盆腹腔上皮样炎性肌纤维母细胞肉瘤的临床病理研究

目的:为了探讨盆腹腔上皮样炎性肌纤维母细胞肉瘤的临床特点、诊断、治疗和预后,对其病理学特点进行研究分析。方法:选取2018年06月我院接诊且病理科病理诊断为盆腹腔上皮样炎性肌纤维母细胞肉瘤的1例患者作为研究对象,分别通过巨检、镜检、免疫表型以及免疫组织化学的方法对病理标本进行分析,并通过查阅相关文献,复习相关文献,对盆腹腔上皮样炎性肌纤维母细胞肉瘤的临床特点做进一步的研究。结果:镜下见肿瘤细胞排列紊乱,连成片状,大小不一,形态多样。主要组成为上皮样细胞,肿瘤细胞形状呈圆形、短梭形、多边形、椭圆形及不规则形。细胞排列较为松散,弥散分布。肿瘤细胞核仁较大,镜下可见核分裂象和瘤巨细胞;间质中见明显的大量炎症细胞浸润,炎性浸润以中性粒细胞为主,伴有少量浆细胞、淋巴细胞及嗜酸性粒细胞;胞质丰富,肿瘤细胞有明显水肿和黏液样变性。部分癌变区域排列较为密集,细胞形态不清。肿瘤细胞免疫组化结果vimentin、desmin、核膜ALK、CD30弥漫性表达强阳性,而ALK特异性的在细胞核膜阳性表达。部分肿瘤细胞EMA、INI-1、AAT、SMA、CK部分、p53部分阳性。FISH检测结果显示肿瘤细胞多有单一红色信号及红绿分离信号,多为阳性。结论:盆腹腔上皮样炎性肌纤维母细胞肉瘤发病较为罕见,是高度恶性肿瘤,预后较差。但是其病理学和免疫组化特点较为突出,根据病理学结果更有利于临床诊断。     

子宫不典型平滑肌瘤的超微结构观察

目的 了解电镜下子宫不典型平滑肌瘤细胞的超微结构特点.方法选择光镜诊断的4例子宫不典型平滑肌瘤组织标本,在电镜下观察细胞的超微结构.结果 子宫不典型平滑肌瘤的细胞排列不规则;细胞核大、核的形态极不规则、核切迹很深;核内染色质明显、可见核小体;核仁粗大明显、部分呈网状或同心圆层状分布;胞质内的细胞器较丰富、粗细肌丝排列紊乱、无明显密斑密体.结论 子宫不典型平滑肌瘤起源于平滑肌细胞,是平滑肌瘤的异常表型,呈现细胞分化不成熟和合成功能增强的恶性超微病理特点.     

促纤维增生性恶性黑色素瘤临床病理分析

[目的]总结促纤维增生性恶性黑色素瘤的临床病理学特征。[方法]对5例促纤维增生性恶性黑色素瘤术后标本行HE、免疫组织化学(En Vision法)染色、电镜观察及随访。[结果]光镜下见肿瘤性梭形黑色素细胞位于成熟的胶原束内或之间,梭形肿瘤细胞排列混乱,肿瘤细胞胞质内几乎未见黑色素。免疫组化示肿瘤细胞S-100、NSE及SMA阳性。电镜示成纤维细胞样的肿瘤细胞。[结论]促纤维增生性恶性黑色素瘤是一种罕见疾病,临床易与其它梭形细胞肿瘤混淆,特征性的病理组织学及免疫组织化学特征有助于诊断及鉴别诊断。     

浅表宫颈阴道肌纤维母细胞瘤临床病理学分析

目的:探讨浅表宫颈阴道肌纤维母细胞瘤(superficial cervicovaginal myofibroblastoma,SCVM)的临床病理学特点、免疫表型、超微结构以及鉴别诊断。方法:对1例发生于阴道的SCVM进行组织学观察、免疫组化标记及电镜观察。结果:患者,女,48岁。在无任何症状下常规体检时妇检发现阴道前壁有一包块。临床诊断为囊肿。肿瘤大小3.5cm×2.5cm×2cm,境界清楚,无包膜,切面灰白色,胶冻状。镜下见瘤细胞形态较一致,呈梭形或星形,胞质红染,细胞核圆形或椭圆形。细胞的排列结构多样,最常见的为束状和席纹状排列。细胞异型性不明显,核分裂象<1/10HPF,不见坏死。散在有炎细胞浸润,主要为肥大细胞。肿瘤内散布有薄壁血管,在中央区较周边区密集,血管周围没有细胞聚集现象,血管壁无玻璃样变性。免疫组化标记显示瘤细胞弥漫性表达vimentin、desmin、ER、PR和CD34,不表达α-SMA、S100、HMB45、CD31、CD57、CK。电镜观察见有肌纤维母细胞分化。肿瘤完整切除后随访半年无复发和转移。结论:SCVM是一种发生于女性下生殖道少见的良性软组织肿瘤。约1/3病例与他莫昔芬或激素治疗相关。免疫标记和电镜观察均显示有肌纤维母细胞分化。组织学上应与多种梭形细胞肿瘤鉴别。     

