外耳道近端型上皮样肉瘤2例并文献复习

目的 探讨近端型上皮样肉瘤(proximal-type epithelioid sarcoma,PES)的临床病理特点、诊断及鉴别诊断.方法 报道2例外耳道PES,镜下观察组织学形态特征并结合免疫组化染色结果进行分析.结果 镜下见肿瘤由梭形或上皮样细胞组成,可见横纹肌样细胞;呈结节状排列,伴片状坏死;免疫表型:CKpan、vimentin、CD34均呈阳性,CD31、S-100、CD68、HMB-45、ALK、SMA、CK7、CK20、TTF-1、desmin、Syn、CgA均呈阴性.结论 PES是一种少见的软组织恶性肿瘤,形态复杂多样,极易误诊.需临床、组织学及免疫表型等相结合,才能作出准确的诊断.     

近端型上皮样肉瘤3例临床病理分析

目的探讨近端型上皮样肉瘤的临床病理学特征、诊断及鉴别诊断、治疗及预后。方法回顾性分析3例近端型上皮样肉瘤的临床病理特征、免疫表型等,并复习相关文献。结果 3例均为男性,发病年龄23～83岁,分别位于左足、右侧锁骨上窝和睾丸。临床均表现为无痛性生长的结节状肿块。镜下肿瘤组织呈多结节性、巢团状或片状浸润性生长,均可见坏死。肿瘤细胞体积较大,呈圆形、卵圆形上皮样,异型性明显,染色质空泡状,可见明显核仁。部分区域,瘤细胞呈横纹肌样。免疫表型:3例瘤细胞均表达vimentin、CKpan和EMA,1例表达CD34。2例近端型上皮样肉瘤均行肿物局部切除或扩大切除,其中1例辅以化疗;1例行肿块和肠黏膜活检术。例1在3年内复发5次,发生淋巴结转移后仍存活;例2确诊后拒绝治疗,3个月后死亡,例3手术切除后随访3年无瘤生存。结论近端型上皮样肉瘤是一种较罕见的恶性肿瘤,临床表现无特异性,确诊需依赖病理形态及免疫表型。近端型上皮样肉瘤切除后局部复发率高,易发生淋巴结和肺部转移。     

上皮样肉瘤临床病理特征并文献复习

目的:探讨上皮样肉瘤的临床病理学特点及诊断要点。方法:对2例上皮样肉瘤进行临床资料、病理形态学及免疫组化观察,并结合文献对其诊断及鉴别诊断进行探讨。结果:镜下肿瘤由上皮样细胞或梭形细胞构成,呈结节状排列,浸润性生长;免疫组化示肿瘤细胞Vimentin、CK、EMA、CD34呈阳性。结论:上皮样肉瘤,临床少见,因此缺乏对其认识,从而易与其他肿瘤混淆导致误诊。提高对上皮样肉瘤的认识,对避免误诊是至关重要的。     

硬化性上皮样纤维肉瘤1例

患者女性,34岁,发现左手虎口区肿物1年余,明显增大1个月。查体:左手虎口区可扪及皮下肿物,大小为3 cm×2 cm×2 cm,质地硬,活动度差。X线示左手虎口区皮下软组织内有分叶状实性肿块。手术切除肿物,术中见肿物位于拇指深肌肉下方,与掌骨骨膜粘连,边界不清。病理检查眼观:不规则组织一堆,大小为3.5 cm×2cm×2 cm,切面灰白色,质中等硬。镜检:大部分区域瘤细胞稀少,呈单个散在条索状排列。在肿瘤细胞相对集中区域,     

上皮样血管肉瘤1例报道并文献复习

目的 探讨软组织上皮样血管肉瘤(epithelioid angiosarcoma,EAS)的临床病理特点、诊断和鉴别诊断.方法 报道1例臀部软组织EAS临床资料、光镜及免疫组化染色结果,并结合文献复习进行讨论.结果 光镜下肿瘤成分几乎全部为上皮样肿瘤细胞组成,细胞排列成小巢状、片状或条索状,可见原始血管结构形成.免疫组化染色:瘤细胞高表达CD34、CKpan及vimentin,并表达CD31、CD99;而SMA、S-100、Myoglohin及HMB-45均阴性;Ki-67增殖指数＜20%.结论 软组织血管肉瘤罕见但上皮样型更为罕见,经组织病理学详细观察及免疫组化染色协助,对于正确诊断和鉴别诊断有重要意义.     

