恶性孤立性纤维性肿瘤的临床病理特征分析并文献复习

目的:探讨恶性孤立性纤维性肿瘤的临床病理特征及鉴别诊断。方法:对9例恶性孤立性纤维性肿瘤进行光镜及免疫组织化学研究,并复习相关文献。结果:男性6例,平均年龄为49.7岁;女性3例,平均年龄为48.3岁;平均肿瘤直径为9.39 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、旋涡状排列,可见血管外皮瘤样结构;瘤细胞呈梭形,异型性明显,有肿瘤性坏死,核分裂均大于4个/10 HPF。免疫表型:肿瘤细胞STAT6和Vimentin一致阳性表达,CD34、BCL-2、CD99不同程度阳性表达。结论:恶性孤立性纤维性肿瘤确诊依据形态学及免疫组化,需与形态学表现相似的其他恶性梭形细胞肿瘤鉴别,其预后不良,易复发和转移,根治性切除是主要治疗手段,必要时辅助放化疗。     

肾脏肌纤维瘤1例临床病理分析

目的：阐明肾脏肌纤维瘤临床病理特点。方法：回顾性分析1例肾脏肌纤维瘤的临床表现、组织学和免疫组织化学特点。结果：患者主要临床表现为腰痛,CT检查发现左肾中下极巨大囊实性占位。大体检查：肾中下极见一灰白结节,体积10cm×7cm×5cm,切面灰白质韧,与周围组织界限较清,其间可见多个小囊腔及结节,直径均小于1cm。光镜观察：低倍镜下,肿瘤呈多结节状增生,结节周边部分一般为梭形肌样细胞排列成短束状或旋涡状结构,瘤细胞胞浆丰富,染色淡,细胞核狭长;结节中心部位瘤细胞圆形、短梭形、多角形,胞浆少,胞核深染,沿薄壁的＂鹿角状＂分支的小血管排列,呈所谓的血管外周细胞瘤样结构,局部间质可见玻璃样变性。部分区域两种类型细胞随意分布。免疫组化染色结果 Vimentin弥漫阳性,SMA、Desmin、Bcl-2、HHF-35灶性阳性,WT-1、EMA、AE1/AE3、HMB45、CD34、S-100、CD117、CD10、ALK均阴性,Ki67阳性细胞指数约为2%。结论：肾脏肌纤维瘤是一种罕见的良性肿瘤,明确诊断必须借助于临床及组织学改变和免疫组织化学特征检查,首选治疗是肿瘤全切手术。     

肾黏液样小管状和梭形细胞癌临床病理观察

 目的 探讨肾黏液样小管状和梭形细胞癌的临床病理特点及鉴别诊断。 方法 观察2例肾黏液样管状和梭形细胞癌的临床资料、组织病理学及免疫组织化学特点,并复习相关文献。 结果 患者男、女各1例,年龄分别为66岁和54岁。1例因体检被发现,另1例因贫血就诊。病理检查：肉眼瘤组织切面呈实性、灰白色,边界多清楚,未见囊性变。镜下瘤组织由多少不一的小管状和梭形细胞区构成,间质富含黏液。瘤细胞部分呈立方状,胞浆透明或弱嗜酸颗粒状,核圆形或不规则形,核分裂相少见。部分细胞梭形,束状或编织样排列,似平滑肌瘤。1例以立方状细胞成分为主,另1例以梭形细胞成分为主。免疫组织化学：2例瘤细胞均呈CK(AE1/AE3)、EMA、Vimentin阳性,其中1例并呈Syn局灶阳性;而desmin、CgA、S－100、SMA、CD117、CD34、CD10、 HMB45、p53、ER及PR均阴性,Ki67指数均小于1％;术后分别随访7月和11月,均未见复发和转移。 结论 肾黏液性管状和梭形细胞癌为罕见的肾脏低度恶性肿瘤,病理诊断时需与其他肾肿瘤鉴别。     

胃肠道间质瘤的病理学特征及诊断

目的探讨胃肠道间质瘤(GIST)的病理学特征及鉴别诊断.方法常规HE染色及免疫组织化学(S-P法)染色观察51例原发性胃肠道间质瘤的病理组织学形态及抗体CD117、CD34、Vimentin、S-100、Syn、NSE、SMA、ACT免疫组织化学表达特点,并对其形态学特征及生物学行为进行分析.结果本组51例GIST中,梭形细胞型27例,上皮样细胞型9例,混合型15例.良性14例,交界性10例,恶性27例.瘤细胞CD117、CD34和Vimentin多为弥漫性强阳性,阳性率分别为100.0%、72.5%和100.0%;部分病例SMA、ACT、S-100、SY-N、NSE呈局灶性阳性.结论本组病例组织形态变化多样,有些特征容易误诊为其他肿瘤.免疫组化CD117(c-kit蛋白)和CD34阳性表达是确诊GIST最有诊断价值的依据.诊断时主要是同消化道的其他梭形细胞肿瘤进行鉴别.依据肿瘤生长的大小、细胞核异形性及核分裂相活性来判断肿瘤的恶性程度,恶性度高的肿瘤往往伴有明显的出血及坏死.     

