孤立性纤维性肿瘤

孤立性纤维性肿瘤 (ｓｏｌｉｔａｒｙｆｉｂｒｏｕｓｔｕｍｏｒ ,ＳＦＴ)是一种罕见肿瘤 ,又因组织学的复杂性、多样性 ,较易误诊。笔者就我院 2例ＳＦＴ结合文献对其组织学、免疫组化和电镜诊断及鉴别诊断等进行讨论。1　临床资料例 1,患者女性 ,6 1岁。体检发现左下肺阴影 2天 ,无明显不适。ＣＴ示左下肺后基底段球形阴影 ,边缘较规整 ;术前诊断错构瘤或隔离肺。术中见肿瘤位于左下肺基底段紧靠肺膜 ,与周围组织分界清 ,极易剥离 ,遂行单纯切除。术后已5年 ,无复发或转移。例 2 ,患者男性 ,45岁。体检发现右腹股沟肿物 2周。查体 :右腹股…     

眼眶孤立性纤维性肿瘤

孤立性纤维性肿瘤 (ｓｏｌｉｔａｒｙｆｉｂｒｏｕｓｔｕｍｏｒ ,ＳＦＴ)主要发生在脏层胸膜 ,随着对其认识的不断深入 ,胸膜外病例报道不断增多 ,眼眶是胸膜外常见部位之一 ,由于其病理形态的多样性等 ,易误诊为恶性肿瘤。现作者就我院 1例眼眶ＳＦＴ结合文献对其临床病理特征、诊断及鉴别诊断进行讨论。1　临床资料患者 ,男性 ,5 5岁。发现左眼眶肿块 2年伴左眼球突出1年入院。ＣＴ :左眼眶内肿瘤侵及上直肌 ,瘤体与眼球边界模糊。手术中见肿瘤位于上直肌间隙 ,肿块与周围组织分界尚清楚 ,表面血供较丰富。2　病理检查2 .1　眼观　灰白、…     

13例孤立性纤维性肿瘤临床病理分析

目的探讨孤立性纤维性肿瘤（SFT）的临床病理形态学和免疫组织化学特点及诊断要点。方法采用光镜及免疫组化对13例SFT进行研究。结果本组病例，男性9例，女性4例，发病年龄30-76岁，平均46．76岁，良性8例，恶性5例，发生于9个部位，其中胸腔2例，肺2例，腹膜后3例，左大腿肌间1例，髂骨1例，肩背部皮下1例，腋窝1例，鼻腔1例，膀胱1例。临床主要表现为局部肿块及其引起的压迫症状。主要病理学改变：肿唐直径2-16cm，平均5．6cm，大部份边界清楚。镜下：瘤组织由梭形、短梭形、圆形细胞构成，细胞稀疏区和密集区交替分布，薄壁血管较多，细胞间富有粗细不等的胶原纤维。免疫组化结果：Vimentin阳性率100％（13／13），CD99阳性率92.3％（12／13），Bcl-2阳性率92．3％（12／13），CD34阳性率69.2％（9／13），S10014．3％（1／7），EMA阳性率28．6％（2／7），Ki67阳性率2-40％。结论SFT大部份为良性，诊断主要靠形态学及免疫组织化学，可见于全身多个部位，根治性切除是主要治疗手段。     

