婴儿色素性神经外胚瘤1例报道并文献复习

目的 探讨婴儿色素性神经外胚瘤的临床病理特征、免疫组化、诊断和鉴别诊断要点。方法 对1例婴儿色素性神经外胚瘤进行组织学和免疫组化观察和文献复习。结果 婴儿色素性神经外胚瘤好发于1岁以内的婴儿,肿瘤多见于上颌骨和颅骨,表现为浸润性和溶骨性破坏。组织学上显示大的并含不等量色素颗粒的上皮样细胞和小的神经母细胞样细胞。免疫组化显示CK、HMB-45、S-100蛋白、NSE在上皮样细胞呈阳性表达,小圆形瘤细胞S-100蛋白、NSE阳性或部分阳性。肿瘤彻底切除,随访3年未发现转移和复发。结论 婴儿色素性神经外胚瘤是一种少见的起源于神经嵴细胞的肿瘤,具有特征性的临床病理改变,需要和神经母细胞瘤、恶性黑色素瘤及其它小圆细胞肿瘤鉴别,生物学行为属于潜在恶性或低度恶性肿瘤,彻底切除预后良好。     

胎盘转移性神经母细胞瘤1例并文献复习

胎盘转移性肿瘤可分为由胎儿转移至胎盘的肿瘤和由母体转移至胎盘的肿瘤两类,十分少见,由胎儿转移至胎盘的神经母细胞瘤就更为罕见,现报道1例并结合文献复习,就其临床和病理特点及病理诊断和鉴别诊断进行探讨。1　临床资料患者女,2 6岁,孕7个月,胎动消失1个月就诊。查体:宫底脐上一指,胎心音消失;外阴经产式;继往身体健康,否认肿瘤病史。生育史G2P1,笫1胎死胎,此为第2胎。入院后引产娩出一死胎,部分皮肤坏死、脱落并感染。大体解剖左侧中腹部腹膜后有一5 0cm×4 5cm×4 5cm实性包块,有部分包膜,切面灰红色,质嫩。胎盘大而不规则,仅送胎…     

神经内神经束膜瘤16例临床病理学分析

目的 探讨神经内神经束膜瘤(intraneural perineurioma, InP)的临床病理学特征。方法 回顾性分析16例InP的临床病理学及免疫表型特征,并复习相关文献。结果 16例InP患者中男性5例,女性11例,年龄5～56岁,平均年龄27.4岁,14例为单发病变(分别位于尺神经、正中神经、桡神经、腓总神经),2例为多发病变(分别位于双侧臂丛神经及C1-3神经根)。患者术前病程2～360个月,常表现为病变神经对应区域缓慢进展的运动功能障碍,部分有感觉功能异常;影像学提示局部神经增粗呈纺锤形或长梭形,边界尚清,肌电图提示周围神经损伤;光镜下见梭形的神经束膜细胞以轴突-施万细胞复合体为中心形成假洋葱球样结构。电镜下见长而细的神经束膜细胞胞质内富含吞噬囊泡,与其伴随的施万细胞一起围绕轴突呈同心圆状排列。免疫表型：神经束膜细胞EMA、Glut-1、Claudin-1阳性,中心的轴突-施万细胞复合体NF、S-100及SOX10阳性。Ki-67增殖指数<5%。结论 InP是一种罕见的起源于神经束膜的良性肿瘤,易与肥大性周围神经病、腓骨肌萎缩症、混杂性神经鞘膜肿瘤、神经鞘瘤、神经纤...     

软组织恶性混合瘤/肌上皮癌1例并文献复习

目的 探讨软组织恶性混合瘤/肌上皮癌(malignant mixed tumor/myopeithelia carcinoma,MMTMC)的临床病理特征、免疫表型、鉴别诊断及预后.方法 对1例原发于大腿的软组织MMTMC进行HE和免疫组化染色,并复习相关文献.结果 患者男性,75岁,3个月前发现右大腿后部占位,进行性...     