胸膜和胸膜外孤立性纤维性肿瘤临床病理分析

目的 探讨胸膜和胸膜外孤立性纤维性肿瘤(solitary fibrous tumor,SFT)的临床病理学特点和鉴别诊断以及对预后的指导意义.方法 采用光镜和免疫组织化学方法标志对6例胸膜SFT和8例胸膜外SFT进行分析.结果 14例(包括6例胸膜和8例胸膜外)SFT由比例不同的梭形和卵圆形肿瘤细胞组成,间质胶原纤维丰富伴有分支状血管.其中11例有典型的细胞稀少区和细胞丰富区交替分布特点,2例仅含细胞丰富区成分,1例为多发结节兼有上述两种形态特点.免疫组织化学染色结果显示14例肿瘤细胞均表达CD34、bcl-2和CD99,不表达desmin、S-100、EMA、CK(AE1/AE3)和p53.2例SMA呈部分弱阳性.SFT复发者2例,其中1例胸膜恶性SFT呈结节状播散性累及胸膜.结论 胸膜和胸膜外孤立性纤维瘤在组织学形态、免疫表型和生物学行为上相似.免疫组织化学CD34、bcl-2和CD99表达阳性对SFT的诊断和鉴别诊断具有指导意义.     

肾黏液小管状和梭形细胞癌1例报告并文献复习

目的:探讨肾黏液小管状和梭形细胞癌的临床病理特点及诊断、鉴别诊断.方法:对1例肾黏液小管状和梭形细胞癌进行临床病理学及免疫组织化学分析.结果:肾黏液小管状和梭形细胞癌患者临床无特殊表现,肿块与周围肾组织分界清楚.镜下观察:肿瘤呈不规则管状结构、间质呈黏液状,瘤细胞呈立方形或梭形、胞浆嗜酸,核形规则,核分裂少见.免疫组化染色AE1/AE3、EMA、Vimentin阳性表达,S-100、Desmin、SMA、CD34、HMB45、CD10、CD15、CgA、Syn、NSE、CD99阴性表达,Ki-67(3%).结论:肾黏液小管状和梭形细胞癌为少见肿瘤,明确该肿瘤的组织起源及病理特征,对于病理与临床诊断和鉴别诊断有重要意义.     

肾黏液小管状和梭形细胞癌1例报告并文献复习

目的：探讨肾黏液小管状和梭形细胞癌的临床病理特点及诊断、鉴别诊断。方法：对1例肾黏液小管状和梭形细胞癌进行临床病理学及免疫组织化学分析。结果：肾黏液小管状和梭形细胞癌患者临床无特殊表现,肿块与周围肾组织分界清楚。镜下观察：肿瘤呈不规则管状结构、间质呈黏液状,瘤细胞呈立方形或梭形、胞浆嗜酸,核形规则,核分裂少见。免疫组化染色AE1／AE3、EMA、Vimentin阳性表达,S-100、Desmin、SMA、CD34、HMB45、CD10、CD15、CgA、Syn、NSE、CD99阴性表达,Ki-67（3％）。结论：肾黏液小管状和梭形细胞癌为少见肿瘤,明确该肿瘤的组织起源及病理特征,对于病理与临床诊断和鉴别诊断有重要意义。     

