肝脏单形性上皮样血管平滑肌脂肪瘤

目的：探讨肝脏单形性上皮样血管平滑肌脂肪瘤的临床病理学特征及诊断、鉴别诊断要点。方法：对1例单形性上皮样血管平滑肌脂肪瘤进行临床病理学分析及免疫组织化学研究。结果：肝脏单形性上皮样血管平滑肌脂肪瘤临床多无症状,光镜下单形性上皮样血管平滑肌脂肪瘤由形态多样的上皮样细胞构成,胞质透明或嗜酸,无脂肪组织及异常血管;免疫表型;HMB45阳性,SMA及vimentin部分阳性,desmin少数阳性,S－100蛋白弱阳性,cytokeratin及AFP阴性,CD34血管内皮细胞阳性。结论：肝脏单形性上皮样血管平滑肌脂肪瘤是极为罕见的间叶性肿瘤,组织起源至今不明,其诊断及鉴别诊断主要依靠病理组织学及免疫组织化学。     

甲状腺样滤泡性肾细胞癌的临床病理特点

甲状腺样滤泡性肾细胞癌(thyroid-like follicular renal cell carcinoma,TLFRCC)是一种非常少见的肾原发性恶性上皮性肿瘤,确诊主要依靠病理组织学及免疫组织化学,外科手术切除是最好的治疗方法,预后好.通过对郑州人民医院2014年收治的1例TLFRCC患者的临床资料、大体特征、组织病理学及免疫表型等进行分析,并结合文献报道的28例,进一步探讨TLFRCC的临床病理特点、免疫表型、诊断与鉴别诊断及遗传学特点.     

脊索瘤2例临床病理特征并文献复习

目的：探讨脊索瘤的临床病理特征、诊断及鉴别诊断、治疗和预后。方法：对本研究组的2例脊索瘤的临床病理特征和免疫组织化学特点进行分析,并结合相关文献复习。结果：脊索瘤瘤细胞呈分叶状结构,细胞散在或呈条索及小巢状,液滴状细胞体积较大,胞浆中含大小不等的空泡;另一种体积小呈星芒状,且胞浆内无空泡。免疫组织化学显示,1例瘤细胞CK、EMA、S-100、Vimentin阳性表达,另1例CK、EMA、Vimentin阳性表达,而S-100阴性。结论：脊索瘤发病率低,但根据其常见的发病部位及特征性的组织形态,结合免疫组织化学方法,有助于其诊断及鉴别诊断。     

中枢神经系统非典型畸胎样/横纹肌样瘤临床病理及免疫表型特征

目的 探讨中枢神经系统非典型畸胎样／横纹肌样瘤的临床病理特征、诊断及鉴别诊断。方法 对2例非典型畸胎样／横纹肌样瘤应用光镜行HE、网状纤维染色及免疫组织化学染色观察,并结合文献复习。结果 非典型畸胎样／横纹肌样瘤具有特征性的横纹肌样细胞,伴有不同程度的原始神经外胚叶、上皮和间质分化。肿瘤组织富于网状纤维,免疫组织化学标记示波形蛋白、CD99、上皮细胞膜抗原、细胞角蛋白、胶质纤维酸性蛋白、S-100蛋白、神经微丝蛋白、结蛋白、平滑肌肌动蛋白阳性,突触素、肌调节蛋白、胎盘碱性磷酸酶和HMB45阴性。结论 非典型畸胎样／横纹肌样瘤是中枢神经系统一种罕见的高度恶性肿瘤,好发于儿童,偶见于成人,呈异源性组织学和免疫组织化学表型。其诊断需与脑内其他多形性肿瘤鉴别。     

肺原发性脑膜瘤1例并文献复习

目的：探讨肺原发性脑膜瘤的临床病理特征、诊断和鉴别诊断、治疗及预后。方法：回顾性分析1例肺原发性脑膜瘤患者的临床资料、组织病理形态、免疫组织化学染色、治疗及随访结果,并回顾相关文献。结果：光镜下肿瘤细胞呈短梭形,细胞呈漩涡状排列,细胞异型性不明显,未见明确核分裂象。免疫组织化学染色结果显示肿瘤细胞胞浆AE1/AE3、CK8/18灶状(+)、EMA(+), S100、SMA、TTF-1、CD34、CD31、PR、ALK、Syn均(–),Ki-67阳性率约1%。组织学和免疫组织化学染色结果均支持良性脑膜瘤的诊断。结论：肺原发性脑膜瘤十分少见,掌握其临床病理特征对该病的诊断、鉴别诊断、治疗及预后具有重要意义。     

膀胱透明细胞腺癌的临床病理分析

目的:探讨膀胱透明细胞腺癌(clear cell adenocarcinoma,CCA)的形态学和免疫组织化学特征.方法:对1例膀胱透明细胞腺癌进行临床病理和免疫组织化学分析,并结合文献讨论其诊断与鉴别诊断.结果:患者临床表现为尿路刺激症、血尿及排尿困难.内镜发现膀胱三角区肿块.组织学显示癌组织呈分化良好的腺管状或管囊...     

