). Copyright © 2014 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd     

去分化脂肪肉瘤潜在核心基因的生物信息学预测及分析

目的 探讨去分化脂肪肉瘤的潜在核心基因在其恶性生物学行为中的作用.方法 获取基因表达数据库(gene expres-sion omnibus,GEO)数据库中GSE21122和GSE52390的芯片数据,通过GEO2R筛选差异表达基因,对差异表达基因进行GO功能、KEGG通路富集分析和蛋白互作分析,并用Cytoscap...     

bcl-2和p53表达在结直肠癌预后评估中的价值

目的　探讨bcl 2和 p5 3表达水平与结直肠癌肿瘤生物学特征及其预后的关系。 方法　应用免疫组化S P法 ,检测93例结直肠癌标本中bcl 2与p5 3蛋白的表达 ,并与临床病理特征进行相关分析。结果　bcl 2蛋白在结直肠癌的阳性表达率为 5 7 0 % (5 3/ 93) ,与淋巴结转移呈负相关 (P <0 0 1) ,p5 3表达阳性率为 4 3% (4 0 / 93) ,与淋巴结转移无相关 (P >0 0 5 ) ,但p5 3表达阳性患者的预后差于 p5 3阴性组 (P =0 0 1)。当分析bcl 2和 p5 3联合表达时 ,Dukes分期及淋巴结转移各组间有差异 (P <0 0 5 )。其中bcl 2 (+) / p5 3(- )表达形式多见于DukesA和B期肿瘤 (4 1 0 % ,2 3/ 5 6 )。经Cox模型多因素分析结果表明 ,淋巴结转移 (P <0 0 1)和 p5 3表达 (P <0 0 1)是结直肠癌独立预后影响因素。其他指标 ,包括bcl 2和 p5 3联合表达情况等均未显示出统计学意义。结论　bcl 2和p5 3表达水平可提示结直肠癌的生物学行为 ,但bcl 2的表达与淋巴结转移状况相关不是一个单独作用的预后指标 ,而 p5 3虽与其它生物学特性关系不大 ,却是一个相对独立的结直肠癌预后指标。     

Malignant Fibrous Histiocytoma of Soft Parts: An Ultrastructural Quantitative Study

Thirty-two cases of soft tissue malignant fibrous histiocytoma (MFH), including 24 of common storiform-pleomorphic type, 7 of myxoid type, and 1 of inflammatory type, were analyzed in an ultrastructural quantitative study, the objective being to clarify the cellular composition and distribution of the tumor. Approximately 100 unselected cells in each tumor were classified into seven types as follows: undifferentiated (10%-62%, m: 29%), histiocytelike (8%-69%, m: 37%), fibroblastlike (0.9%-26%, m: 11%), myofibroblasts (0%-22%, m: 3%), intermediate between histiocytelike and fibroblastlike cells (1%-41%, m: 15%), multinucleated giant (0%-1%), and xanthomatous cells (0%-1%). The undifferentiated, histiocytelike, fibroblastlike, and intermediate cells constituted the principal types, invariably present, although in varying proportions in each tumor. No statistical difference in ultrastructural cellular composition was evident in different portions of the same tumor, and such was also the case between the two main subtypes of MFH, common and myxoid. Naphthyl thiol acetate (NTA) esterase was demonstrated in fibroblastlike cells, using the ultrastructural cytochemical technique, in both cases examined. The possibility that MFH may be of alternative undifferentiated mesenchymal cell origin has to be considered.