微囊型脑膜瘤临床病理、超微结构和免疫组化研究

目的：提高对颅内微囊型脑膜瘤的认识，提高诊断本领。方法；对15例颅内微囊型脑膜瘤进行光镜、超微结构和免疫组化三个方面进行了研究。结果：光镜下肿瘤细胞质淡染有长的突起，间质丰富有许多空泡状微囊。小血管多，肿瘤内无砂粒体或坏死。电镜下，肿瘤细胞胞质伸出指状突起相互围成囊腔，囊内可见束状胶原纤维丝。免疫组化，Vimetin和EMA染色阳性。结论：光镜下该型脑膜瘤具有一定特征，免疫组化有助于该瘤的诊断，电镜可以正确诊断微囊型脑膜瘤。     

浅表宫颈阴道肌纤维母细胞瘤临床病理学分析

目的：探讨浅表宫颈阴道肌纤维母细胞瘤（superficial cervicovaginal myofibroblastoma,SCVM）的临床病理学特点、免疫表型、超微结构以及鉴别诊断。方法：对1例发生于阴道的SCVM进行组织学观察、免疫组化标记及电镜观察。结果：患者,女,48岁。在无任何症状下常规体检时妇检发现阴道前壁有一包块。临床诊断为囊肿。肿瘤大小3.5cm×2.5cm×2cm,境界清楚,无包膜,切面灰白色,胶冻状。镜下见瘤细胞形态较一致,呈梭形或星形,胞质红染,细胞核圆形或椭圆形。细胞的排列结构多样,最常见的为束状和席纹状排列。细胞异型性不明显,核分裂象〈1/10HPF,不见坏死。散在有炎细胞浸润,主要为肥大细胞。肿瘤内散布有薄壁血管,在中央区较周边区密集,血管周围没有细胞聚集现象,血管壁无玻璃样变性。免疫组化标记显示瘤细胞弥漫性表达vimentin、desmin、ER、PR和CD34,不表达α-SMA、S100、HMB45、CD31、CD57、CK。电镜观察见有肌纤维母细胞分化。肿瘤完整切除后随访半年无复发和转移。结论：SCVM是一种发生于女性下生殖道少见的良性软组织肿瘤。约1/3病例与他莫昔芬或激素治疗相关。免疫标记和电镜观察均显示有肌纤维母细胞分化。组织学上应与多种梭形细胞肿瘤鉴别。     

婴儿色素性神经外胚瘤临床病理观察

目的：探讨色素性神经外胚瘤的病理形态、免疫组化及诊断和鉴别要点。对5例色素性神经外胚瘤病例进行临床病理、免疫组化及电镜观察。结果：色素性神经外胚瘤好发于1岁以内的婴儿，3例肿瘤发生在上颌骨，2例发生在附皋。镜下由两种细胞构成，即上皮样瘤细胞和小圆形似成神经细胞样瘤细胞。免疫组化示上皮样瘤细胞CK、HMB45阳性，小圆形瘤细胞NSE阳性，超微结构显示肿瘤细胞内分别可见前黑色素小体、黑色素小体和神经内分泌颗粒。结论：色素性神经外胚瘤是一种少见的原始性神经外胚层肿瘤，生物学行为属于潜在恶性或低度恶性肿瘤。     

胸腺类癌1例及文献复习

目的:探讨胸腺类癌的病理学特点及临床表现.方法:对1例胸腺类癌进行组织学表现、电镜和免疫组化染色观察及文献复习.结果:组织学特点:瘤细胞多排列为不规则巢状,条索状,伴有大量坏死,瘤细胞大小较一致,胞质弱嗜酸,颗粒状,边界不清,胞核呈圆形或椭圆.免疫组化特点:肿瘤细胞表达Syn(+)、CgA(+)、NSE(+).电镜:部分瘤细胞及突起内见有神经内分泌颗粒.结论:胸腺类癌是前纵隔一种少见的恶性肿瘤,主要与胸腺瘤相鉴别,治疗以手术切除的疗效为最好.     