恶性黑色素瘤组织学变异型与鉴别诊断

目的研究恶黑的临床病理特点、组织学变异型及鉴别诊断。方法应用HE染色、免疫组化标记对130例恶黑形态学进行观察、分析临床资料，并划分组织学变异型。结果根据病理形态学发现及免疫组化标记，130例恶黑组织学变异被分为上皮样型、肉瘤样型、癌肉瘤样型、促结缔组织增生型、小细胞型、血管周细胞样型、假腺样型、浆细胞样型、横纹肌样型、巨细胞样型、印戒细胞型、透明细胞型、气球样细胞型及炎性MFH样型。结论恶黑的组织病理结构和形态变异复杂，细胞类型多，少色素、无色素及核仁模糊占大多数，有不同的病理类型，有时诊断很困难，应特别注意与多种类型肿瘤相鉴别。     

内淋巴囊肿瘤的临床病理分析

目的 探讨内淋巴囊肿瘤的临床病理学特征及诊断、鉴别诊断要点。方法 对3例内淋巴囊肿瘤病例进行临床病理分析、PAS及免疫组织化学染色。结果 内淋巴囊肿瘤的临床症状包括进行性加重感音神经性聋、耳鸣、眩晕、颅神经受累等症状。镜下瘤组织呈乳头状囊性腺样结构，其内可见粉染蛋白样物质。部分瘤细胞的胞质透明，核的形态及大小相近，异型性较少见。肿瘤问质为大量增生的纤维结缔组织及透明变性的胶原纤维，可见出血、噬含铁血黄素细胞、异物巨细胞及胆固醇结晶。免疫表型：CK、NSE阳性，GFAP、S-100蛋白、EMA、CgA、TG、Syn阴性。结论 内淋巴囊肿瘤是罕见的肿瘤，其诊断和鉴别诊断主妻靠病理组织学和免疫组织化学。     

浅表性脂肪瘤样痣9例临床病理学观察

目的探讨浅表性脂肪瘤样痣的临床病理特征、鉴别诊断。方法分析9例浅表性脂肪瘤样痣临床资料、组织学特征，对其中6例进行特殊染色并结合文献进行讨论。结果本组男5例、女4例，发病年龄1～42岁。临床上，7例为多发(经典)型，2例为局限型。皮损分布于臀部、尾骶部、大腿后上方、头部和腘窝，呈群集状丘疹和结节。病理特征为在真皮胶原组织内见成熟无包膜的脂肪细胞团，特殊染色示胶原纤维增厚、弹力纤维减少。结论浅表性脂肪瘤样痣的诊断依赖临床病理的特征，在组织形态上浅表性脂肪瘤样痣应与Go1tz综合征、结缔组织痣鉴别。     

涎腺腺泡细胞癌临床病理分析并文献复习

目的：探讨涎腺腺泡细胞癌的临床病理学特点及诊断要点。方法：对1例涎腺腺泡细胞癌进行临床资料、病理形态学及免疫组织化学观察,并结合文献对其诊断及鉴别诊断进行探讨。结果：镜下瘤细胞胞体宽大,胞浆嗜碱性呈细颗粒状,核圆形瘤细胞生长呈腺泡状或实性片状生长,部分区域呈乳头状改变,免疫组化显示AE1/AE3(+)、CK8/18(+)、CK7(+)、AAT(+)、S-100(+)。结论：涎腺腺泡细胞癌发病率低,但根据其常见的发病部位及特征性的组织形态,结合免疫组织化学方法,有助于其诊断及鉴别诊断。     

