假腺性神经鞘瘤

目的：探讨假腺性神经鞘瘤的临床和病理学特征,诊断与鉴别诊断及其发生原因。方法：对2例假腺性神经鞘瘤的组织形态和免疫组化表现进行观察与分析。结果：肿瘤组织具有通常型神经鞘瘤结构外,尚有类似上皮细胞衬覆的腺样或囊样腔隙特征,但衬覆腔隙的细胞下面无基膜存在,而与邻近的梭形瘤细胞移行;黏液和免疫组化CK、EMA、CEA染色均阴性,而S-100蛋白和MBP阳性,结论：衬覆腺样或囊样腔隙的细胞仍系神经鞘细胞,肿瘤内的腺样或囊样腔隙及其内衬覆有似上皮细胞的改变可能与肿瘤的变性有关。     

上皮样型恶性周围神经鞘瘤

我们拟探讨上皮样型恶性周围神经鞘瘤形态学特征与预后的关系。１２例经光镜和免疫组化观察，其中７例作了电镜观察。组织学可分为纯上皮样型（５例）；混合型、伴有梭形细胞区（５例）和骨化型（２例）。１２例均呈Ｓ－１００蛋白阳性表达，并显示多种细胞骨架异质性，包括（１）神经细胞分化：表现为ＮＳＥ和神经微丝阳性反应，节细胞分化；（２）上皮性分化：角蛋白阳性反应，电镜见有桥粒和张力微丝束；（３）间叶性分化：波形蛋白阳性、骨化生和横纹肌样表型。该瘤的异质性与侵袭性同步，异质性明显的３例均因肺转移死于就诊后７个月至４年。     

胃神经鞘瘤临床病理分析

目的：探讨胃神经鞘瘤（gastric schwannoma）的临床病理学特点、免疫表型、诊断及鉴别诊断。方法：对8例胃神经鞘瘤的临床特点、组织形态学和免疫组织化学结果进行分析,并复习相关文献。结果：患者女性5例,男性3例,平均年龄61岁（30~81岁）。8例胃神经鞘瘤直径1.0~7.0 cm（平均3.0 cm）,无包膜,切面灰白色,镜下,瘤细胞短梭形,束状、栅栏状排列,肿瘤的边缘见淋巴细胞套。免疫组织化学染色：S-100全阳性,胶质纤维酸性蛋白（glial fibrillary acidic protein,GFAP）7例阳性,CD117、CD34、Dog1、a-平滑肌肌动蛋白（a-Smooth muscle actin,a-SMA）、结蛋白（Desmin）阴性。3例发现血小板衍化生长因子受体（platelet-derived growth factor receptor,PDGFR）阳性,但未发现c-kit和PDGFRα的基因突变。结论：胃神经鞘瘤是一种良性肿瘤,需与胃肠道间质瘤鉴别。     

神经鞘瘤假腺性结构的本质

目的：认识神经鞘瘤假腺性结构的本质及其发生原因,决定是否采纳假腺性神经鞘瘤之命名。方法：运用免疫组化ABC法观察126例神经鞘瘤的病理形态表现。结果：颅内和椎管内神经鞘瘤除了具有躯体神经普通型神经鞘瘤的A、B区结构表现外,尚易发生出血、囊性变、核多形性,泡沫细胞和假腺样结构等变化。免疫组化证明这些假腺样或腔隙状结构由神经鞘瘤的瘤细胞组成。对S-100蛋白、MBP呈阳性表达,对EMA和CEA呈阴性表达。结论：神经鞘瘤的假腺样和腔隙状结构和颅内椎管内神经鞘瘤的出血,囊性变,核异形,泡沫细胞等改变一样,可能是在肿瘤的退行性改变中对囊液的反应性改变,以不采用“假腺性神经鞘瘤”为妥。     