肢端黏液样炎性纤维母细胞肉瘤2例及文献复习

目的探讨肢端黏液样炎性纤维母细胞肉瘤的临床病理学特征及鉴别诊断。方法对2例发生在下肢末端的黏液样炎性纤维母细胞肉瘤进行光镜观察和免疫组化标记，并复习文献。结果2例发生在下肢末端的病程较长的渐进性肿块，术后局部复发。镜检：病变呈多结节状，边界不清；黏液样基质中见数量不等的各类炎细胞浸润，散在或灶性分布梭形、奇异形和多空泡状脂肪母细胞样3种形态的瘤细胞。免疫表型：肿瘤细胞Vim弥漫阳性，CD68和CD34灶性阳性，CK、SMA、HHF-35、S-100蛋白、CD45、CD45R0、CD15、CD30均阴性。结论此病病程较长，术后易局部复发，是一种低度恶性的肿瘤。鉴于病变黏液样基质及各类炎细胞浸润的背景较为突出，而特征性的瘤细胞稀疏，应注意与炎症性病变或具有相似组织形态的良性或恶性肿瘤鉴别。     

阴茎假血管肉瘤样鳞状细胞癌1例及文献复习

目的探讨阴茎假血管肉瘤样鳞状细胞癌(SCC)的诊断、组织学和免疫组化特征以及鉴别诊断。方法通过HE、免疫组化及特殊染色观察1例阴茎原发假血管肉瘤样鳞状细胞癌，并复习文献。结果镜下可见显著的棘细胞松解，肿瘤由排列呈腔隙状、网状结构的长梭形细胞组成，细胞异型性明显，核分裂象易见，胞质大多红染，局部区域弥漫出血坏死。免疫表型：CK、Vim阳性，CD34、CD31、SMA、FⅧRAg阴性。网状纤维染色不显示血管外形。结论假血管肉瘤样SCC是一种罕见的易与敌国管肉瘤混淆的特殊形态的SCC．需与血管肉瘤、癌肉瘤等鉴别。     

甲状腺上皮样血管肉瘤2例报道及文献复习

目的：探讨甲状腺肿瘤上皮样血管肉瘤的临床病理学特征。方法：对2例原发于甲状腺的上皮样血管肉瘤进行光镜观察和免疫组化标记。结果：2例均为老年患者，以颈部包块就诊，其中1例伴有地方性甲状腺肿。镜下显示，肿瘤主要由实性片状或巢状排列的上皮样细胞组成，瘤细胞核大，空泡状，含有明显的核仁，部分瘤细胞的胞质内可见含有红细胞的空泡或小管腔形成。除实性区域外，可见内衬上皮样瘤细胞的不规则性血管腔，网状纤维染色能清晰显示这些管腔结构，特别是在HE染色达CK8和Tg。结论：原发于甲状腺的上皮样血管肉瘤具有一定的临床病理学特点，光镜形态、免疫组化标记及电镜观察均支持瘤细胞具内皮细胞分化，应被视为甲状腺肿瘤中一种独立的病理学类型，而不应笼统地归入到未分化癌中。     

近端型上皮样肉瘤一例

患者男,51岁。右会阴肿物2个多月,疼痛难忍,行走时加剧,于2005年3月入院。体检:右侧会阴部一5cm×2cm 质硬肿物,压痛,边缘清,活动,皮肤无红肿和破溃,皮温稍高,双侧腹股沟淋巴结无肿大。MRI 示皮下组织深面与右侧阴茎海绵体间包块,考虑为尿道周围腺炎性包块。术中见:皮下肿物,约5cm×5cm,包膜完整。病理检查:4cm×4cm×2cm 肿物,表面光滑,切面暗红灰白色,部分囊性变,质地中等。镜下观察:肿瘤细胞呈结节状或片状分布(图1),主要由大的上皮样细胞和横纹肌样细胞构成,核分裂象多见(>10个/10 HPF),上皮样瘤细胞胞     

