孤立性纤维瘤

1　临床资料患者,男,36岁。1年前无明显诱因出现左腰背部酸痛,呈非持续性隐痛,疼痛无明显规律。同时出现大便次数的减少,由每日1次改为三四天1次,但大便性状无明显改变。Ｂ超提示:“下腹部实质性不均质占位”,ＣＴ示:“下腹部、膀胱上方有一个81ｃｍ×89ｃｍ×10ｃｍ巨大肿块,考虑为肌肉或神经源性”。专科检查:腹平软,未及肠型及蠕动波,脐下5ｃｍ处可及10ｃｍ×8ｃｍ的肿块,质偏硬,可活动,边界清,未及明显压痛、反跳痛及肌卫,肝脾肋下未及,移动性浊音(-),肠鸣音3次/分,肛检(-)。术中见:盆腔肿块,生长于结肠系膜,约12ｃｍ×10ｃｍ×8ｃｍ大…     

胸膜孤立性纤维瘤的形态学特征分析

目的 探讨胸膜孤立性纤维瘤(SFTP)患者的临床影像学表现及病理特征.方法 采用2004年1月至2010年12月在我院就诊的30例SFTP患者的临床资料,总结SFTP的临床影像学表现及主要的病理特征.结果 30例SFTP患者平均年龄53岁(28～77岁),影像学表现以胸腔及纵膈内肿块为主要表现(63.3%,19/30...     

肾脏孤立性纤维瘤一例

患者女 ,33岁。 2个月前无明显诱因出现左腰部疼痛 ,呈持续性隐痛 ,无阵发性加剧 ,无尿频、尿急、尿痛 ,无血尿。ＭＲＩ:右肾盂占位性病变 ,考虑为乳头状瘤或乳头状癌 ,合并肾上盏积水。专科检查 :双侧肾区无隆起 ,未扪及包块 ,无触压痛。术中见右肾盂肿瘤 2ｃｍ× 2ｃｍ× 2ｃｍ ,境界较清 ;肾上盏扩张积水 ,未压迫肾动静脉。病理检查 :右肾 1个 ,大小为 11ｃｍ× 5ｃｍ× 4ｃｍ ,肾门正中见一 2ｃｍ× 2ｃｍ× 2ｃｍ灰白色结节 ,界清 ,压迫肾盂 ,肿瘤部分为肾盂包绕 ,部分与肾实质粘连 ,切面灰白 ,编织状 ,质韧。镜下观察 :肿瘤组织由成纤…     

胸膜外孤立性纤维瘤的诊断及鉴别诊断

胸膜外孤立性纤维瘤 (ｅｘｔｒａｐｌｅｕｒａｌｓｏｌｉｔａｒｙｆｉｂｒｏｕｓｔｕｍｏｒ ,Ｅ ＳＦＴ)是一种少见的梭形细胞软组织肿瘤 ,因其形态学变异大 ,并且发生部位广泛 (几乎囊括躯体所有的解剖部位 ) ,在诊断或鉴别诊断时往往不易想到。笔者通过简要介绍胸膜外孤立性纤维瘤的临床病理学特征 ,重点描述其形态学变化 ,以期对该肿瘤的诊断及鉴别诊断有所帮助。1　组织学起源ＳＦＴ最初由Ｋｌｅｍｐｅｒｅｒ和Ｒａｂｉｎ于 1931年报道〔1〕,主要发生在脏层胸膜 ,表现为与胸膜相连、界限清楚的孤立性肿块 ,常带蒂。ＳＦＴ的同义名称包括…     

肝脏巨大孤立性纤维瘤

患者男性 ,38岁 ,因右上腹持续性隐痛 3月余 ,于 1995年 5月入本院。Ｂ超发现肝内巨大回声增强肿块 ,提示肝内巨大血管瘤 ,行“肝动脉结扎介入”治疗 ,未做活检。术后半年复查Ｂ超 ,示右肝后叶 6 9ｃｍ× 5 6ｃｍ肿块 ,肿块进行性增大。 1998年 3月ＣＴ检查 ,示肝脏肿块大小 12ｃｍ× 12ｃｍ ,并出现腹胀、腰酸及下坠感 ,于 2 0 0 0年 6月再次入院治疗 ,术中右肝叶见一巨大肿瘤 ,行右半肝叶切除术。病理检查　眼观 :肿瘤分叶状 ,大小 4 5ｃｍ× 2 5ｃｍ× 15ｃｍ ,重 910 0ｇ表示光滑 ,与肝组织分界清楚 ,切面灰黄 ,灰白 ,质中 ,小区囊性…     