Askin瘤尸检1例并文献复习

目的 探讨Askin瘤的临床病理特点、诊断及鉴别诊断.方法 总结1例Askin瘤患者的临床资料和尸检病理学特点;通过光镜和免疫组化及网状纤维染色,对尸体解剖后进行组织病理学观察.结果 临床上表现为青少年胸肺部肿块伴疼痛,患者生存期短,预后差.病理组织学特征主要为肿瘤组织呈弥漫性或结节状分布.组织学上以稀少的纤维血管间质将形态单一的小圆、卵圆形瘤细胞围绕成巢状或小叶状结构.免疫组化显示:瘤细胞CD99(+),NSE弱(+),CgA弱(+).网状纤维染色示肿瘤细胞间网状纤维少.结论 Askin瘤为一种位于胸肺部神经内分泌源性的高度恶性肿瘤,它实际上是PNET/Ewing肉瘤家族的一员,只是发生部位较特殊,预后不良.     

软组织炎性肌纤维母细胞瘤1例报道及文献复习

患者女，26岁。右膝上方感到酸痛肿胀2个月就诊。查体：右膝关节上方偏内侧，局部肿胀，表面无红肿，深部可触及直径4cm的肿块，边界欠清，不活动，余未见明显异常。CT及MRI提示软组织良性肿瘤，但不排除低度恶性肉瘤。ECT显示肿瘤内血运丰富，提示肌间血管瘤。彩超检查提示软组织肉瘤。局部肿块穿刺，抽出不凝固血液，涂片检查，未见异型细胞。而后行肿块扩大切除。     

长骨原发性神经鞘瘤3例并文献复习

目的探讨长骨原发性神经鞘瘤临床、病理、影像学特征、鉴别诊断、治疗及预后。方法采用HE染色及免疫组化EnVision法分析3例原发于长骨的神经鞘瘤病理学、免疫表型,并复习相关文献。结果镜下见交替分布的Antoni A区和Antoni B区,并见出血及含铁血黄素沉着、坏死、间质黏液样变、不规则的血管伴血管壁增厚及胶原变性、血管周见泡沫样组织细胞反应及淋巴细胞浸润、肿瘤细胞破坏周围骨质等现象;未见明显的细胞异型及核分裂象。免疫表型:CD56、vimentin、S-100蛋白均呈弥漫强阳性,Ki-67增殖指数2%~7%。结论发生于长骨内的神经鞘瘤临床罕见,对产生溶骨性破坏者需结合临床、影像、病理学及免疫表型进行鉴别诊断,避免误诊为骨源性低度恶性肿瘤而导致过度治疗。     

血管瘤样纤维组织细胞瘤1例报道并文献复习

血管瘤样恶性纤维组织细胞瘤(angiomatoid malignant fibroos histocytona,AMFH)是一种少见的软组织肿瘤,也有文献称之为血管瘤样纤维组织细胞瘤.WHO(1994)将AMFH确认为中间型(低度恶性)纤维组织细胞肿瘤,其临床及病理学特点不同于普通类型恶性纤维组织细胞瘤,易误诊.笔者报道1例AMFH的光镜及免疫组化观察结果,并结合文献讨论其临床病理特征及诊断与鉴别诊断.     

卵巢多胚瘤1例报道并文献复习

卵巢多胚瘤是一种极其罕见的原始生殖细胞肿瘤,临床特征不明显,易误诊．我们报道1例卵巢多胚瘤结合文献复习,对其病理形态、免疫表型特点、诊断和鉴别诊断进行探讨,为临床和病理医师正确认识该肿瘤提供帮助。[第一段]     

胃肠道神经束膜瘤4例临床病理分析

目的探讨胃肠道神经束膜瘤的临床病理特征。方法分析4例胃肠道神经束膜瘤的临床资料,总结其临床病理和免疫表型。结果 1例位于胃体大弯,1例位于结肠,2例位于直肠;瘤体直径0.3～1 cm,其特征是黏膜固有层内见温和的梭形细胞增生,表达神经束膜标志物,梭形细胞呈层状、束状排列,或围绕腺体漩涡状分布,周围扩张的隐窝常伴锯齿状结构。免疫表型:温和的梭形细胞不同程度表达EMA和CD34。随访4例患者均未见肿瘤复发和转移。结论胃肠道神经束膜瘤是一种良性的外周神经鞘膜肿瘤,可能被误诊为其他更常见的胃肠道梭形细胞肿瘤。免疫组化标记EMA和CD34阳性有助于鉴别诊断,患者预后良好。     