神经鞘黏液瘤1例

患者男,19岁,右拇指肿块5～6年,无痛及不适感,于2007年2月行肿瘤切除术。     

会阴部丛状神经鞘瘤临床病理分析

目的 探讨会阴部丛状神经鞘瘤(plexiform neurilem-moma,PN)临床病理特点和鉴别诊断.方法 对1例发生在会阴部的PN进行组织形态学观察、免疫组化标记并复习文献.结果 患者女,31岁,会阴部肿块渐增大4年,无疼痛.边界清楚,切面灰白,实性,呈多结节状,质地中等.镜检:肿物位于皮下,约有30个大小不等的结节组成,直径0.5～2.0cm不等,每个结节均见纤维包膜包绕,大多数结节以细胞致密区(Antoni A)为主,无或少有细胞疏松区(Antoni B).结节内瘤细胞呈长梭形,核呈锥形、仿锤形或波浪状,呈栅栏状和螺旋状排列,可见verocay小体.免疫组化标记显示:瘤细胞vimentin和S-100蛋白弥漫阳性,GFAP与GPG 9.5部分呈阳性.结节外包膜EMA、CD34阳性,瘤细胞阴性.NSE、CD57、ER、PR、CD68、α-SMA、desmin和H-Caldesmon均阴性.结论 丛状神经鞘瘤是一种具有特殊形态学特点的外周神经肿瘤,应与丛状神经纤维瘤、丛状纤维组织细胞和婴儿和儿童丛状恶性外周神经鞘膜瘤等相鉴别.     

恶性黑色素瘤的免疫组织化学鉴别诊断

以Ｓ－１００、Ｖｉｍｅｎｔｉｎ、ＨＭＢ４５、Ｋｅｒａｔｉｎ、ＥＭＡ、ＬＣＡ抗体，ＳＰ法对原病理诊断或疑诊的３５例恶性黑色素瘤（ＭＭ）进行染色。结果１０例典型的少色素性ＭＭ均呈Ｓ－１００、Ｖｉｍｅｎｔｉｎ和ＨＭＢ４５阳性，符合原ＭＭ诊断。原病理诊断１９例和疑诊６例共２５例无色素性恶性黑色素瘤（ＡＭＭ）中，２１例同时呈Ｓ－１００、ｖｉｍｅｎｔｉｎ和ＨＭＢ４５阳性，故确诊为ＡＭＭ；４例Ｓ－１００和ＨＭＢ４５阴性肿瘤中，３例为Ｋｅｒａｔｉｎ阳性、ＥＭＡ弱阳性，１例为ＬＣＡ和Ｖｉｍｅｎｔｉｎ阳性，证实不是ＭＭ而是癌和淋巴瘤。结果表明，Ｓ－１００和ＨＭＢ４５是ＭＭ诊断性标志物，后者具有特异性。     

结肠色素性恶性上皮样神经鞘瘤1例

患者女 ,37岁。因左腹部不适 6个月 ,加重伴腹部包块及便血 1个月入院 ,既往史无特殊。查体 :左侧腹部扪及 4ｃｍ× 5ｃｍ× 7ｃｍ包块 ,质中 ,表面光滑。钡灌肠提示左半结肠占位性病变。临床诊断 :结肠癌 ,不完全性肠梗阻。行剖腹探查术 ,术中见少量清亮腹水 ,左半结肠 -结肠套叠 ,降结肠肠腔内有一肿块 ,约 5ｃｍ× 6ｃｍ× 9ｃｍ ,质中。右半结肠及回肠扩张 ,肠系膜淋巴结肿大。探查肝、胆、子宫及双侧附件未发现异常。切除左半结肠及肠系膜淋巴结。病理检查　眼观 :肠管一段 ,长 2 3ｃｍ ,直径 4ｃｍ ,距一切端 1 3 5ｃｍ处肠管增粗 ,直…     

富于细胞性神经鞘瘤的临床病理学观察

1．材料：收集复旦大学附属肿瘤医院病理科1990—2004年4月间的常规病例4例和会诊病例6例。患者的临床表现、肿瘤的大体形态和随访结果来自于病理申请单或病历记录。另选取10例胃肠道型神经鞘瘤作对照性研究。     