脑膜恶性孤立性纤维性肿瘤临床病理学观察

目的:探讨脑膜恶性孤立性纤维性肿瘤的临床病理特征、核磁共振特点、鉴别诊断及治疗和预后。方法:报道一例脑膜恶性孤立性纤维性肿瘤临床病理、影像学资料并复习文献。结果:患者,男性,65岁,临床主要表现为四肢乏困,无力,记忆力差,MRI示左颞枕叶可见约8.5cm×6.5cm×6cm的囊实性病灶,术中发现肿瘤累及脑膜,与周围脑组织界限清楚,送检肿瘤组织灰白色均质状,有囊性变。镜检:肿瘤由交替性分布的细胞丰富区和细胞稀疏区组成,细胞排列呈条束状、席纹状或无结构样,部分区域呈血管外皮瘤样结构,部分区域细胞丰富密集,有异型性,核分裂象多见(>4个/10HPF),未见明显坏死。免疫表型:肿瘤细胞Vimentin、CD99、bcl-2、CD34弥漫阳性,p53弱阳性,Ki-67>10%。病理诊断:左颞枕叶脑膜恶性孤立性纤维性肿瘤。结论:脑膜恶性孤立性纤维性肿瘤非常罕见,确诊主要依靠病理形态学及免疫组织化学标记,并与脑的其它梭形细胞肿瘤鉴别。治疗以手术完整切除为主,可预防性放疗并密切随访。     

脑膜恶性孤立性纤维性肿瘤临床病理学观察

目的：探讨脑膜恶性孤立性纤维性肿瘤的临床病理特征、核磁共振特点、鉴别诊断及治疗和预后。方法：报道一例脑膜恶性孤立性纤维性肿瘤临床病理、影像学资料并复习文献。结果：患者,男性,65岁,临床主要表现为四肢乏困,无力,记忆力差,MRI示左颞枕叶可见约8.5cm×6.5cm×6cm的囊实性病灶,术中发现肿瘤累及脑膜,与周围脑组织界限清楚,送检肿瘤组织灰白色均质状,有囊性变。镜检：肿瘤由交替性分布的细胞丰富区和细胞稀疏区组成,细胞排列呈条束状、席纹状或无结构样,部分区域呈血管外皮瘤样结构,部分区域细胞丰富密集,有异型性,核分裂象多见（〉4个/10HPF）,未见明显坏死。免疫表型：肿瘤细胞Vimentin、CD99、bcl-2、CD34弥漫阳性,p53弱阳性,Ki-67〉10%。病理诊断：左颞枕叶脑膜恶性孤立性纤维性肿瘤。结论：脑膜恶性孤立性纤维性肿瘤非常罕见,确诊主要依靠病理形态学及免疫组织化学标记,并与脑的其它梭形细胞肿瘤鉴别。治疗以手术完整切除为主,可预防性放疗并密切随访。     

54例胃肠道间质瘤临床病理及免疫组织化学研究

目的探讨胃肠道间质瘤（GIST）的临床病理组织形态学和免疫组织化学特点。方法用CD117和CD34等6种抗体,通过贫疫组化S-P法对发生于胃肠道的间叶源性肿瘤进行研究。确诊54例间质瘤,对其进行临床病理组织学和免疫组化分析。结果本组GIST共54例,发病年龄为32-75岁,中位年龄54岁。临床表现为腹痛、腹胀及发现腹部包块等。大体形态上GISTs肿块可单发或多发,体积大小不等,可见出血、坏死及囊性变。镜下GISTs瘤细胞形态主要为梭形和上皮样形,大部分梭形瘤细胞排列呈束状或交织状,灶性可呈车辐状、栅栏状及围绕血管呈器官样排列。本研究54例肿瘤中梭形细胞型28例（51．8％）,上皮样细胞型9例〈16．7％）,混合型17例（31．5％）。38例（70．3％）出现交叉束状排列,30例（55．6％）出现弥漫片状或巢状排列,18例（33．3％）出现栅栏状排列,16例（29．6％）围绕血管呈器官样,31例（57．4％）同时具有多种排列结构。免疫组化显示54例GIST中CD117阳性表达率为92．6％,CD34的阳性表达率为77．8％;Aetin与S-100阳性瘤细胞大部分为散在或小灶状分布,Desmin均为阴性表达。结论GIST多发于中老年。肿瘤细胞形态多样,结构多样;免疫组化CD117和CD34阳性表达是确诊间质瘤最有诊断价值的依据,但间质瘤良、恶性诊断上仍需结合肿瘤的大体、组织学形态及生物学行为等综合考虑。     

