Elastofibroma: a histochemical, immunohistochemical, and ultrastructural study of two patients

Elastofibroma is a rare neoplasm. In this article, we report our study of the pathogenesis of fibrosis in elastofibroma. Three tumors obtained from two patients were selected. One patient was a 57-year-old Japanese woman who had a bilateral tumor, and the other patient was a 83-year-old Japanese man. All tumors occurred in the infrascapular region. Macroscopically, the cut surface of all tumors showed a poorly defined and whitish mass with yellowish foci. Microscopically, the tumor consisted of collagen fiber bundles, abnormal elastic fibers, and spindle cells suggestive of fibroblasts. Elastica-van Gieson and Masson-trichrome stain identified abnormal elastic fibers and abundant collagen fibers, respectively, in elastofibroma. Immunohistochemically, fibroblasts were positive for CD34 but negative for alpha-smooth muscle actin and h-caldesmon. Additionally, the cytoplasm of many fibroblasts was positive for TGF-beta in all tumors. Ultrastructurally, some fibroblasts with abundant organelles in one tumor were observed in the adjacent area to amorphous elastic mass and bundles of collagen fibers. However, no myofibroblasts were ultrastructurally identified in the tumor. Finally, our study supplies further evidence that elastofibroma may show the proliferation of CD34-positive fibroblasts and contain no myofibroblasts, and that fibroblasts may produce both abnormal elastic fibers and collagen fibers through the secretion of TGF-beta.     

隆突性皮肤纤维肉瘤72例临床病理学观察

目的 观察隆突性皮肤纤维肉瘤(DFSP)的临床病理特点，探讨诊断、鉴别诊断及其组织起源。方法 对72例DFSP病例进行临床表现、组织形态学、免疫组化研究，16例真皮纤维瘤(DF)、19例神经纤维瘤(NF)、17例纤维肉瘤(FS)分别作为对照。结果 72例DFSP好发中青年男性，肿瘤为单发或多发性结节，位于真皮，可浸润皮下脂肪及横纹肌。组织形态除经典的车辐状或席纹状结构外，还存在一些变异如黏液变性、伴FS区域、Bednar瘤等。DFSP组87％CD34阳性，NF组42％CD34阳性。结论 掌握DFSP的临床病理特点，避免与其它皮肤梭形细胞肿瘤尤其是DF、NF及黏液性肿瘤混淆。CD34阳性提示DFSP可能与神经鞘膜有关。     

十二指肠间质瘤的临床病理及免疫组织化学研究

目的：研究十二指肠间质瘤临床病理学特点和免疫组织化学表达特征。方法：对18例十二指肠间质瘤作了临床病理形态学观察和免疫组织化学分析。结果：18例肿瘤良性3例，恶性15例，基本细胞类型为梭形细胞，1例肿瘤细胞外基质可见丝团样纤维。免疫表型特征为：C-kit18例（100％）胞质强阳性表达；CD347例（38．9％）阳性；S－100蛋白9例（50％）呈局灶性或散在阳性，其中包括2例良性，7例恶性；SMA仅1例阳性。结论：十二指肠间质瘤恶性发生率较高，细胞类型以梭形细胞为主。C-kit因其敏感性高、特异性强成为十二指肠间质瘤的可靠标记物，但不能作为良恶性判断指标，CD34阳性表达率低；神经化生率较高平滑肌方向分化率低。     

恶性颗粒细胞瘤临床病理、免疫组化和超微结构观察

目的：探讨恶性颗粒细胞瘤的病理学诊断和鉴别诊断要点及组织学起源。方法：对３例恶性颗粒细胞瘤进行临床病理、免疫组化及超微结构观察研究。结果：男性２例，女性１例，平均年龄为４９岁。部位分别为颈部１例，右大腿２例。其中２例分别于术后２年半及７年复发，并伴有区域淋巴结转移。组织学上３例与良性颗粒细胞瘤十分相似，局部区域出现梭形瘤细胞，空泡状核及明显的核仁，其中１例在肿瘤的周边部可见到瘤细胞与外周神经束支之间有直接移行关系。免疫酶标结果显示瘤细胞强阳性表达Ｓ－１００蛋白和神经特异性烯醇化酶（ＮＳＥ），１例电镜检测显示胞浆内充满膜包被复合性溶酶体。结论：对临床明显恶性而组织学上却极似良性的恶性颗粒细胞瘤，以下几点能提示恶性诊断：（１）肿瘤超过４ｃｍ；（２）核分裂象超过２个／１０ＨＰＦ；（３）核呈空泡状并有明显的核仁；（４）出现梭形瘤细胞；（５）有肿瘤性坏死。此外，免疫组化标记及超微结构观察有助于鉴别诊断及揭示组织学起源。     