泌尿生殖系统孤立性纤维性肿瘤5例临床病理分析

目的 探讨泌尿生殖系统孤立性纤维性肿瘤(solitary fibrous tumor, SFT)的临床病理学特征。方法 回顾性分析5例SFT的临床病理资料,行HE和免疫组化染色,并复习相关文献。结果 5例SFT中男性3例,女性2例,年龄46～68岁,肿瘤发生部位：右肾2例,前列腺2例,膀胱1例。患者无特异性临床表现,仅出现腰背部疼痛不适、酸胀、血尿或排尿困难等症状。眼观：肿瘤为椭圆形或圆形肿块,边界清楚,最大径2～9 cm,平均4.94 cm。镜检：瘤细胞梭形、核圆形或卵圆形,呈短束状、席纹状及不规则漩涡状排列,表现为细胞丰富区和稀疏区交替分布,之间有胶原带分隔。免疫表型：STAT6、CD34、BCL-2和vimentin均阳性,CD99不同程度阳性;CK、SMA、desmin、HMB-45、Melan-A、S-100、EMA、WT-1、CD10、CD117和DOG1均阴性,Ki-67增殖指数≤5%。4例患者获得随访,随访时间18～85个月,均无复发及转移。结论 泌尿生殖系统SFT是一种少见的间叶源性梭形细胞肿瘤,临床行为较惰性,多数患者预后良好,免疫组化标记STAT6、CD34、CD...     

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.     

孤立性纤维性肿瘤

一、材料与方法1.材料 :10例孤立性纤维性肿瘤 (ＳＦＴ)病例系我院1990～ 2 0 0 0年间手术标本 ,男性 5例 ,女性 5例 ;年龄 37～ 74岁 ,平均年龄 5 5 .5岁。病程数天至数十年不等。肿瘤位于肺内 4例 ,眼眶 2例 ,下胸腔、纵隔、外耳道及肺叶间裂各 1例。首发症状为肿块所在部位引起的局部表现 ,如眼球突出、面瘫、胸闷、气急、咳嗽、下肢浮肿等 ,有者则是扪及肿块或体检发现肿块。2 .方法 :所有标本均经常规固定 ,石蜡包埋 ,4μｍ连续切片 ,除常规ＨＥ染色外均同时采用ＳＰ法免疫组织化学方法染色标记。使用抗体ＣＤ34、波形蛋白、ＨＨＦ35、…     

恶性孤立性纤维性肿瘤4例临床病理分析

目的探讨恶性孤立性纤维性肿瘤(malignant solitary fibroustumor,MSFT)的临床病理特征、组织发生、诊断、鉴别诊断、治疗及预后。方法报道4例罕见的MSFT,并结合文献进行复习。结果女性2例,男性2例,年龄22岁～63岁,中位年龄42.5岁。发生部位:中枢神经系统2例,临床表现为头痛、头晕和恶心等;胸腔2例,无明显症状,体检发现。肿块直径3～14cm,平均8.5cm。镜检:低倍镜下肿瘤细胞分布疏密不均。密集区肿瘤细胞丰富,呈束状、漩涡状或不规则状排列,可见血管外皮瘤样结构;瘤细胞梭形、短梭形,核染色质粗且不均匀,异型性明显,核分裂象多见(4个/10HPF),局灶出血坏死。疏松区细胞稀少,纤维化伴黏液变性。免疫表型:肿瘤细胞Bcl-2、CD99、vimentin弥漫阳性;CD34、MSA、α-SMA、h-caldesmon局灶阳性,Ki-67增殖指数25%～50%;EMA、CD117、GFAP和S-100阴性。结论 MSFT非常罕见,确诊主要依赖病理形态学和免疫表型,需与滑膜肉瘤、脑膜瘤、恶性外周神经鞘膜瘤、恶性纤维组织细胞瘤和纤维肉瘤等相鉴别。MSFT的预后不佳,易复发和转移,手术完整切除后,应注意随访。     

恶性孤立性纤维性肿瘤4例临床病理分析并文献复习

目的：探讨恶性孤立性纤维性肿瘤(malignant solitary fibrous tumor,MSFT)临床病理特点、诊断和鉴别诊断及治疗预后。方法：对4例MSFT回顾并分析其临床病理特点,并复习相关文献。结果：男性2例,女性2例,发病年龄43~64岁。肿瘤部位分别为胸腔内、乙状结肠系膜、右肩部、左足。镜下肿瘤呈细胞丰富区与稀疏区相间的特点,局灶形态类似血管外皮瘤。瘤细胞梭形或短梭形,异型性明显,核分裂像易见,局灶可见坏死。免疫组化：瘤细胞表达Vim、CD34、CD99、Bcl-2,不表达CK、S-100。结论：MFST少见,确诊依靠形态学和免疫组织化学,需与血管外皮瘤、纤维肉瘤、滑膜肉瘤、恶性间皮瘤及恶性外周神经鞘瘤相鉴别。MFST易复发和转移,手术完整切除是其主要治疗手段,需长期随访。     