腹腔上皮样炎性肌纤维母细胞肉瘤临床病理分析并文献复习

目的：通过对上皮样炎性肌纤维母细胞肉瘤(epithelioid inflammatory myofibroblastic sarcoma, EIMS)的临床病理学特征的分析,提高对其诊断认识能力,减少误诊。方法：分析3例EIMS的临床病理特征并复习相关文献。结果：术后镜下示：肿瘤细胞弥漫分布,呈圆形、上皮样,胞浆丰富、嗜酸性、核偏位,核仁明显,部分可见梭形细胞,背景显著黏液变性伴突出的炎症细胞浸润,以中性粒细胞为主。免疫表型：肿瘤细胞表达ALK、Desmin、CD30、EMA、INI-1;部分表达：CK和SMA;不表达LCA、CD15、MyoD1、S-100、HMB-45、CD20、CD3、CD34、CD117和DOG-1。FISH检测显示3例均有ALK基因相关易位。结论：EIMS是高度恶性肿瘤,发生率比较低,细胞学形态特征、免疫组织化学特点及分子特征对本病的诊断具有重要价值。     

Prostatic stromal sarcoma: case report and review of the literature

We report a case of a 65-year-old man with rare prostatic stromal sarcoma in which suprapubital radical prostatectomy was performed, but neither chemotherapy nor radiation therapy were administered before or after the operation. The well-circumscribed tumor, measuring 5 cm in diameter, showed a homogeneous white grayish cut surface with a hard consistency. Histopathologically, the tumor consisted mainly of medium-sized rounded cells with a sarcomatous and epithelioid appearance intermingled with collagen fiber. Hyalinized foci were also noted in the tumor. Immunohistochemistry revealed that the tumor cells were diffusely positive for vimentin and focally positive for progesterone receptor and CD34, but not for EMA, cytokeratin or estrogen receptor. No recurrence or distant metastasis of the tumor has occurred in 8 years of follow up. The tumor was diagnosed as prostatic stromal sarcoma (PSS) showing epithelioid differentiation and of a progesterone-dependent nature. Possible favorable nature of the PSS might be expected after complete resection.     

软骨样脂肪瘤1例报道及文献复习

目的探讨软骨样脂肪瘤的临床、病理学特点和鉴别诊断。方法报道1例右小腿软骨样脂肪瘤的临床资料、光镜、组化、免疫组化及电镜观察结果,结合文献讨论。结果镜下见肿瘤由3种成分以不同比例混合构成:①较成熟脂肪细胞体积小的单泡状和多泡状脂肪母细胞,胞质淡染为主,少数细胞呈嗜酸性,核形多样,无异型性及核分裂象,排列成片状和巢状;②脂肪母细胞间黏液透明性软骨样基质;③多少不等的成熟脂肪细胞。PAS染色见脂肪母细胞的胞质内含许多可被淀粉酶消化的深红染颗粒,提示存在糖原。AB染色见黏液透明性软骨样基质呈阳性反应,并部分耐透明质酸酶消化,提示含有硫酸软骨素。脂肪母细胞和成熟脂肪细胞表达S-100蛋白,其中脂肪母细胞对S-100蛋白的表达与脂肪母细胞分化成熟程度相关。电镜观察可见处于不同发育阶段的脂肪母细胞,脂肪母细胞周围被絮状的软骨样基质围绕。结论软骨样脂肪瘤是一种十分罕见的良性脂肪细胞肿瘤的特殊类型,具有独特的组织学形态,应注意与黏液型脂肪肉瘤和骨外黏液样软骨肉瘤等肿瘤鉴别。     