23例原发性十二指肠癌临床病理与免疫组化分析

应用免疫组化方法和临床病理资料对２３例十二指肠癌加以分析，结果表明，原发性十二指肠癌患者发病高峰期为５０～６０岁，肿瘤发生在乳头部最多见（１５例，占６５％），在组织分型上，以管状腺癌（１２例）和乳头状腺癌（７例）多见。免疫组化染色显示，１１例病人伴有神经内分泌细胞分化，其中伴有胰多肽表达者９例，胃泌素８例，生长抑素４例，胰高血糖素３例，两种激素同时表达者４例，三种激素表达者４例，单一激素表达者３例     

胃肠道间质瘤76例的临床病理及免疫组织化学特征

目的：探讨胃肠道间质瘤（GIST）的临床表现、病理组织形态学和免疫组织化学特点及良恶性参考指标。方法：用CD117、CD34等抗体,通过免疫组织化学EnVision法对原发性消化道间叶源性肿瘤进行研究,确诊76例GIST,并结合随访资料,对其生物学行为进行分析。结果：本组GIST均为成年人,年龄32－81岁（平均54岁）,男性39例,女性37例,发生于胃34例,小肠23例,结肠2例,直肠17例,最常见症状为腹部肿块、腹部胀痛不适及消化道出血。黏膜下生长者3例,浆膜外生长者25例,余48例主要位于肌壁。大体上,肿瘤较易出现出血、坏死、囊性变等继发性改变。镜下观察,梭形细胞型46例,上皮样细胞型9例,梭形／上皮样细胞混合型21例,呈交叉束状、弥漫片状、栅栏状、漩涡状、小巢状、器官样及假菊形团样排列,瘤细胞胞质空亮、淡伊红、轻至中度嗜伊红及略嗜碱,核梭形、卵圆、圆形或镰状。CD117和CD34多为弥漫强阳性,阳性率分别为98．7％、68．4％,α-平滑肌肌动蛋白（ α-SMA）、肌特异性肌动蛋白、S-100、蛋白基因产物9．5呈片状或局灶阳性,阳性率分别为25．0％、19．7％、23．7％、17．1％,波形蛋白均阳性,结蛋白、胶质纤维酸性蛋白、神经丝蛋白均阴性。76例GIST中,良性9例,交界性19例,恶性48例。良性及交界性获访20例均健在,恶性组获访34例,10例无瘤生存,10例复发及转移,14例死亡。各组之间术中黏连、瘤体直径大于5cm、核分裂象大于5／50HPF差异有显著性意义,肿瘤性坏死、核分裂象大于10／50HPF、细胞密集明显异型者均在恶性组。恶性组内,肿瘤性坏死或核分裂象大于5／50HPF者3年生存率的差异有显著性意义。结论：GISF好发于中老年人,肿瘤细胞形态多变,排列结构多样;免疫组织化学特征为CD117、CD34阳性,结蛋白阴性;除转移和浸润外,肿瘤性坏死、核分裂象大于10／50HPF、细胞密集明显异型等提示恶性,此外,术中粘连、瘤体直径大于5cm、核分裂象大于5／50HPF可作为良恶性参考指标。     

43例食管基底样鳞状细胞癌临床病理分析

目的探讨食管基底样鳞状细胞癌（basaloid squamous cell carcinoma,BSCC）临床病理特点。方法对43例食管BSCC进行组织形态学及免疫组化观察,并对其临床病理特点及随访资料进行分析。结果BSCC由类似于鳞状上皮基底细胞样的细胞组成,细胞排列呈实性巢状、小梁状、假腺样或筛状结构,巢周边瘤细胞常呈栅栏状排列,巢中央可见粉刺样坏死。24例（55.8％）与普通鳞癌或腺鳞癌并存,11例（25.6％）出现局灶鳞化,28例（65．1％）见到脉管内癌栓。免疫组化染色CKpan、AE1、AE3均呈不同程度的阳性表达,p53、Ki-67、PCNA呈中至强阳性表达,Syn、CgA分别有1例呈弱阳性,S-100蛋白、SMA、CEA均为阴性。结论BSCC是食管一种少见的鳞状细胞癌变异型,具有独特的形态特点和明显的侵袭性行为,预后较差。免疫组化无特异性,诊断主要依靠形态学特点。     