Atypical soft tissue perineurioma in the tongue of a young girl

Perineuriomas are uncommon benign peripheral nerve sheath tumors that include soft tissue, sclerosing, reticular, and intraneural variants. Soft tissue perineuriomas arise in a wide anatomic distribution and mostly in patients older than 20 years of age. We report an atypical perineurioma in a 7-year-old girl. The tumor, located in the tongue, was uniformly hypercellular. The tumor cells were spindle-shaped with a slender, elongated, bipolar, wavy cytoplasmic process formation and wavy elongated nuclei, and the architecture was composed of predominantly short fascicles with areas exhibiting a vague storiform pattern. Although the tumor cells generally appeared bland, the tumor showed worrisome features including an infiltrative pattern and occasional mitotic figures. Psammoma bodies were observed in the periphery of the tumor. Immunohistochemically, the cells were positive for epithelial membrane antigen, vimentin, claudin-1, and GLUT-1, but negative for S-100 protein, CD34, and type IV collagen. The authors document a case of soft tissue perineurioma with atypical histological features that occurred in the tongue of a child.     

Intraneural malignant perineurioma: a case report and review of literature

The great majority of malignant peripheral nerve sheath tumors (MPNSTs) exhibit Schwann differentiation. Few MPNSTs with perineurial differentiation are also named malignant perineuriomas. Benign perineurioms were classified as intraneural, extraneural (soft tissue), sclerosing, and reticular variant. Histopathological features of intraneural perineurioma are individual nerve axons surrounded by whorls of spindle-shaped cells arranged in an onion bulb-like pattern. However, intraneural malignant perineurioma is uncommon, its characteristic histological features were not clearly described yet. Positive for epithelial membrane antigen (EMA), glut-1 and claudin-1, is characteristic of malignant perineurioma. Herein, we report an intraneural malignant perineurioma in median nerve of a 13-year-old girl. The clinicopathological features were summarized and the related literatures were reviewed.     

硬化性上皮样纤维肉瘤临床病理特点及其诊断

目的探讨硬化性上皮样纤维肉瘤(sclerosing epithelioid fibrosarcoma,SEF)的临床病理学特征、诊断及鉴别诊断。方法对1例SEF进行组织学观察和免疫组化标记,同时复习相关文献。结果SEF主要以上皮样细胞伴大量胶原纤维为特征,瘤细胞胞质透亮,似上皮细胞,排列呈条索状,间质中大量胶原纤维发生透明变性,形成硬化性基质。肿瘤细胞vimentin阳性,cytokeratin和actin阴性。结论SEF是一种较为罕见的软组织肿瘤,应注意与一些具有上皮样形态和硬化性间质的肿瘤相鉴别。     

Capillary hemangioblastoma of soft tissue: report of a case and review of the literature

Capillary hemangioblastoma (CH) is a tumor of unknown histogenesis that arises primarily in the posterior cranial fossa, either as a sporadic event or in association with von Hippel-Lindau disease. To date, only 6 examples of a tumor with morphological features of CH arising in the somatic soft tissues have been documented in case reports and small series, and 3 of these tumors were associated with a peripheral nerve. Herein, we report a case of CH arising in the gastrocnemius muscle and not associated with a peripheral nerve in a 53-year-old woman with no clinical stigmata or family history of von Hippel-Lindau disease.     