肺血管瘤样纤维组织瘤临床病理分析并文献复习

目的：探讨肺血管瘤样纤维组织细胞瘤(angiomatoid fibrous histiocytoma,AFH)的临床特点、组织病理学及分子病理学特征。方法：收集1例肺AFH,进行光镜观察、免疫组织化学染色及EWSR1基因检测并复习相关文献。结果：镜下观肿瘤周围可见不完整纤维性假包膜,并见不等量淋巴细胞和浆细胞浸润,在肿瘤周围形成淋巴组织套;肿瘤内见散在假血管性腔隙;肿瘤细胞呈漩涡状、束状、席纹状排列,细胞核呈梭形、圆形和卵圆形,有一定异型性,罕见核分裂象与坏死。免疫表型：肿瘤细胞Vimentin和CD99均呈弥漫阳性,Ki-67增殖指数为5%。结论：血管瘤样纤维组织细胞瘤是一种罕见的交界性肿瘤,其诊断主要依靠病理形态学特征,免疫组织化学标记及FISH检测EWSR1基因有助于诊断和鉴别诊断;局部适当扩大切除,术后随访患者是主要处理原则。     

Compound nevus with congenital features and balloon cell changes--an immunohistochemical study

Balloon cell nevi and balloon cell changes in nevi have rarely been reported in the literature. We describe a compound nevus showing focal balloon cell changes in a 20-year-old man. The melanocytic nature of the balloon cells is confirmed by an immunohistochemical study. The findings are compared with those in balloon cell melanomas.     

易混淆的膀胱活检小细胞性浸润性尿路上皮癌的诊断与鉴别诊断：附1例报告

目的：探讨膀胱活检中小细胞性浸润性尿路上皮癌的病理形态学特征以及与其它小细胞性病变的鉴别诊断。方法：对北京中医药大学东直门医院1例膀胱黏膜活检的病理及临床资料进行回顾性分析,并对相关国内外文献进行复习。结果：患者,男性,82岁,膀胱镜下见：在膀胱颈部10~12点可见黏膜突起如蕈状,蒂不明显。活检组织光镜下见：1)尿路上皮下小细胞肿瘤细胞呈弥漫性及巢状分布,间质增生;2)瘤细胞体积小,呈短梭形或淋巴细胞样,胞浆极少,局部瘤细胞胞浆宽广透明;3)核为圆形、椭圆形或梭形,核深染且结构不清,部分核不规则,可见双核、核重叠及多核瘤巨细胞,核仁不明显;4)未见核分裂象;5)局部组织可见挤压现象及局灶凝固性坏死;6)可见脉管浸润;7)局部异型增生的尿路上皮基底层处与固有膜内的小细胞性肿瘤成分有移行。免疫组织化学结果显示免疫表型CK少数细胞弱(+),其余标记均(?),病理诊断为：小细胞低分化癌。临床行经尿道膀胱肿瘤电切术,未送病理,数月后发现肠系膜多个转移瘤结节,瘤细胞形态与活检相似,局部瘤细胞核偏位,可见核仁及病理性核分裂象,免疫组化显示上皮性标记、p63及CD44V6均(+),神经内分泌标记(?),综合考虑最后病理诊断为：膀胱小细胞性浸润性尿路上皮癌伴肠壁转移。结论：膀胱小细胞性浸润性尿路上皮癌在膀胱镜活检标本中诊断难度较大,应重视其病理特点,鉴别诊断需结合临床和免疫组化特征综合评价,当二者不能提供有价值的帮助时,确诊还需以HE切片形态学特征为主,同时在报告中加以注明。膀胱镜活检标本病理诊断尿路上皮癌核分裂象应是有或无。     

Patient age in Spitz nevus and malignant melanoma: implication of Bayes rule for differential diagnosis

In the differential diagnosis of Spitz nevus vs malignant melanoma, patient age provides a critical piece of clinical information, because Spitz nevi occur mostly in children and melanomas occur mostly in adults. Nevertheless, there is overlap in the age distributions of Spitz nevus and melanoma. The issue to consider is how these age distributions and their governing probability densities can impact the a priori probability that a lesion is a Spitz nevus vs a melanoma. Herein I introduce a quantitative approach that uses Bayes rule together with previous published data on the age distributions in Spitz nevi and melanoma. The resulting algorithm yields plots and a table of predictive a priori probabilities of Spitz nevus, given patient age occurring within narrow intervals, and I believe these provide useful guidelines for using age in the differential diagnosis of Spitz nevus and malignant melanoma.     