脾脏淋巴管瘤的临床病理观察

目的探讨脾脏淋巴管瘤的临床病理特征和鉴别诊断特点。方法结合文献回顾,对18例脾脏淋巴管瘤进行临床资料分析、病理形态学观察及免疫表型检测。结果18例淋巴管瘤患者年龄9～72岁,中位年龄40岁;男13例,女5例;可无明显临床症状,也可表现以腹痛为主的消化道症状或脾功能亢进的相关症状。18例患者随访率72．2％,随访时间从5个月到15年不等,患者均无瘤生存,无复发及转移。脾脏多有肿大,病灶大体形态多样,包括囊性（8例）、实性（5例）和蜂窝状（5例）,可单发（5例）或多发（13例）;组织形态上分为海绵状淋巴管瘤（9例）、囊状淋巴管瘤（5例）及混合型（4例）。16例（100％）均表达CD9;7例（43．8％）表达D2-40。结论发生在脾脏的淋巴管瘤非常少见,应注意与脾脏血管瘤等鉴别。     

原发性硬膜淋巴瘤2例临床病理分析

目的探讨原发性硬膜淋巴瘤的临床病理特点。方法对2例原发性硬膜淋巴瘤大体与镜下形态进行分析并复习文献。结果1例为原发性硬脊膜外中心/中心母细胞淋巴瘤(CD20 ),瘤细胞体积相当于1~3个小淋巴细胞,核卵圆或成角或核膜呈锯齿状,染色质粗密,核较大者可见核仁。1例为原发性硬脊膜下腔大B细胞淋巴瘤,瘤细胞体积大,胞质中等,核圆形,可见核仁。结论现有资料提示硬脊膜外淋巴瘤以高中度恶性居多,硬脑膜外淋巴瘤以低度恶性居多。硬膜淋巴瘤可能源于颅及椎管外淋巴组织迁入硬膜外甚至硬膜下腔隙的淋巴细胞。     

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。     

混合性节细胞瘤/垂体腺瘤4例临床病理观察

目的探讨混合性节细胞瘤／垂体腺瘤发生机制、临床病理特征、诊断及鉴别诊断。方法复习4例混合性节细胞瘤／垂体腺瘤患者的临床资料，并对其进行组织学观察和免疫组化（EnVision—plus法）标记。结果4例混合性节细胞瘤／垂体腺瘤患者均为女性，年龄10～35岁，平均26．5岁。临床上3例表现内分泌症状，1例有癫痫症状。组织学上肿瘤由两种不同成分组成，一种结构主要由较多神经节细胞组成，节细胞体积大，可见圆形的尼氏小体，核大，核仁突出，免疫组化标记瘤细胞表达CgA、Syn、S-100蛋白，不表达GFAP；另一部分腺瘤细胞大小形态较一致，圆形或卵圆形，胞质丰富，嗜酸性或空淡，间质血窦丰富，瘤细胞表达GH和PRL。结论混合性节细胞瘤／垂体腺瘤是蝶鞍区极少见的肿瘤，好发于青年女性，常合并肢端肥大症。该瘤的诊断主要依靠组织病理学，并辅以免疫组化标记。治疗主要采用单纯手术切除，预后较好。     

Lymphohistiocytoid mesothelioma: a clinical, immunohistochemical and ultrastructural study of four cases and literature review

Lymphohistiocytoid mesothelioma (LHM) is a term proposed to designate a variant of mesothelioma that assumes a histiocytoid morphology and contains intense lymphocytic infiltrates. Reports on this variant are few, and its clinicopathologic and ultrastructural characteristics are still to be defined. The authors have studied 4 such cases that were identified among 120 mesotheliomas in the electron microscopy (EM) files of a single institution between 1982 and 2002. Histologically, all 4 lesions were composed of diffuse proliferations of cells with a histiocytoid appearance, admixed with an intense lymphocytic infiltrate. All 4 were associated with an unequivocal sarcomatoid component. Immunohistochemical (IHC) studies showed that the majority of histiocytoid cells were positive for CD68 and vimentin in all 4 cases, suggesting fibrohistiocytic differentiation. Immunoreactivity for calretinin and AE1:AE3 was only very focally identified in 3 of 4 cases. All cases were negative for CEA (M), Leu-M1, and B72.3. The lymphocytic component in all 4 cases was almost entirely composed of CD3- and CD8-positive, small, mature T cells, with only a minor component of CD20-positive cells and occasional eosinophils. Although all cases contained numerous CD68 positive atypical cells, co-expression of CD68 and either calretinin or keratin by individual cells was difficult to demonstrate by immunohistochemistry. Ultrastructurally, 3 of the 4 cases demonstrated very focal mesothelial differentiation as evidenced by long and slender surface microvilli, including the case with negative immunoreactivity for calretinin and cytokeratin. Review of the literature yielded 6 additional LHM cases. Analysis of all 10 cases showed a male predominance (8:2) with a mean age of 58 years (31-73 years). All 10 cases involved the pleura. Three of 10 patients had known asbestos exposure. Six of 10 patients died of disease at 2-20 months after the diagnosis (mean, 6.9 months). The findings suggest that LHM is a distinct morphological variant of sarcomatoid mesothelioma for which mesothelial differentiation is difficult to document. Many of the cells composing these tumors exhibit fibrohistiocytic differentiation. The unusual morphological pattern of LHM makes a combined modality approach, including IHC, EM, and a knowledge of the clinical/radiologic findings, essential in achieving a correct diagnosis.     