恶性孤立性纤维性肿瘤的临床病理观察及文献复习

目的:探讨恶性孤立性纤维性肿瘤的临床病理特点、免疫表型、诊断和鉴别诊断,以提高对其临床及病理改变的认识.方法:回顾性分析7例恶性孤立性纤维性肿瘤的临床特征、组织学形态及免疫组化结果,并复习相关文献结合随访结果进行讨论.结果:7例患者中,男性3例,女性4例,年龄33~72岁,平均47.9岁.发生部位:肾脏、前列腺、腹膜后、小脑幕、肩胛区、枕部和足底.肿瘤直径2.8~11.2cm.镜下:尽管部分病例中仍保留良性孤立性纤维性肿瘤的特殊形态学特征,但所有病例都具备一些恶性的指征,包括瘤细胞密度较高、异型性明显、核分裂象多见(>4个/10HPF)以及出血和坏死.除以上特点之外,本文病例中有一例局灶出现上皮样分化,即卵圆形或圆形肿瘤细胞呈巢片状或假腺样排列.免疫表型:vimentin呈弥漫强阳性表达,CD34、CD99和BCL-2表达情况各不相同,STAT6阳性率为85.7%.另外,2例病例局灶表达Keratin,1例局灶表达EMA,1例局灶表达Calponin.结论:本文综合分析了七例恶性孤立性纤维性肿瘤的临床病理特点,而且首次报道了1例"具有上皮样特征的"MSFTs.与经典型良性SFT不同,大部分MSFTs或多或少缺失三种标志物(CD34、CD99和BCL-2)的表达.MSFTs的预后不佳,易复发和转移.     

Spindle cell carcinoma of the nasal cavity

We report an extremely rare case of spindle cell carcinoma of the sinonasal cavity. A 75-year-old man was admitted to our hospital because of right nasal obstruction. Nasal endoscopy showed a polypoid tumor measuring 3?×?3?cm at the nasal septum in the right nasal cavity, and an excisional biopsy was performed. Computed tomography (CT) demonstrated the nasal tumor extended to the maxillary sinus. Histologically, the tumor consisted of malignant spindle cells with hyperchromatic nuclei. Mitotic figures and necrosis were recognized. In some areas, edematous changes were recognized. No apparent differentiation was noted. The tumor cells were free of keratinization and intercellular bridge formations; therefore, there were no squamous cell components. Immunohistochemically, the tumor cells were positive for pancytokeratin, cytokeratin (CK)5/6, CK18, CK19, high molecular weight CK, p63, and vimentin. The tumor cells were negative for epithelial membrane antigen, CK7, CK14, p53 protein, S100 protein, HMB45, chromogranin, synaptophysin, CD34, CD56, glial fibrillary acidic protein, neuron-specific enolase, neurofilaments, ??-smooth muscle actin, neuroblastoma, myoglobin, carcinoembryonic antigen (CEA), carbohydrate antigen (CA)19-9, CD3, CD20, CD30, CD45RO, and CD45. A pathological diagnosis of spindle cell carcinoma of sinonasal lesion was made. The patient underwent resection of right maxilla followed by chemotherapy and radiation and was alive without metastasis 5?years after initial presentation.     

膀胱孤立性纤维性肿瘤 1 例报告并文献复习

目的:探讨膀胱孤立性纤维性肿瘤(solitary fibrous tumor，SFT)的临床表现、影像学表现、病理学形态、免疫表型、生物学行为、诊断、鉴别诊断、治疗方法及其预后。方法:回顾性分析 1 例膀胱 SFT 的临床资料、病理学形态、免疫表型、诊断治疗及随访情况，并结合国内外文献进行分析。结果:本例患者行根治性肿瘤切除术，肿瘤肉眼观表面光滑，切面灰白，质略韧，有包膜，无出血、坏死；镜检:肿瘤界清，无明显侵袭性，细胞稀少区与丰富区交替分布，以梭形细胞为主，核深染，染色质均匀；免疫表型:肿瘤细胞弥漫表达 CD34、Bcl-2；病理诊断为:（膀胱）孤立性纤维性肿瘤。术后随访 24 个月，体格检查正常，行盆腔 MRI 检查未见复发及远处转移。结论:膀胱SFT的诊断依赖临床表现、影像学以及病理组织学检查，确诊则须依靠病理学的免疫组化检查。膀胱 SFT生物学特性大多呈良性表现，根治性切除是主要治疗手段，但少数亦可局部复发或转变为恶性，故有必要对膀胱SFT患者术后进行长期随访。     

Malignant solitary fibrous tumor

BACKGROUND:

Malignant solitary fibrous tumors (SFTs) are extremely uncommon and poorly understood mesenchymal neoplasms. There are only rare published accounts of the cytopathologic features of these tumors, prompting the current study.

DESIGN:

All cases of malignant SFT with preoperative fine‐needle aspirations (FNAs) from 1999 to 2008 were retrieved from the archives of 3 large teaching hospitals. FNA smears and cell block material including immunoperoxidase stains were reviewed, and the cytologic characteristics were described.