Balloon cell nevus of the iris

Balloon cell nevus is a rare histopathological lesion characterized by a predominance of large, vesicular and clear cells, called balloon cells. There is only 1 case of balloon cell nevus of the iris reported in the literature.Case reportA 55 year-old man presented a pigmented elevated lesion in the right iris since the age of 12 years old. The lesion had been growing for the past 2 years and excision was performed. Histopathological examination showed a balloon cell nevus composed of clear and vacuolated cells without atypia. A typical spindle cell nevus of the iris was also observed. The differential diagnosis included xanthomatous lesions, brown adipocyte or other adipocytic lesions, clear cell hidradenoma, metastatic clear cell carcinoma of the kidney and clear cell sarcoma. The tumor was positive for Melan A, S100 protein and HMB45.ConclusionBalloon cell nevus of the iris is rare but should be considered in the differential diagnosis of melanocytic lesions of the iris.     

Updates on molecular diagnostic assays in melanocytic pathology

Melanoma is the leading cause of death among cutaneous neoplasms. For the great majority of melanocytic tumors histopathologic examination can reliably distinguish nevi from melanomas; however, there is a subset of melanocytic neoplasms that defy an accurate diagnosis using histopathological criteria alone. For such tumors, additional ancillary tests would be beneficial in adjudicating a more definitive diagnosis. Conventional ancillary studies such as immunohistochemistry have only a limited utility in distinguishing an atypical nevus from melanoma. In recent years few molecular ancillary tests have been developed to help guide the diagnosis of ambiguous melanocytic proliferations. These tests are based either on the identification of genomic copy number changes or on the differential expression pattern of selected genes in nevi and melanomas. This review will present an updated overview of the major ancillary tests used in clinical practice for the diagnosis of challenging melanocytic neoplasms.     

Hypopigmentierte spindelzellige melanozytäre Tumoren

Hypopigmented and achromatic melanocytic tumors with spindle cells represent a diagnostic challenge. Spindle cell nevi resemble neural tumors. Desmoplastic nevi imitate dermatofibromas. Hypopigmented and amelanotic blue nevi are variants of the common and cell-rich blue nevus with an enhanced difficulty to make a correct diagnosis due to the lack of pigment. All of the above benign melanocytic tumors with proliferations of hypopigmented spindle cells can more or less show aspects of desmoplastic melanoma. The differential diagnosis of these entities demands a combination of clinical and histological parameters as well as supporting immunostaining. Regarding desmoplastic melanoma, diagnoses frequently made are benign spindle cell neoplasms, scar or unspecific inflammatory condition. As the histological aspects can be so misleading, attention is necessary in order to make the correct diagnosis. Particular care must be taken to rule out desmoplastic melanoma in the case of spindle cell proliferations, a cicatricial or inflammatory process in the classical settings of face, volar skin, or mucous membranes. The spindle cell melanoma must be distinguished from other types of malignant spindle cell neoplasms, which can involve the skin. The differential diagnosis with such tumors is entrusted mainly to immunostaining.     

儿童特殊类型肾母细胞瘤5例临床病理学分析

目的：探讨儿童特殊类型肾母细胞瘤的临床病理学特点、诊断要点及鉴别诊断。方法：收集5例儿童特殊类型肾母细胞瘤病例进行临床资料、病理形态学及免疫组织化学分析,并结合文献探讨其诊断与鉴别诊断。结果：5例患儿中男童3例,女童2例,年龄4月~2岁,中位年龄1岁,主因腹部包块、无痛性肉眼血尿或腹胀、发热就诊,左肾(3例)多于右肾(2例)。其中部分囊状分化型3例,镜下见肿瘤由大小不一的囊腔构成,内衬扁平、立方或鞋钉样上皮细胞,间隔见间叶、胚芽或上皮的肾母细胞瘤成分;肾母细胞瘤伴肾源性残余2例,镜下见由间叶、胚芽和上皮等构成的典型肾母细胞瘤成分,周围见胚芽和上皮细胞与正常肾组织混合存在。结论：部分囊状分化的肾母细胞瘤(cystic partially differentiated nephroblastoma,CPDN)、肾母细胞瘤伴肾源性残余是少见特殊类型的肾母细胞瘤,术前临床诊断及鉴别诊断困难,只有依靠术后病理作出准确诊断,为患儿治疗及预后提供可靠依据。     

Blue nevus of the colorectal mucosa

The blue nevus is a well-described benign melanocytic proliferation that generally occurs on the skin. Infrequently, blue nevi are found on mucosal surfaces. The most common location for mucosal blue nevi is the oral mucosa, with reported cases in the sinonasal mucosa and genital tract, as well as in other locations. To our knowledge, blue nevi of the rectal mucosa have not been described. Here, we describe a case of blue nevus arising in the rectal mucosa. Blue nevi are benign melanocytic proliferations with the potential for malignant transformation and should be included in the differential diagnosis of pigmented mucosal lesions of the rectum.