腹腔内纤维瘤病临床病理、免疫组化及超微结构研究

目的；探讨腹内纤维瘤病临床病理、免疫组化和超微结构特点,讨论其鉴别诊断。方法；用免疫且化及电镜的方法，结合HE形态学观察，对17例腹内纤维瘤病进行研究。结果：男7例，女10例，年龄19－78岁，平均42．9岁，中位年龄42岁。4例有手术史，2例有Gardner综合征。15例单结节，2例2枚结节。肿块直径5－25cm，平均13．5cm，多数境界清楚、切面灰白质韧、实性、半透明状。光镜下主要有纤细梭长和星芒状两种形态的(肌纤维母细胞，间质大量胶原。超微结构显示增生细胞周围大量胶原原纤维，质膜清楚，无基板，胞质内有丰富的粗面内质网。免疫表型：Vim弥漫强阳性；46．7％（7／15）；α－SMA局灶阳性；40％（6／15）MSA局灶阳性；CD117、CD34、S－100蛋白、PGP9．5、desmin阴性。行完整肿块及部分肠段切除者15 例。7例无复发（2－13年，平均8年），3例失访，5例随访中。姑息术者2例，1例死于肠梗阻，1例再手术后失访。结论：腹内纤维瘤病是少见且易误诊的疾病，部分与手术史以有Gardner综合征有关，可能的机制为胶原合成和降解失衡，免疫组化显示部分肌纤维母细胞分化的特点，手术完整切除者治疗效果好。     

Salivary duct carcinoma: A clinicopathologic study of three cases with a review of the literature

Three cases of salivary duct carcinoma are presented. They occurred in a 60 year old man, a 66 year old man and a 57 year old woman. All of the lesions were located In the parotid gland. The tumor size ranged from 3 to 5 cm across the largest diameter. Facial paralysis was observed in two cases. Histologically, intreductal and invasive adenocarcinoma showing papillary, cribriform, and solid patterns with comede like necrosis was observed. lmmunohistochemically, the tumor cells were positive for keratin and epithelial membrane antigen. No myoepithelial cells were demonstrated within the tumor by staining for S-100 protein, α-smooth muscle actin or muscle specific actin. Ultrastncturally, intracytoplasmic lumina with microvilli, a moderate number of mltochrondria, lysosomes, and tight junctions were found. Regional lymph node metastasis was observed in one case, and distant metastasis developed in two cases. All of me patients were treated with adjuvant postoperative irradiation. One patient died of disease at 11 months after the initial diagnosis, another was alive with disease at 8 months, and the third patient was alive without disease at 2 years and 3 months. Salivary duct carcinoma should be ditterentiated from low-grade salivary gland carcinomas using morphologic and clinical criteria because of its poor prognosis even with aggressive therapy.     

Renal myopericytoma: a clinicopathologic study of six cases and review of the literature

To evaluate the morphologic features, immunohistochemical profiles, and biological behavior of renal myopericytoma. Six cases of renal myopericytoma are retrospectively retrieved and analyzed by H&E and immunohistochemical staining. Clinically, patient’s age ranged from 33 to 70 years (median, 56 years). Male to female ratio was 5:1. Five of the six patients were asymptomatic of the urinary tract, the remained one presented with abdomen pain. Grossly, all six tumors were solitary masses with sizes ranging from 1.8 to 7.3 cm of maximum diameter (mean, 4.4 cm). Five tumors were described as well-circumscribed, and 1 case was showed as ill-defined. Histologically, in all cases, numerous thin-walled vessels and a perivascular arrangement of ovoid, spindled or round myoid tumor cells were seen. However, a broad morphologic spectrum ranging from fibroma-like (3 cases), glomangiopericytoma-like (3 cases), angioleiomyoma-like (2 cases), glomoid- like (2 cases), and myofibroma-like (2 cases) components were observed. In addition, 1 neoplasm with immature cellular features and another infiltrating myopericytoma were found. A coexisting papillary adenoma was detected in 1 case. Nuclear atypia was seen in 2 cases. Immunohistochemically, SMA, caldesmon, and MSA were positive in all 6 cases, whereas CD34 and desmin was partial positive in 1 case, respectively. Ki67 index was aproximately 5% in 1 case but less than 2% in the others. All patients are free of disease by follow-up ranging from 14 to 66 months (mean, 38.7 months).     