心脏恶性神经鞘瘤一例

患者女，１９岁。无明显诱因于１９９８年５月２７日出现双足水肿，１周后口唇紫绀，在当地医院以“急性肾炎”治疗后水肿消失，但口唇紫绀加重，伴活动后心悸、气促于１９９８年７月１８日入我院。体检：口唇明显紫绀，心浊音界向左扩大，心律齐，肝肿大。心电图示：窦性心律，全导联低电压。超声心动图示：右心房带蒂肿物，右室流出道阻塞。手术所见：肿瘤蒂部位于三尖瓣，呈浸润性生长，突入右心房和右心室。病理检查：巨检：手术切除肿瘤组织３块，体积分别为７．５ｃｍ×４．５ｃｍ×４．０、６．５ｃｍ×４．３ｃｍ×４．０、６．０…     

Peripheral Nerve Sheath Tumors of the Thyroid Gland: A Series of Four Cases and a Review of the Literature

Primary peripheral nerve sheath tumors (PNSTs) of the thyroid gland are exceptionally rare. Two schwannomas and two malignant PNSTs (MPNSTs), arising primarily within the thyroid gland, were identified in the files of the Endocrine Tumor Registry at the Armed Forces Institute of Pathology. The patients included two females, age 69 and 80 yr, and two males, age 18 and 33 yr. The patients presented with a mass in the thyroid gland confined to a single lobe of the thyroid without involvement of the cervical neck region. None of the patients had a history of neurofibromatosis. The benign tumors were encapsulated, one of them cystic, with the characteristic cellular and nuclear features of schwannomas. The MPNSTs were invasive tumors, effacing the thyroid parenchyma, with a fascicular pattern of growth composed of neural appearing cells with increased cellularity, increased mitotic activity and with focal necrosis. Immunoreactivity for S-100 protein and vimentin was seen in all tumors. The patients with schwannomas, treated only by surgical resection, were alive without evidence of disease, over a period of 5–33 yr. Both patients with MPNSTs died of the disease 8 mo and 42 mo, respectively, with widely disseminated disease. Primary thyroid PNSTs are exceptionally rare tumors. MPNSTs, in this limited experience, have a fatal outcome irrespective of aggressive adjuvant therapy. This work was presented at the 85th Annual Meeting of the US and Canadian Academy of Pathology, Washington, DC, March 1996.     

Glial FibriUary Acidic Protein (GFAP) Immunoreactivity in Peripheral Nerve Sheath Tumors

A spectrum of 24 benign and malignant nerve sheath tumors and 10 non-neural spindle-cell tumors were studied by iight microscopy for the presence of glial fibrillary acidic protein (GFAP) immunoreactivity by the peroxidase-antiperoxidase (PAP) technique. In 8 cases, these results were compared to their electron mocroscopic appearances. Seventy percent (7 of 10) of begnine schwannomas and 50% (4 of 8) of benign neurofibromas demonstrated focal to diffuse GFAP immunoreactivity. None of the malignant nerve sheath tumors nor any of the non-neural spindle-cell neoplasms contained demonstrable GFAP immunoreactivity. Similarly, no GFAP immunoreactivity could be detected in Schwann cells in normal peripheral nerves. The solitary benign schwannoma available for electron microscopic study demonstrated diffuse and abundant cytoplasmic intermediate filaments, and this tumor displayed diffuse and intense GFAP immunoreactivity. Two benign neurofibromas showed a more variable content of intermediate filaments ultrastruc-turally, and their GFAP immunoreactivity was variable. All five malignant nerve sheath tumors studied by electron microscopy displayed a variable complement of intermediate filaments; however, none of these tumors possessed GFAP immunoreactivity, suggesting that these intermediate filaments are either members of a different class of intermediate filaments or may perhaps represent “altered” GFAP not recognized by these antisera.     

Glial FibriUary Acidic Protein (GFAP) Immunoreactivity in Peripheral Nerve Sheath Tumors

A spectrum of 24 benign and malignant nerve sheath tumors and 10 non-neural spindle-cell tumors were studied by iight microscopy for the presence of glial fibrillary acidic protein (GFAP) immunoreactivity by the peroxidase-antiperoxidase (PAP) technique. In 8 cases, these results were compared to their electron mocroscopic appearances. Seventy percent (7 of 10) of begnine schwannomas and 50% (4 of 8) of benign neurofibromas demonstrated focal to diffuse GFAP immunoreactivity. None of the malignant nerve sheath tumors nor any of the non-neural spindle-cell neoplasms contained demonstrable GFAP immunoreactivity. Similarly, no GFAP immunoreactivity could be detected in Schwann cells in normal peripheral nerves. The solitary benign schwannoma available for electron microscopic study demonstrated diffuse and abundant cytoplasmic intermediate filaments, and this tumor displayed diffuse and intense GFAP immunoreactivity. Two benign neurofibromas showed a more variable content of intermediate filaments ultrastruc-turally, and their GFAP immunoreactivity was variable. All five malignant nerve sheath tumors studied by electron microscopy displayed a variable complement of intermediate filaments; however, none of these tumors possessed GFAP immunoreactivity, suggesting that these intermediate filaments are either members of a different class of intermediate filaments or may perhaps represent “altered” GFAP not recognized by these antisera.     