胸膜外孤立性纤维瘤39例临床病理分析

目的探讨胸膜外孤立性纤维性肿瘤的临床表现、病理形态学、免疫组化及生物学行为。方法收集39例胸膜外孤立性纤维性肿瘤,观察其临床病理特点并进行免疫组化分析。结果男性14例,女性25例,年龄15～81岁,平均41.2岁。肿瘤发生部位为软组织15例,眼眶8例,腹膜后4例,盆腔3例,颅内4例,肾脏2例,膀胱1例,肺1例,鼻腔1例。光镜下形态多变,由富含胶原的细胞稀少的纤维样区至富于细胞区,分支状的血管外皮瘤样结构;可见黏液变、脂肪细胞形成。肿瘤共分为四型,即为纤维型、细胞型、脂肪形成型、富于巨细胞型。免疫表型:几乎所有肿瘤vimentin、CD34呈强阳性、弥漫表达,CD99(20/24)、bcl-2(18/24)呈阳性表达。结论孤立性纤维性肿瘤可发生在全身各部位。需与其他多种梭形细胞肿瘤进行鉴别诊断,其组织学特点及免疫组织化学染色可以帮助鉴别。     

泌尿生殖系统孤立性纤维瘤的临床特征和治疗策略

目的分析、总结并探究泌尿生殖系统孤立性纤维瘤(SFT)的诊断方法及治疗策略。方法报道1例男性泌尿生殖系统SFT病例,并总结该患者的诊治过程。回顾2008年至2018年国内外报道的总计35例患者的人口学特征、临床和病理特征、治疗及随访情况。结果 35例患者中有24例(68.6%)具有相关泌尿系统症状,而11例(31.4%)在确诊时无明显不适主诉。在病理结果方面,34例(97.1%)患者的手术病理标本表现为CD34~+,此外27例(77.1%)的手术病理标本表现为Bcl-2~+。结论磁共振成像(MRI)和计算机断层扫描(CT)是检查SFTs的最有效影像学方法;手术病理标本的免疫组化是明确诊断的关键,尤其是CD34、Bcl-2、STAT6、ALDH2的特征性染色对明确诊断有重要价值;此外,分子病理学检测NAB2-STAT6融合蛋白的存在亦可提示SFTs的诊断。手术切除可能是SFTs最好的治疗方法。     

自身免疫性胰腺炎的临床及影像学表现分析

目的：探讨自身免疫性胰腺炎的临床及CT、MRI表现。方法回顾性分析12例AIP患者的临床及影像学资料。结果12例患者实验室检查均有IgG4增高。8例表现为胰腺弥漫性肿大,呈"腊肠样",2例表现为体尾部局限性肿大,1例表现为胰头部局限性肿大。12例CT均表现为密度均匀减低,增强后强化减低,7例在MRI上表现为T1WI信号减低T2WI信号增高,增强后低强化。8例周围有假包膜样结构,6例合并肝内外胆管扩张。结论AIP影像学具有一定的特征性,影像学检查在AIP的诊断中起着重要的作用,结合临床特点有助于明确诊断,减少误诊。     

CT动态增强扫描对孤立性肺结节病灶的临床价值

目的 评价CT增强特性对孤立性肺结节病灶(SPN)性质的鉴别诊断价值.方法 对经手术、病理证实的孤立性肺结节病患者76例,行螺旋CT动态增强扫描,分析动态增强时间-密度曲线.结果 恶性结节的强化性质(18～68HU)明显高于良性结节(5～45HU),炎性结节呈明显强化(范围20～78HU),恶生结节动态增强时间-密度曲线呈缓慢上升型,炎性结节呈持续上升型,其他良性结节为平坦型.结论 CT动态增强扫描对孤立性肺结节性病灶的诊断和鉴别诊断有较高临床价值.     

儿童非骨化性纤维瘤X线——组织病理学

目的：提高对儿童非骨化性纤维瘤的临床病理学认识，以能及时诊治。方法：分析了5例儿童非骨化性纤维瘤临床特点。诊疗途径和组织学特征。结果：5例均发生于长骨，手术病理证实非骨化性纤维瘤。随访最长5年7月，无一例复发。结论：本病发病率极低，易误诊，X线及病理均有特征性改变，手术预后良好。应与纤维性皮质骨缺损、骨囊肿等相 鉴别。     

Mediastinal epithelioid solitary fibrous tumor

We describe the case of a 74-year-old man with a mediastinal tumor composed predominantly of epithelioid cells exhibiting histopathologic and immunohistochemical features intermediate between those of a solitary fibrous tumor and those of a cellular adenomatoid tumor. We discuss the differential diagnosis and possible histogenesis of this unusual neoplasm, and we propose the term epithelioid solitary fibrous tumor for this entity.     