RESULTS:

Thirteen cases of malignant SFT were identified in 11 patients. Mean age was 58 years, with a men:women ratio of 1:2.6. The tumors were generally large, with a mean size of 13.4 cm. Cytomorphologic features included mostly hypercellular smears with tissue fragments of monotonous, plump spindled cells with blunt‐ended and indented nuclei and fragile, wispy cytoplasm. Also seen were bare nuclei, occasional mitoses, and rare necrosis. Some cases showed a predominance of epithelioid cells, whereas others displayed a loose myxomatous matrix. There was a general lack of single cells. None of the cases was diagnosed accurately as malignant SFT on FNA, and only 6 cases were called malignant or suspicious for malignancy.

CONCLUSIONS:

The FNA diagnosis of malignant SFT is extremely difficult and needs histologic material for accurate interpretation. Predominant FNA diagnoses were SFT or spindle cell neoplasm. Malignant SFT must be included in the differential diagnosis of a spindle cell neoplasm of any anatomic site, particularly if it displays features not typical of benign SFT. Immunoperoxidase staining has some utility, mainly in ruling out other neoplasms in the differential diagnosis. Cancer (Cancer Cytopathol) 2010. © 2010 American Cancer Society.     

A case of solitary fibrous tumor of the meninges

We report a rare case of solitary fibrous tumor (SFT) of the meninges of the posterior fossa presenting as an intracerebellar hemorrhage. A 29-year-old woman was admitted with sudden-onset severe headache, nausea, and vomiting. A computed tomographic (CT) scan of the brain revealed an intracerebellar hemorrhage 3.5 cm in diameter. Gadolinium-enhanced magnetic resonance imaging (MRI) showed a heterogeneous enhancement mass. A posterior craniotomy found a firm, highly vascular tumor attached to the meninges. Histologically, the tumor showed mostly sclerotic tissues with spindle cells. In few areas, the tumor had a more compact arrangement of spindle-shaped cells with vascular spaces and highly cellular components. Immunohistochemical study revealed strong CD-34 immunopositivity in many tumor cells. The tumor was diagnosed as SFT of the meninges. We report the clinical and histological features of this newly described tumor with a heterogeneous component.     

Clinicopathological analysis of solitary fibrous tumor

Objective

The aim of this study was to investigate the clinicopathologic characteristics, diagnosis and differential diagnosis, molecular genetics, treatment and prognosis of solitary fibrous tumor (SFT).

Methods

The clinicopathological manifestations were analyzed retrospectively in 22 patients with surgically confirmed SFT.

Results

There were 12 male patients and 10 female patients, with the age range 33–67 (mean 48.62) years. The SFTs originated from different from parts of the body, including 13 in the chest, 2 in the lungs, 3 in the abdomen, 1 in the lumbosacral area, 2 in the pelvis, and 1 in the left shoulder. There were 19 benign and 3 malignant tumors. Major clinical presentations were local masses and compression symptoms. Microscopy: the tumor was composed of areas of alternating hypercellularity and hypocellularity. The tumor cells were spindle to short-spindle shaped and arranged in fascicular or storiform pattern and hemangiopericytoma-like structure was presented. Immunohistochemically, Vimentin positive rate was 100% (22/22), Bcl-2 positive rate was 95.5% (21/22), CD99 positive rate was 86.4% (19/22), CD34 positive rate was 81.8 (18/22), focally positive for P53, as well as negative CK, S100 and Desmin. Ki67 labelling index was 2%–30%.

Conclusion

SFT is a rare tumor which may be found in various parts of human body. SFT mostly is a benign tumor, but a few could be malignant. Its diagnosis mainly rely on its morphologic features and immunohistochemical profiles. The major treatment is to completely resect it by operation and long-term clinical follow-up is necessary.     

CD44V3、CD44V6基因蛋白在卵巢恶性肿瘤中的表达及临床意义

 目的 探讨恶性卵巢肿瘤组织中CD44V₃ 、CD44V₆ 的表达及其临床意义。方法 用免疫组织化学方法,检测41 例卵巢恶性肿瘤、20 例卵巢良性肿瘤和21 例正常卵巢组织中CD44V₃ 、CD44V₆ 基因蛋白的表达情况并分析相关的临床病理因素。结果 CD44V₃、CD44V₆ 表达阳性率恶性组显着高于良性组和正常组;有转移者CD44V₃ 、CD44V₆ 阳性率显着高于无转移者,转移灶阳性率高于原发灶;CD44V₃、CD44V₆ 阳性表达者近期疗效比阴性者差;CD44V₃ 、CD44V₆ 阳性表达率与病理类型、组织学级别无关。结论 CD44V₃、CD44V₆ 与近期疗效有关,可作为评估预后的指标。