胃肠道恶性神经外胚层肿瘤临床病理分析合并文献复习

目的 探讨胃肠道恶性神经外胚层肿瘤的临床病理学特点、免疫表型、分子遗传学改变、诊断及鉴别诊断要点.方法 回顾性分析胃肠道恶性神经外胚层肿瘤的临床资料、光镜观察、免疫组化标记及荧光原位杂交检测,并结合相关文献分析.结果 肿瘤位于升结肠,浸润性生长,侵犯肠壁全层.肿瘤组织弥漫成片状或巢状生长,局部区域呈腺泡状及流水样排列.肿瘤细胞呈上皮样,局部呈梭形,胞质嗜酸性或透明,细胞核圆形或卵圆形,染色质稀疏,有小核仁.核分裂象6个/10HPF.免疫组化:Vimentin(+),SOX10(+),S-100(+),Syn灶性(+),CD56(+),Fli1(+),Ki-67增殖指数20％,荧光原位杂交检测显示EWSR1基因重排.结论 胃肠道恶性神经外胚层肿瘤非常罕见,具有高度侵袭性,应与其它胃肠道原始上皮及梭形细胞肿瘤相区别.联合应用免疫表型、超微结构和分子学分析,有助于识别该肿瘤并与其他类似病变鉴别.     

扁桃体上皮样滤泡树突细胞肉瘤临床病理学分析

目的:探讨扁桃体上皮样滤泡树突细胞肉瘤(follicular dendritic cell sarcoma,FDC sarcoma)的临床病理学特点、免疫表型、超微结构以及鉴别诊断.方法:对1例发生于扁桃体的上皮样FDC肉瘤进行组织学观察、免疫组化标记及电镜观察.结果:患者女性,55岁.咽部不适感2年,近期症状加重而就诊.术中见左扁桃体肿大伴有糜烂.临床考虑为恶性淋巴瘤.镜下见瘤细胞形态较一致,由大上皮样细胞组成,未见梭形瘤细胞.瘤细胞境界不清,丰富的嗜酸性胞质,空泡状核,核仁明显.瘤细胞间散在小淋巴细胞浸润.免疫组化标记显示瘤细胞弥漫性表达vimentin、CD21、CD23、CXCL13和D2 - 40.电镜观察于瘤细胞胞浆内可见粗面内质网和线粒体,少量由桥粒连接的细胞突,明显的椭圆形核及核内的大核仁.肿块切除后随访4个月无复发及转移.结论:扁桃体上皮样亚型的滤泡树突细胞肉瘤非常罕见.诊断时应与多种具有上皮样形态的肿瘤鉴别.     

1例双侧隐睾患者腹膜后精原细胞瘤并颈部淋巴结转移病案报道及文献回顾

目的:报道1例双侧隐睾患者腹膜后精原细胞瘤并颈部淋巴结转移病例,探讨其临床病理学特征.方法: 对1例双侧隐睾患者腹膜后精原细胞瘤并颈部淋巴结转移的病例进行临床、组织病理学及免疫组化的观察.结果: 显微镜下,瘤细胞被纤维组织分隔呈巢状,间质中可见淋巴细胞浸润.瘤细胞大小基本一致,圆形或多角形,细胞胞界清楚,胞质丰富透亮,核大规则,核分裂像可见;免疫组化示肿瘤细胞表达PLAP(++)、CD117(+),CD43和CD45RO淋巴细胞表达阳性,不表达CK(-)、EMA(-)、Vimentin(-)、CHA(-)、S-100(-)、Syn(-)、HCG(-)、AFP(-).结论: 颈部淋巴结肿大患者,特别是成年男性,除了要考虑原发性病变,转移癌、淋巴瘤与神经内分泌癌外,还应考虑转移性精原细胞瘤.     