伴有巨细胞的血管外皮细胞瘤/孤立性纤维性肿瘤的临床病理观察

目的 探讨伴有巨细胞的血管外皮细胞瘤/孤立性纤维性肿瘤的病理学特点、诊断及鉴别诊断要点.方法 采用HE及免疫组织化学(EnVision法)方法 ,对7例分别发生于眼眶及眶外的伴有巨细胞的血管外皮细胞瘤/孤立性纤维性肿瘤进行病理学分析.结果 2例为发生于眼眶的肿瘤,其中1例为复发病例;5例为眶外肿瘤.镜下特征:肿瘤境界清楚,由无明显异型性的圆形或梭形的肿瘤细胞与胶原混杂组成,富含血管,部分区域形成假血管腔隙样结构,多核巨细胞衬于腔隙的内壁或散布于间质中.免疫组织化学标记显示肿瘤细胞和巨细胞表达CD34.7例均行肿块切除术,术后随访4例,均无复发.结论 伴有巨细胞的血管外皮细胞瘤/孤立性纤维性肿瘤是一种眼眶和眶外组织的中间型软组织肿瘤,局部切除多可治愈,组织学上需与巨细胞纤维母细胞瘤、软组织多形性透明变性血管扩张性肿瘤、血管瘤样型纤维组织细胞瘤相鉴别.     

Immunohistochemical comparison of gastrointestinal stromal tumor and solitary fibrous tumor

CONTEXT: The differential diagnosis of gastrointestinal stromal tumors (GIST) and solitary fibrous tumors (SFT) may be a diagnostic challenging because of overlapping clinicopathologic features. Many studies have shown consistent immunoreactivity for CD117 (c-Kit) in GIST. However, only a few studies have evaluated CD117 expression in SFT, and these studies have used an antibody from Santa Cruz Biotechnology. In non-GIST lesions, reactivity with this antibody has been shown to differ from that with a CD117 antibody from Dako Corporation. The immunoreactivity of SFT with the Dako CD117 antibody has not been reported. Conversely, CD99 is a marker for SFT, and its expression in GIST has not been evaluated. OBJECTIVE: To study the immunohistochemical profiles of GIST and SFT to evaluate their diagnostic overlap. DESIGN: We studied the immunoreactivity of 27 unequivocal GIST and 19 unequivocal extra-abdominal SFT for CD117, CD34, CD99, alpha-smooth muscle actin, vimentin, CD31, S100 protein, and muscle-specific actin. All antibodies, including CD117, were from Dako Corporation. RESULTS: We found positive immunoreactivity for CD117 in 100% of GIST and none of SFT; for CD34 in 89% of GIST, and 100% of SFT; for CD99 in 89% of GIST and 100% of SFT; for alpha-smooth muscle actin in 48% of GIST and 31% of SFT; for vimentin in 89% of GIST and 90% of SFT; and for muscle-specific actin in 22% of GIST and none of SFT. None of the GIST or SFT showed immunoreactivity for CD31 and S100 protein. CONCLUSIONS: The major difference between GIST and SFT was strong CD117 immunoexpression in all GIST and an absence of this expression in all SFT. With the exception of muscle-specific actin, the prevalence of immunoreactivity for the markers studied did not differ substantially between these 2 tumors. We conclude that GIST and SFT show distinctly divergent immunoprofiles with respect to CD117 and muscle-specific actin.     

Malignant solitary fibrous tumor of the meninges

Increasing numbers of solitary fibrous tumors (SFTs) in the meninges have been reported since this entity was first recognized. While most cases previously reported were considered to be benign, the malignant potential of extrathoracic SFTs has not been excluded. The authors report a rare case of a meningeal SFT with malignant behavior occurring in a Japanese female patient, initially resected when she was 44 years old and recurring in the same place four times during a 26-year follow-up period. A metastatic tumor to the right lung arose 25 years after the resection of the first meningeal tumor and focal invasion into the cerebellum was also observed with her last (5th) meningeal tumor. Immunohistochemical analysis showed all tumors to be diffusely positive for CD34 and negative for EMA, with a so-called patternless histological pattern, featuring thin collagen fibers between tumor cells. A focal staghorn vascular pattern was also observed. Ki67 (MIB-1) labeling indices and mitosis rates were 3.1±1.2% and less than 1/10 high power fields (HPF) in the first meningeal tumor and 16.1±6.4% and 6/10HPF in the last (5th) one, respectively. Thus, the present case suggests that meningeal SFTs possess malignant potential so that careful long-term follow up is required.     