恶性外周神经鞘瘤52例临床病理分析

目的观察恶性外周神经鞘瘤的临床病理学特征、诊断及鉴别诊断。方法回顾性分析52例恶性外周神经鞘瘤的临床病理学及免疫表型特征并复习相关文献。结果 52例患者中,男女发病率为1∶1,年龄4~71岁,头颈部18例(35%),四肢12例(23%),躯干9例(17%),深部组织8例(15%),椎管内4例(8%),生殖道1例(2%)。镜下肿瘤组织呈束状或漩涡状排列,瘤细胞短纺锤形、卵圆形、梭形,核分裂象易见。免疫表型:瘤细胞局灶表达S-100蛋白,Ki-67增殖指数10%~70%。结论恶性外周神经鞘瘤罕见,侵袭性高,预后差,其组织形态复杂多样,需与滑膜肉瘤、纤维肉瘤、血管外皮瘤、富于细胞性神经鞘瘤、纤维型脑膜瘤以及平滑肌肉瘤等鉴别。     

Fine‐needle aspiration cytology of epithelioid malignant peripheral nerve sheath tumor: A case report and review of the literature

The epithelioid variant of malignant peripheral nerve sheath tumor (eMPNST) is an extremely rare soft tissue neoplasm comprising less than 5% of all MPNSTs. It is distinguished cytomorphologically from a conventional MPNST by the presence of polymorphous round epithelioid cells arranged in loose clusters with or without spindled tumor cells. These features pose a diagnostic challenge because the differential diagnosis involves a variety of mesenchymal and non‐mesenchymal tumors including epithelioid sarcoma, sclerosing epithelioid fibrosarcoma, malignant rhabdoid tumor, chordoma, metastatic carcinomas, and melanoma. Thus, it may become imperative to perform immunochemical stains on cell blocks of FNA aspirates to arrive at definitive diagnosis. Reports describing the cytologic features of eMPNST are rare. Herein, we report a case of eMPNST with focus on cytomorphologic and cytoimmunochemical features and differential diagnosis. Diagn. Cytopathol. 2016;44:226–231. © 2015 Wiley Periodicals, Inc.     

Perineurial Cell Differentiation in Benign Tumors and Tumorlike Proliferation of Peripheral Nerves

An unusual, benign, spindle cell peripheral nerve sheath tumor (PNST) is reported that on ultra-structural examination was composed only of perineurial cells. The neoplastic cells showed positive immunoreactivity for S-100 protein and negative immunoreactivity for epithelial membrane antigen; this is not the expected immunophenotype of a perineurioma. Continued examination of benign PNSTs using electron microscopy and immunohistochemistry has the potential to reveal additional variations in the cellular composition and immunophenotype of these soft tissue tumors. PNSTs such as the one described will renew the debate about the cytogenesis of the perineurial cell and its role in PNSTs.     

Perineurial Cell Differentiation in Benign Tumors and Tumorlike Proliferation of Peripheral Nerves

An unusual, benign, spindle cell peripheral nerve sheath tumor (PNST) is reported that on ultra-structural examination was composed only of perineurial cells. The neoplastic cells showed positive immunoreactivity for S-100 protein and negative immunoreactivity for epithelial membrane antigen; this is not the expected immunophenotype of a perineurioma. Continued examination of benign PNSTs using electron microscopy and immunohistochemistry has the potential to reveal additional variations in the cellular composition and immunophenotype of these soft tissue tumors. PNSTs such as the one described will renew the debate about the cytogenesis of the perineurial cell and its role in PNSTs.