肺动脉内膜肉瘤1例并文献复习

目的探讨血管内膜肉瘤的临床及病理特点。方法对1例肺动脉内膜肉瘤进行光镜、电镜观察和免疫组化检测并复习文献。结果肿瘤位于肺动脉血管腔内,阻塞管腔,并延伸至周围小血管。光镜下肿瘤细胞主要为梭形细胞,散在分布上皮样细胞和多核巨细胞,核分裂象约46／50HPF。电镜观察见细胞内含有丰富的粗面内质网、线粒体,无向特殊细胞分化的特征。免疫组化染色vimentin呈弥漫阳性、α—SMA灶性阳性、HMB-45散在弱阳性,CKpan、CD34、CD31和S-100蛋白等均阴性。结论内膜肉瘤是一种罕见的发生于大血管壁的恶性肿瘤,预后差,免疫表型无特异性,了解该肿瘤发病部位、临床及病理特点有助于做出正确诊断。     

肾透明细胞肉瘤的临床病理及免疫表型特征

目的 探讨肾透明细胞肉瘤（clear cell sarcoma of the kidney,CCSK）的临床病理特点、免疫表型特征及鉴别诊断。方法 应用HE和免疫组化vimentin、bcl-2、desmin、S-100蛋白、CD99、CD34、CDll7、CK、EMA染色,观察2例CCSK的病理组织学形态,并复习文献。结果 镜下见瘤细胞为上皮样或短梭形,被分枝状纤维血管间质分隔成巢团状,部分区域见黏液样变性微囊肿和细胞外胶原玻璃样变类似骨样组织的硬化型等形态变异。免疫组化示：瘤细胞vimentin和bcl-2弥漫阳性,余为阴性。结论 CCSK是一种罕见的儿童期恶性肾肿瘤,诊断主要依靠组织病理学和免疫组化,熟悉其形态学变异有利于与其它类似病变如肾母细胞瘤、先天性中胚叶肾瘤、肾恶性横纹肌样瘤、原始神经外胚叶肿瘤等鉴别。     

透明细胞肉瘤临床病理分析

目的探讨透明细胞肉瘤（clear cell sarcoma,CCS）的临床病理特点及诊断与鉴别诊断。方法对9例CCS进行临床病理分析,并行组织学、免疫组化染色和电镜观察。结果9例CCS患者年龄19～62岁,平均38．6岁;肿瘤多数位于肢体末端（6／9）。组织学观察：肿瘤由被纤维组织分隔成巢或束状的胞质透亮或淡伊红染的上皮样细胞和梭形细胞构成,核圆形或卵圆形,较空淡,可见明显核仁。1例见少数多核巨细胞,1例胞质内含色素颗粒,2例间质黏液样变性,1例局部侵及表皮。术后随访6—24个月,6例中2例局部复发伴转移。免疫组化观察：9例CCS均表达vimentin和HMB-45,7／9例S-100蛋白阳性,5／9例NSE阳性,4／9例melan-A阳性,1／9例Syn阳性。电镜观察：胞质内可见不同时期的黑色素小体。结论CCS是好发年轻人肢体末端的软组织肉瘤,预后不良,属高度恶性。诊断应结合临床与病理形态,免疫组化在诊断和鉴别诊断中起重要作用。     

Clear cell sarcoma of the pancreas: a case report and review of literature

Clear cell sarcoma (CCS), is an uncommon malignant soft tissue neoplasm that displays melanocytic differentiation with a distinct molecular profile. It is very rarely localized in gastrointestinal tract. We reported the first case of a primary CCS arising in pancreas. A 36-year-old man presented with jaundice for one month. A preoperative abdominal computer tomography showed a low-density mass in the head of pancreas. Whipple procedure was performed and the tumor was resected. Pathological examination showed polygonal or fusiform cells arranged in a uniform nested to fascicular growth pattern with thin fibrous septa. Immunohistochemical studies revealed positivity for HMB-45, Melan A, S-100, MiTF and vimentin protein. Fluorescence in situ hybridization on paraffin section showed a translocation involving the EWSR1 gene region. No BRAF and NRAS mutation was detected. The patient underwent transcatheter arterial chemoembolization (TACE) six times and eventually died of diffuse liver metastasis 10 months later. This case illustrates that the pancreas is a potential site for primary clear cell sarcoma and molecular studies play an important role in making a conclusive diagnosis.     