Pleomorphic hyalinizing angiectatic tumor of soft parts: a case report and literature review

Pleomorphic hyalinizing angiectatic tumor (PHAT) is a rare, recently recognized neoplasm occurring predominantly in the subcutaneous tissue of the lower limbs of adults. We report a case of PHAT in an 83-year-old woman who presented with a 5.0 x 5.0 x 2.0 cm mass in the soft part of her left thigh. Histologically, the tumor was well circumscribed by a thin fibrous capsule and predominantly composed of fusiform cells with eosinophilic cytoplasm and round-to-oval or pleomorphic nuclei. The tumor cells resembled those of malignant fibrous histiocytoma, but differed from them by less prominent mitotic figures. Immunohistochemically, the tumor cells were diffusely and strongly positive for CD34; partially positive for vimentin and CD99 (MIC-2); and negative for epithelial and non-epithelial markers. Ultrastructurally, the tumor cells had pleomorphic cytoplasm and nucleus. Intermediate-sized cytoplasmic filaments were observed in a few tumor cells, but neurosecretory-type granule-like intracytoplasmic organelles were not seen. These findings suggest that this tumor is derived from stromal fibroblast, such as solitary fibrous tumors or giant cell angiofibroma.     

富于细胞性血管脂肪瘤的临床病理学观察

目的 探讨富于细胞性血管脂肪瘤的临床病理学特点、免疫学表型和鉴别诊断.方法 回顾性复习2例富于细胞性血管脂肪瘤的临床资料和组织学形态,行网状纤维染色,并采用CD31、CD34、FⅧRAg、UEA-1、α-SMA和vimentin等抗体进行免疫组化标记(EnVision法).结果 患者均为男性,年龄分别为38岁和33岁.临床上分别表现为左上臂皮下(例1)、躯干和四肢皮下(例2)多发性小结节,部分结节有疼痛感和触痛感.大体上,结节均有包膜,直径为1～1.5 cm.镜下,例1中的1枚结节呈经典的血管脂肪瘤形态,另2枚结节主要由大量的血管组成,而脂肪组织较少(<5%).网状纤维染色清晰显示丰富的血管结构.部分小血管内可见纤维素性血栓.例2中的2枚结节主要由大量的血管和丰富的梭形间质细胞组成,脂肪组织仅占5%和10%.免疫组化标记显示,富于小血管的区域弥漫强阳性表达CD31、CD34、FⅧRAg、UEA-1和α-SMA,血管之间的梭形间质细胞主要表达vimentin.结论 富于细胞血管脂肪瘤是血管脂肪瘤的一种极为罕见的亚型,可被误诊为梭形细胞脂肪瘤、梭形细胞血管瘤、富于细胞性血管瘤和Kaposi肉瘤等肿瘤.     

背部皮肤软组织放线菌病一例并文献回顾

目的探讨皮肤软组织放线菌病的临床表现、诊断、鉴别诊断及治疗，提高创面外科医师对皮肤软组织放线菌病的认识。 方法报道1例背部皮肤软组织放线菌病的诊治经过，分别以"放线菌、放线菌感染、皮肤、软组织"及"actinomycosis，cutaneous，soft tissue"为检索词检索中国知网和PubMed的相关文献并进行分析。 结果与所检索20篇文献相比，本例皮肤放线菌病的临床表现、鉴别诊断及治疗既典型又具自身特点。本例患者入院时并未表现出明显皮肤肿物，但皮下感染组织内多个窦道，伴较多土褐色脓液流出较为典型。本例患者入院第9天才培养出放线菌，但无论是术中肉眼观察、细菌培养还是组织病理学检查均未见典型"硫磺颗粒"。本例患者查体、术中所见及辅助检查结果和气性坏疽的临床表现高度相似，因此最初曾高度怀疑产气荚膜梭菌感染，但多次细菌培养及涂片均未提示产气荚膜梭菌感染。治疗主要包括全身状态的调整、手术清创及抗生素的应用。 结论原发于皮肤软组织的放线菌病较少见，临床表现多样，即便有相对典型特征，也并无特异性，诊断需综合考虑并鉴别诊断。皮肤软组织放线菌感染发病率低，不易诊断，需引起创面外科医师的注意，及早对本病作出诊断及治疗。     