恶性孤立性纤维性肿瘤2例临床病理分析

目的:探讨恶性孤立性纤维性肿瘤(malignant solitary fibrous tumor,MSFT)的临床病理特征、诊断、鉴别诊断、分子遗传学、治疗及预后.方法:对2例MSFT进行光镜、免疫组织化学研究,并复习相关文献.结果:男性2例,年龄分别为66岁及48岁.发生部位:骶前1例,临床表现为进行性二便障碍3年;后上纵隔1例,临床表现为活动后心慌半个月.肿瘤大小分别为10cm×9 cm×6 cm及12 cm×10 cm×9 cm.镜检:细胞稀少区和细胞丰富区交替分布,两者之间有粗的玻璃样变胶原纤维分隔.密集区肿瘤细胞丰富,呈束状、漩涡状排列,可见血管外皮瘤样结构;瘤细胞呈梭形、短梭形,核染色质粗,异型性明显,有肿瘤性坏死、较多量核分裂( ＞4/10 HPF),局部出血.免疫表型:肿瘤细胞CD34,Bcl-2,CD99及Vimentin弥漫阳性;P53部分阳性,上皮细胞膜抗原局灶阳性;ki-67增殖指数20％ ～ 30％;S100,Desmin,CK及胶质纤维酸性蛋白阴性.结论:MSFT是一种少见的间叶肿瘤,确诊主要依靠形态学及免疫组织化学,需与血管外皮细胞瘤、恶性纤维组织细胞瘤、纤维肉瘤、滑膜肉瘤、恶性间皮瘤、恶性外周神经鞘膜瘤和脑膜瘤等相鉴别.MSFT的预后不良,易复发和转移,根治性切除是主要治疗手段,术后应长期随访.     

Heterologous osteosarcomatous and rhabdomyosarcomatous elements in dedifferentiated solitary fibrous tumor: further support for the concept of dedifferentiation in solitary fibrous tumor

Dedifferentiation within solitary fibrous tumor is a rare and only recently characterized phenomenon. It differs from malignant solitary fibrous tumor in that there is abrupt transition between classical solitary fibrous tumor and the dedifferentiated component. The latter is a high-grade sarcoma, which can exhibit a number of morphologies, but heterologous differentiation is exceptionally rare. We report a case of dedifferentiated solitary fibrous tumor, with heterologous osteosarcomatous and rhabdomyosarcomatous elements, arising in the deep soft tissue of the thigh of a 59-year-old man. This comprised morphologically and immunohistochemically typical solitary fibrous tumor, juxtaposed to pleomorphic, high-grade malignant neoplasm of 2 distinct lineages. The sharp demarcation between well-differentiated and dedifferentiated components is typical of the dedifferentiation seen in other mesenchymal neoplasms. This expands the range of histopathology of this rare, newly characterized type of malignant progression in solitary fibrous tumor.     

孤立性纤维性肿瘤临床病理学观察

目的探讨孤立性纤维性肿瘤(solitary fibrous tumor,SFT)的临床、病理组织学、免疫组化特征,提高对该病的认识。方法对7例SFT病例进行大体观察、病理组织学及免疫组化En V ision二步法标记,观察V im、CD34、CD99、bc l-2 Des、SMA、HMBE-1、CK、S-100蛋白在SFT组织中的表达。结果7例SFT分别发生于胸腔、腹腔、纵隔、眼眶及胸壁软组织,临床表现为肿块压迫所致的症状,肿块1.5 cm×1.5 cm×1 cm~20 cm×20 cm×18 cm,光镜下肿瘤细胞由长梭形细胞构成束状、编织状排列,间质内有多少不一的胶原纤维,无特殊的组织构型及形态的多样性为其组织学特点。免疫表型:CD34、V im、CD99均为弥漫强阳性,bc l-2 5例阳性,Des、SMA、HMBE-1、CK、S-100蛋白阴性。结论SFT是一组形态多样、无特殊组织构型及有多少不一胶原纤维的梭形细胞肿瘤,其特殊的免疫表型,有助于对该病的诊断,但SFT的确诊必须结合组织形态和免疫组化共同完成。SFT的生物学行为取决于其组织学形态和肿块的大小及生长方式,完全切除肿块者预后较好。