Ovarian sex-cord tumor with annular tubules. Case report with ultrastructural findings

An ovarian sex-cord tumor with annular tubules (SCTAT) and with Charcot-B?ttcher bodies in a 29-year-old woman with primary infertility, secondary amenorrhea, and without evidence of the Peutz-Jeghers syndrome was studied by light and electron microscopy. At laparotomy, a right ovarian tumor was removed, and there was no evidence of metastases. The patient has been well and disease free for a period of 3 months after surgery. The tumor was histologically composed of nests of cells arranged in complex tubules with hyaline bodies. Ultrastructurally, cells were joined by specialized junctions along their lateral adjacent borders. Microvilli and cilia were absent. Concentrically arranged membranes were seen in the cytoplasm. Charcot-B?ttcher filaments were seen in paranuclear region. The findings of Charcot-B?ttcher filament in an ovarian SCTAT support the hypothesis of the Sertoli nature of this neoplasm.     

Askin瘤18例临床病理分析

目的探讨Askin瘤的临床病理特点。方法收集南京军区南京总医院2002-05-10-2013-04-16手术或穿刺以及会诊病理确诊的18例Askin瘤患者资料,分析其临床病理和免疫组化特点。结果 18例Askin瘤患者年龄7～48岁,中位年龄20岁,平均年龄24岁,男女比例2.6∶1。临床通常表现为进行性增大的胸壁肿块,镜检见肿瘤细胞为弥漫或结节状分布的形态单一小圆细胞,胞质少,核呈椭圆形或短梭形,核染色质细颗粒状或稀疏,核分裂像易见,肿瘤内散在不规则坏死灶,部分间质内可见较多厚壁小血管或血窦样腔隙及典型的Homer-Wright菊形团结构。免疫表型,12例CD99（＋）,3例FLI-1（＋）。随访13例患者中10例死亡,生存时间3～48个月,平均生存时间8个月。结论 Askin瘤是发生在胸肺区的一种少见且高度恶性的原始小圆细胞肿瘤,CD99和FLi-1对Askin瘤的诊断和鉴别诊断具有重要参考价值。     

Extraskeletal neoplasm resembling Ewing's sarcoma.

This article reviews the pathologic features and the behavior of 39 small, round, or oval cell sarcomas occurring in the soft tissues and considered histologically indistinguishable from Ewing's sarcoma of bone. The tumors affected chiefly young adults (median age 20 years) and most commonly involved the soft tissues of the lower extremity and the paravertebral region. Microscopically, they consisted of solidly packed small, round, or ovoid cells of great uniformity, arranged in sheets or lobules separated by strands of fibrous connective tissue. The nucleus of the tumor cells contained finely divided chromatin, a distinct nuclear membrane, and frequently a minute nucleolus. The scanty ill-defined cytoplasm contained varying amounts of glycogen. Sometimes the histologic picture was dominated by a "peritheliomatous" pattern, or by large areas of necrosis or hemorrhage. Followup data ranging from 1 month to 14 years were available in 35 of the 39 cases (93%). Of these, 13 were alive and 22 had died. In the majority of the fatal cases, the clinical course was rapid; metastatic lesions developed within a few months after the primary tumor was excised. The lungs and the skeleton were the two most common sites of metastasis. Cure may be achieved by wide local excision of the tumor at an early stage of the disease, combined with radiation therapy and chemotherapy.     

嗅神经母细胞瘤15例临床病理及免疫组化分析

Objective: We studied the clinicopathological and immunohistochemical features of olfactory neuroblastoma (ONB). Methods: The clinic pathological data and immunohistochemical features of 15 cases with ONB were analyzed ret- rospectively, and the related literatures were reviewed. Results: The tumors were all located in the nasal cavity in 15 cases. According to Kadish's staging system, 7 cases were in stage A, 5 cases in stage B, and 3 cases in stage C. The morphological features showed small round or ovoid tumor cells divided into pieces or trabeculae by connective tissues which were rich in capillaries. The tumor cells had round or oval nuclei and fine chromatins and lack of cytoplasma. Flexner rosette and Homer- Wright rosette were found in some cases. Acidophilic fibrins were composed of cytoplasmic projection among tumor cells. All cases were positive for NSE, Syn, CgA, 1 case was positive for Vimentin, 2 cases were positive for S-100, while CKpan, LCA and HMB45 were all negative. Conclusion: ONB is a type of very rare malignant tumors, which could be diagnosed by pathology, and immunohistochemistry is helpful in the diagnosis and differential diagnosis.     