乳头状肾细胞癌32例临床病理分析

目的：探讨乳头状肾细胞癌( papillary renal cell carcinoma, PRCC)的临床病理特征、免疫表型、鉴别诊断和预后。方法回顾性分析32例PRCC患者的临床和病理资料,采用免疫组化EnVision法染色,并对患者进行随访。其中21例行根治性肾切除术,11例行肾部分切除术。结果770例肾上皮性肿瘤中32例为PRCC(4.2%)。镜下见PRCC主要由多少不等的乳头状和管状结构组成,被覆单层立方或多层柱状肿瘤细胞,乳头轴心及间质内可见泡沫细胞、砂砾体沉积,部分肿瘤细胞胞质内可见含铁血黄素。Ⅰ型18例,细胞呈立方形,胞质少,嗜碱性,淡染,Fuhrman分级低级别16例;Ⅱ型14例,细胞呈高柱状,胞质丰富,嗜酸性,Fuhrman分级高级别12例。Ⅰ型和Ⅱ型PRCC不同程度地表达vimentin、EMA、CK(AE1/AE3)、CK7、CD10和AMACR,均不表达CK(34βE12)和TFE-3。31例患者获得随访,1例术后肝、肺转移,4个月后死亡,3例术后1年分别出现骨、肺、肝等处转移,2年后死亡;死亡患者中Ⅱ型3例、Ⅰ型1例。其余27例均无瘤生存。高核分级、血管内癌栓、淋巴结转移、高临床分期提示患者预后较差。结论 PRCC国内少见,具有独特的病理形态特征,Ⅱ型PRCC较Ⅰ型患者预后差。 PRCC细胞核分级高、出现肉瘤样成分或有透明细胞癌结构可能提示肿瘤具有侵袭性,预后不良。诊断时需结合病理组织学特征、免疫表型和细胞遗传学分析。     

Solitary fibrous tumor of the central nervous system: report of 2 cases and review of literature

Solitary fibrous tumors (SFTs) rarely occur in the central nervous system (CNS). Involvement of the brainstem and pineal gland is rarely recorded. Herein, we represent 2 cases of SFTs and firstly report SFT of the pineal gland. Cranial MR imaging showed isointense to hypointense signal intensity, and marked enhancement. Microscopically, the tumors showed characteristic “patternless-pattern” architecture. Elongated tumour cells formed fascicles alternating with hypocellular densely collagenous stroma. Immunohistochemistry for CD34, BCL2, and CD99 favors the definitive diagnosis of SFT. It is difficult to predict prognosis in patients with intraventricular SFT. In general, complete surgical resection may offer the best chance of a favorable clinical outcome.     

Rasmussen encephalitis: a clinicopathologic and immunohistochemical study of seven patients

We retrospectively reviewed the clinicopathologic features and immunohistochemical profiles of 7 patients with Rasmussen encephalitis (age range, 3.5-15 years at surgery). All had medically intractable seizures (6 months' to 7 years' duration); all but 1 developed unilateral hemiparesis. Histologically, all cases were characterized by leptomeningeal and parenchymal perivascular chronic inflammation consisting primarily of T lymphocytes (CD3+, CD5+, CD7+). In all but 1 case, a predominance of CD8+ T-cytotoxic/suppressor lymphoid cells over CD4+ cells was observed. All cases had rare B lymphocytes (CD79a+, CD20+). Rare CD10+ and no CD56+ cells were noted. All cases were marked by diffuse proliferation of microglial cells, highlighted on CD68 immunostaining. Focal microglial nodule formations were observed in 4 cases and focal cortical atrophy in 5 cases. Viral inclusions were not noted. There was no evidence of Epstein-Barr virus by LMP-1 antibody immunostaining. The histologic findings of Rasmussen encephalitis resemble those of viral meningoencephalitis. The pathologicfindings may be only focally present, and missed, if diagnosis is made or confirmed with biopsy alone. Most lymphoid cells have a T-cell immunophenotype, with a predominance of CD8+ cells in most cases.