甲状腺孤立性纤维性肿瘤（附7例报道）

孤立性纤维性肿瘤 (ＳＦＴ)是一种ＣＤ34阳性的梭形细胞肿瘤 ,最早报道发生于胸膜 ,以后陆续有报道在其他很多部位也可发生。其临床行为通常是良性 ,但也有少数报道高度富于细胞、核异型、核分裂象指数高和有坏死的病例可以复发甚至转移。1993年Ｔａｃｃａｇｎｉ最早报道 3例发生在甲状腺的ＳＦＴ ,此后又有学者报道 3例。本文报道 7例甲状腺ＳＦＴ ,病人年龄 4 3～ 6 3岁 (平均 5 2岁 )。肿块直径 2～ 6ｃｍ。眼观 :ＳＦＴ为单个孤立性结节 ,实性 ,灰白至棕黄色 ,质地坚韧 ,境界清楚 ,但无包膜 ,其中 1例切面见囊性腔 ,直径 0 1～ 1ｃｍ。…     

肾脏原发性孤立性纤维性肿瘤临床病理分析

孤立性纤维性肿瘤（solitary fibrous tumor，SFT），最初描述是一般发生在胸膜，其次是腹膜，称为良性纤维性间皮瘤，目前认为其组织发生与间皮并无关连，而是来源于具有纤维母或肌纤维母细胞特征的问质细胞。已报道SFT可发生在许多部位，如浆膜、鼻腔、胸腔、纵隔、甲状腺、眼眶、腹膜后、乳房、肝脏^[1]等，并易误诊为其他梭形细胞肿瘤。     

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。     

D2-40-positive solitary fibrous tumors of the pleura: diagnostic pitfall of biopsy specimen

Solitary fibrous tumor of the pleura (SFTP) is an uncommon spindle-cell neoplasm and is sometimes confused with other spindle-cell tumors, such as sarcomatoid mesotheliomas. D2-40 is a new monoclonal antibody recognizing podoplanin and several studies have confirmed the reliability of D2-40 in the diagnosis of pleural mesothelioma. The authors encountered a case of SFTP that was immunoreactive to D2-40. A 56-year-old woman was admitted to hospital because of an abnormal shadow on a chest X-ray. Percutaneous needle biopsy indicated a spindle-cell tumor showing positive immunoreactivity for CD34, supporting the diagnosis of SFTP. However, the tumor was also immunoreactive to D2-40 and thus malignant mesothelioma could not be ruled out. The tumor was surgically resected to make a definitive pathological diagnosis. The tumor had a patternless architecture and immunohistochemistry was positive for CD34 and D2-40 but was negative for calretinin and cytokeratin 5/6. Therefore a pathological diagnosis of SFTP was made. An additional six tumors diagnosed as SFTP were also tested. Focal immunoreactivity to D2-40 was positive in three out of seven cases, including the first case. Care is required in diagnosing biopsy specimens of D2-40-positive pleural spindle-cell tumors, especially in making the differential diagnosis between SFTP and malignant mesothelioma.     