14例宫颈小细胞神经内分泌癌临床病理分析

目的:探讨宫颈小细胞神经内分泌癌的临床病理特征、诊断与鉴别。 方法:通过回顾性方法收集2015年1月至2017年12月共14例宫颈小细胞神经内分泌癌。分析其临床特点、病理形态特征和免疫组化标记。 结果:最常见的临床表现是接触性阴道流血流液、不规则阴道流血、腹部包块、隐痛。体格检查最常见为宫颈糜烂、宫颈肥大和萎缩。光学显微镜检查:细胞较小,大小不一,分散分布在间质血管周围,圆形或者梭形,胞浆较少而核染色较深,粗颗粒状,核仁不明显或者无核仁,核分裂象多见甚至有坏死。免疫组化检测:PCK(+)、EMA(+)、CGA(+)、CD45(-)、CD56(+)、CK8/18(+)、SYN(+)、NSE(+)、Ki-67(+,80%~95%)。 结论:宫颈小细胞神经内分泌癌确诊需联合组织病理学及免疫组织化学检查,其恶性程度高,预后差,易复发,准确的病理诊断对临床治疗具有重要的作用。     

Mucinous adenocarcinoma of the submandibular gland

A rare tumor not easily classifiable among published histologic categories for salivary gland tumors is reported. The neoplasm developed within the submandibular gland of a 78-year-old woman with invasion of the mandible and metastasis to regional lymph nodes. Histopathologically, cuboidal cells possessing clear cytoplasm and displaced round nuclei proliferated and exhibited an adenomatous pattern. Many cystic spaces surrounded by tumor cell strands were seen, mucus substance filled in the cystic spaces, and the tumor cells seemed mucus-secreting, but neither epidermoid cells nor papillary appearance could be observed. Electromicroscopically, numerous mucous droplets of low electron density were prominent in the cytoplasm, and the tumor cells had sparse irregular microvilli on the luminal surface. Mucin histochemistry, including paradoxical concanavalin A staining, revealed that the tumor cells contained neutral and acid mucins, and these were identified as class II and III mucosubstances. No other neoplastic lesion, except recurrent metastatic neck nodes, has been detected 6 years after the first examination, and it seems that the tumor is a rare primary mucinous adenocarcinoma of the submandibular gland.     

骨外软组织Ewing肉瘤的临床病理学分析

背景与目的：骨外软组织Ewing肉瘤是临床上十分罕见的高度恶性肿瘤，报告2例病例并探讨其临床病理学特征。方法：运用光镜和电镜观察、组织化学及免疫组化染色方法，并复习以往相关文献。结果：骨外软组织Ewing肉瘤瘤细胞呈小圆形，异型性明显，较少或无菊形团（Homer-wright）结构。PAS特殊染色胞质中大量阳性细小颗粒沉积。免疫组化CD99（MIC2）和Vimentin标记呈强阳性表达。电镜观察可见瘤细胞质内有少量神经内分泌颗粒及丰富的糖原颗粒分布。结论：骨外软组织Ewing肉瘤是Ewing／pPNET家族肿瘤的一个亚型。骨外软组织Ewing肉瘤具有高度恶性、易复发和转移的临床特点，若出现神经分化的特点可能是具有更高侵袭性和不良预后的标志。     

1例双侧隐睾患者腹膜后精原细胞瘤并颈部淋巴结转移病案报道及文献回顾

目的：报道1例双侧隐睾患者腹膜后精原细胞瘤并颈部淋巴结转移病例,探讨其临床病理学特征。方法：对1例双侧隐睾患者腹膜后精原细胞瘤并颈部淋巴结转移的病例进行临床、组织病理学及免疫组化的观察。结果：显微镜下,瘤细胞被纤维组织分隔呈巢状,间质中可见淋巴细胞浸润。瘤细胞大小基本一致,圆形或多角形,细胞胞界清楚,胞质丰富透亮,核大规则,核分裂像可见;免疫组化示肿瘤细胞表达PLAP（＋＋）、CD117（＋）,CIM3和CIM5RO淋巴细胞表达阳性,不表达CK（-）、EMA（-）、Vimentin（-）、CHA（-）、S-100（-）、Syn（-）、HCG（-）、AFP（-）。结论：颈部淋巴结肿大患者,特别是成年男性,除了要考虑原发性病变．转移痛、淋巴瘤与神绎内分泌痛外．还席考虑转移件精原细胞瘤．