肾集合管癌1例报道及文献复习

目的 探讨起源于肾髓质集合管的肾集合管癌，分析其病理形态学特征及鉴别诊断。方法 对1例肾集合管癌进行大体、光镜及免疫组化观察并结合文献分析。结果 镜下肿瘤呈弥漫性或腺管状、乳头状排列。肿瘤纤维性间质反应性增生。免疫表型肿瘤细胞高分子CK、EMA、vimentin、溶菌酶阳性。结论 肾集合管癌是一种起源于肾髓质集合管的罕见的肾肿瘤，恶性度高，预后差。     

Infant pleuropulmonary blastoma: report of a rare case and review of literature

In infants, pleuropulmonary blastoma is a rare but aggressive tumor. The typical histopathological presentation includes the aggregation of malignant primitive small cells, usually observed in sheets. So as to provide proper and timely treatment, the differential diagnosis includes pulmonary blastoma, sarcomatoid mesothelioma, fetal rhabdomyoma, synovial sarcoma, and primitive neuroectodermal tumor. Herein, we will present one male pediatric patient with pleuropulmonary blastoma. The patient was a 4-month-old male infant, who had a prolonged cough and dyspnea for 4 months that was complicated by cyanosis for 3 days. A physical examination revealed a solid mass in the right lung that was sized 9.0 × 6.0 × 4.0 cm and had a grayish-white cross section. The boundary between the mass and lung tissue was clear; the mass already occupied a great portion of the lung. A microscopic examination suggested that the tumor was composed of round or orbicular-ovate primitive fetal cells. The cells were medium sized, having little cytoplasm, but had a clearly visualized nucleolus and active karyokinesis. The tumor mass was biphasic, namely, fasciculated sarcoma (composed of spindle-shaped cells and short spindle-shaped cells) and malignant fibrous histiocytoma containing well-differentiated cartilage islands or cartilaginous nodes. Immunohistochemistry was performed for further detection: vimentin (+), S-100 protein (+), CK (AE1/AE3), EMA and TTF-1 in residual epithelial components (+), NSE (focal +), SMA (mesenchymal cells, focal +), CD99 (weak +), Bcl-2 (weak +), desmin (-), myoglobin (-), calretinin (-), calponin (-), FLI (-), MyoD-1 (-), and CD34 (-). Pleuropulmonary blastoma is extremely rare but highly aggressive neoplasm in children. Its typical histopathological presentation is the aggregation of primitive malignant small cells. Combining imaging and histopathological examinations and clinical data should help in determining the diagnosis of pleural pulmonary blastoma.     

脊索瘤样脑膜瘤1例并文献复习

目的:探讨脊索瘤样脑膜瘤(chordoid meningiomas,CM)的临床与病理特点.方法:应用组织病理学、组织化学以及免疫组织化学方法对1例CM进行观察,同时复习相关文献进行讨论.结果:CM病理特点为黏液样基质中有呈分叶状或条索状排列的肿瘤细胞,细胞质呈淡染或嗜酸性染色,部分细胞呈液滴状,分布于黏液样基质中,类似脊索瘤样细胞;肿瘤细胞中亦可见典型脑膜上皮细胞区域.组织化学染色阿尔辛蓝-过碘酸-Schiff反应(alcian blue and peridic acid-schiff's reacyion,AB/PAS)呈阳性反应.免疫组织化学染色显示:肿瘤细胞波形蛋白(vimentin)、上皮膜抗原(epithelial membrane antigen,EMA)表达阳性,孕激素受体(progesterone receptor,PR)灶状阳性,广谱细胞角蛋白(pan cytokeratin,CKpan)、S-100钙结合蛋白(S-100 calcium binding protein,S-100)表达阴性.结论:CM是发生于颅内的较少见的脑膜瘤亚型,需要与脊索瘤、黏液性软骨肉瘤及转移性黏液腺癌等鉴别.