胃肠道钙化性纤维性肿瘤临床病理分析

目的探讨胃肠道钙化性纤维性肿瘤(calcifying fibrous tumor,CFT)的临床病理学特征、免疫表型、诊断及鉴别诊断。方法回顾性分析7例胃肠道CFT的临床病理学及免疫表型特征,并复习相关文献。结果 7例患者中,男性2例,女性5例。发病部位:胃4例、小肠2例、直肠1例。临床表现:发生于胃的患者主要为上腹痛,发生于小肠的1例患者出现肠套叠,其余为体检或手术偶然发现,大体为黏膜下直径0.7~4 cm包块。镜下见肿瘤边界清晰,主要由丰富的透明变性的胶原纤维构成,呈漩涡状、束状及席纹状排列,肿瘤细胞呈梭形,无细胞异型性及核分裂象;7例均可见散在分布的砂砾体及多少不一的炎细胞浸润,2例可见营养不良性钙化。免疫表型:瘤细胞vimentin阳性,1例CD34局部阳性,1例可见CD117散在阳性细胞,desmin、DOG1、S-100、SMA、H-caldesmon和ALK均阴性,β-catenin胞核阴性,Ki-67增殖指数1%~3%。7例患者6例无复发,1例失访。结论 CFT是一种少见的间叶源性良性肿瘤,发生于胃肠道者临床罕见,临床表现及检查无特异性,特征性的组织学形态较易诊断,但需与其他间叶源性疾病鉴别。     

Clinicopathological features of solitary fibrous tumor of the meninges: An immunohistochemical reappraisal of cases previously diagnosed to be fibrous meningioma or hemangiopericytoma

Cases of solitary fibrous tumor (SFT) of the meninges are increasingly being reported. However, the real incidence of SFT among meningeal tumors has yet to be determined. We therefore clinicopathologically re-examined 64 meningeal tumors originally diagnosed to be either fibrous meningioma (FM group, n = 46) or hemangiopericytoma (HPC group, n = 18) while paying special attention to SFT. We thus reclassified one case from the FM group (2%) and one case from the HPC group (6%) to be SFT, both of which showed diffuse CD34-immunoreactivity and dense intercellular reticulin fibers but neither epithelial membrane antigen nor S-100 protein expression. The MIB-1 staining index of these cases were 6. 2% and 3.9%, respectively. The former recurred 15 years after the initial surgery and the patient underwent a second removal of the tumor. The patient has been alive with no evidence of recurrence for 7 years after the second surgery. The latter patient has been alive with no evidence of recurrence for 3 years postoperatively. The results confirmed that the incidence of SFT among meningeal tumors is relatively low, however, because of its clinically indolent nature, a careful histochemical examination is necessary to differentiate SFT from other neoplasms with a more aggressive nature. Our findings emphasize the need to clinically recognize this lesion as a distinct entity.     

Extrathoracic solitary fibrous tumors: their histological variability and potentially aggressive behavior

The histological variability of solitary fibrous tumors may contribute to the difficulty in diagnosing these neoplasms, especially when they arise in extrathoracic sites. Like intrathoracic lesions, the behavior of extrathoracic solitary fibrous tumors is currently unpredictable because these types of tumor have only recently been recognized. This study therefore was undertaken to examine the clinical behavior and histological, immunohistochemical, and ultrastructural features of 24 extrathoracic solitary fibrous tumors with long-term follow-up. The patients comprised 10 men and 14 women, between 30 and 85 years of age (mean, 51 years). Ten tumors were located in the retroperitoneum or pelvis, 5 in the trunk, 4 in the extremities, 2 in the orbital region, and 1 each in the kidney, uterine cervix, and meninges. All of the tumors showed a classic morphological appearance, diffuse and strong immunoreactivity for both vimentin and CD34, and variable reactivity for bcl-2. All 7 cases examined ultrastructurally contained fibroblasts and myofibroblasts. Six tumors contained multinucleated giant cells, and in 4 cases these lined pseudovascular spaces with mononuclear cells, thus resembling giant cell angiofibroma and giant cell fibroblastoma. Other potentially similar spindle cell neoplasms mixed with adipose tissue, such as dendritic fibromyxolipoma, lipomatous hemangiopericytoma, cellular angiofibroma, and spindle cell lipoma, were considered in the differential diagnosis. One tumor displayed atypical histological features in the form of increased cellularity and nuclear pleomorphism, but this patient has remained free of disease for 14 years. Another 2 patients developed local recurrences at 6 months and 5 years, and a further patient developed pulmonary metastases that were diagnosed after 7 years. These tumors lacked any atypical histological features in the primary lesions. No patient has so far died of the disease. In conclusion, most extrathoracic solitary fibrous tumors appear to pursue a benign course, although, because some have the potential to recur or metastasize, careful long-term follow-up is necessary for all patients.     