Cutaneous sclerosing Pacinian-like perineurioma

AIMS: The term perineurioma has been used to designate a variety of clinically and histologically different proliferations of perineurial cells based on immunohistochemical and/or ultrastructural characterization. There are two different groups of neoplasms derived from perineurial cells: extraneural or soft tissue perineuriomas, and intraneural perineuriomas. Recently, a sclerosing variant of cutaneous perineurioma has been described. METHODS AND RESULTS: We report a case of a cutaneous form of perineurioma, combining features of the intraneural and sclerosing varieties, as well as showing a Pacinian pattern of growth. In order to assess the neoplastic nature of the lesion, we performed fluorescence in-situ hybridization (FISH) analysis using a probe which maps to the chromosome band 22q11 and 22q13, allowing us to show deletion or loss of one chromosome 22 in the tumour cells. CONCLUSIONS: This case may be considered a new variant of perineurioma with Pacinian-like features, for which we propose the designation 'sclerosing Pacinian-like perineurioma'.     

侵袭性血管黏液瘤7例临床病理分析

目的探讨侵袭性血管黏液瘤(aggressive anginomyxoma,AAM)的临床病理特征与鉴别诊断。方法收集7例AAM,并与2例血管肌纤维母细胞瘤(AMFb)、1例浅表性血管黏液瘤作对照观察。免疫组织化学(采用SP法)所用抗体为vimentin、desmin、SMA、MSA、CD34、ER/PR、S-100蛋白、CK和CD68。结果7例AAM患者均为女性,23~54岁,中位年龄43·5岁。病变部位位于外阴部3例,累及阴道1例,位于盆腔及髂窝部2例,会阴累及坐骨结节1例。肿瘤直径3·5~17cm,平均7·5cm。位于皮下或较深部,呈浸润性生长,切面均质灰白或灰黄褐色,黏液胶冻样或肉质样。镜下瘤细胞幼稚,呈星形、梭形,弥漫或结节样分布,基质黏液样变性并见胶原纤维及为数众多的大小厚薄不一的血管分布其中,厚壁血管常透明变性,其周围常有增生肌纤维束,切面呈“袖套状”,间质可见肥大细胞和红细胞外渗,1例可见多核巨细胞,肿瘤边缘浸润等特点。组织化学奥辛蓝染色阳性,免疫组化:7例AAM vimentin均阳性,5例SMA和desmin阳性,4例MSA阳性,3例CD34阳性,4例中有3例ER/PR阳性,而S-100蛋白、CK、CD68均阴性,多核巨细胞CD68阳性。5例有随访,术后1~2年有3例复发,2例未见复发和转移,健在。结论侵袭性血管黏液瘤较为少见,好发于成年女性盆腔及会阴的软组织,侵袭性和复发性是其重要的临床特征。病理诊断易误诊或漏诊。免疫组化desmin、SMA、MSA、CD34等联合检测对确定AAM病变有所帮助,但与AMFb、浅表性血管黏液瘤在鉴别诊断上意义不大。     

A benign neoplasm with histopathological features of both schwannoma and retiform perineurioma (benign schwannoma-perineurioma): a report of six cases of a distinctive soft tissue tumor with a predilection for the fingers

We present six cases of a distinctive soft tissue tumor which occurred in five women and one man. None of the patients had signs of neurofibromatosis. All tumors occurred on the fingers (n=5) or the thenar eminence of the hand (n=1). The mean age of the patients was 33 years. The tumors were 1–2.5 cm in diameter (mean size 1.6 cm). Three patients with follow-up were without signs of recurrence or metastasis. Microscopically the lesions were nonencapsulated and featured a multilobular architecture and both myxoid and pseudocystic change. The lobules varied in size and shape and were separated by variably thickened, dense, sclerotic/collagenous septae. The lobules were composed of two components: schwannomatous and perineuriomatous. The schwannomatous component was immunohistochemically S-100 protein positive and CD34 and EMA negative, and the perineuriomatous component had the appearance of retiform perineurioma. The perineurial parts were mostly S-100 protein and CD34 negative and EMA positive. These two components either formed separate nodules or the schwannomatous tissue surrounded the perineurial parts located in the centers of the lobules. We interpreted the lesions as hybrid tumors with features of schwannoma and retiform perineurioma.