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.     

Pelvic cavity malignant solitary fibrous tumor with dedifferentiation and multifocal cytokeratin expression

Pelvic cavity small-sized malignant solitary fibrous tumors are rare. Surgeons easily misjudge these tumors as benign lesions, and they are usually resected by laparoscopic surgery. When accompanied by dedifferentiation, malignant solitary fibrous tumors are difficult to diagnose by pathologists. Here, we describe a challenging case. A 47-year-old man was accidentally found to have a pelvic mass after three months. Surgeons assumed a diagnosis of a benign tumor according to the Computed Tomography and Ultrasonography reports. The patient underwent laparoscopic surgery to resect the tumor. After the operation, the patient was diagnosed as having a malignant solitary fibrous tumor with dedifferentiation. The patient had a recurrence one year later. This pelvic cavity malignant solitary fibrous tumor was a rare case because of its dedifferentiation and cytokeratin expression. The expression of cytokeratin and the absence of vimentin and CD34 were pitfalls to diagnosis. In this case, there was still a high amount of malignancy despite the small size of the tumor. Clinical image and pathologic multidisciplinary analysis and core needle biopsies before surgery had a great effect on diagnosis and therapy of this disease. The case is a cautionary tale not only for pathologists but also for surgeons.     

Report of an intrapulmonary solitary fibrous tumor: Fine-needle aspiration cytologic findings,clinicopathological, and immunohistochemical features

The authors describe a rare case of intrapulmonary solitary fibrous tumor (SFT). Morphologically, the tumor showed a disorganized or “patternless” arrangement of plump to spindle cells in a collagenous stroma. Tumor cells were immunoreactive for vimentin, but not for keratin, carcinoembryonic antigen, epithelial membrane antigen, factor-VIII related antigen, S-100 protein, desmin, and actin. These immunohistochemical findings suggested fibroblastic differentiation, in keeping with the putative submesothelial origin of the tumor. The problems concerning the fine-needle aspiration (FNA) preoperative diagnosis of SFT are briefly discussed. Diagn Cytopathol 1996;14:64–67. © 1996 Wiley-Liss, Inc.     

外阴孤立性纤维性肿瘤1例临床病理观察及文献复习

目的 探讨外阴孤立性纤维性肿瘤的临床病理学特点、免疫组化特征及鉴别诊断.方法 对1例外阴孤立性纤维性肿瘤进行组织学观察,行EnVision两步法免疫组化CD34、bcl-2、CD99、vimentin、CKpan、HMB-45、α-SMA、CD117、S-100蛋白、ER、PR、Ki-67染色及随访,并复习相关文献.结果 患者女,62岁.发现右侧外阴肿物4年余.眼观:肿瘤呈结节状,大小6 cm×5 cm×3 cm,切面灰白、实性,质韧.镜检:肿瘤由交替性分布的富于细胞和稀疏细胞区组成,梭形细胞呈束状、旋涡状或不规则状排列,部分与胶原纤维混杂,部分呈血管外皮瘤样结构.上皮样细胞有的围绕血管周围.细胞间细条索状、粗绳索样或疤痕样胶原纤维不规则性或石棉样的胶原物质沉积.瘤细胞CD34、bcl-2、CD99、vimentin均为阳性,CKpan、HMB-45、α-SMA、CD117、S-100蛋白、ER、PR均为阴性,Ki-67增殖指数<1%.结论 外阴孤立性纤维性肿瘤罕见,应与该部位相似形态的病变和血管外皮瘤样的肿瘤相鉴别.