膀胱具有横纹肌样形态的癌1例报道及文献复习

目的 探讨膀胱具有横纹肌样形态的癌的临床病理特征、诊断、鉴别诊断,以提高对本瘤的认识.方法 应用光镜、特殊染色和免疫组化染色对1例罕见的膀胱具有横纹肌样形态的癌进行观察,并复习相关文献.结果 瘤细胞松散,黏附性差,单个散在或弥漫片状排列,侵犯膀胱肌层.瘤细胞体积较大,圆形或多边形,胞质丰富,嗜酸性,核圆或卵圆形,居中或偏位,核内染色质细,核仁多不明显,核分裂象可见,散在单核或多核瘤巨细胞.免疫组化:瘤细胞呈CK-pan、EMA、CK18及CEA弥漫强阳性,CK20及34BE12散在阳性;而vimentin、SMA、desmin、myoD1、myoglobin、CK7、p63、CD38、S-100、HMB45、melan A、CgA、Syn、CD45、CD30、ALK和CD34均阴性.结论 膀胱具有横纹肌样形态的癌极罕见,可能为尿路上皮癌的组织学变异.诊断时须与其他具有横纹肌样细胞形态的肿瘤鉴别.     

横纹肌样表型大细胞肺癌1例

患者女性, 71岁,以咯血 1月余收入院。CT示:左肺下叶背段高密度影。术前诊断:左肺下叶癌。行左肺下叶切除,术中见左下肺背段有一质硬肿块, 5cm×3cm×3cm,分叶状。病理检查　眼观:送检左下肺叶切除标本, 11cm×9cm4cm,残端支气管处肺组织中可见一中央型肿物,分叶状,面积 5cm×3cm,切面灰黄灰黑相间,中央坏死明显,沿残端支气管剖开,管腔通畅,管壁光滑。肺门处见淋巴结 1枚,直1 5cm,包膜完整,表面光滑,切面碳黑色,质中。镜检:瘤细胞体积大,异型性明显,呈蝌蚪状、带状或梭形,界限清,胞质丰富,嗜伊红色,有的瘤细胞质内含有棕黑色颗粒状物或…     

子宫Müllerian腺肉瘤伴间质横纹肌样瘤分化1例并文献复习

目的探讨子宫Muellerian腺肉瘤伴间质横纹肌样瘤分化的临床病理特征。方法用光镜、组织化学及免疫组化方法观察其病理组织学表现。结果肿瘤由良性上皮成分和肉瘤性间质成分组成，肉瘤成分过度生长。肿瘤细胞弥漫浸润性分布，细胞大，胞质丰富嗜酸性并可见嗜伊红包涵体。免疫表型：vimentin、CK、NF、CD57、CD99、CgA、Syn阳性，SMA散在阳性，而desmin、EMA、CD10、GFAP、MyoD1、Inhibin—α、HMB45和S-100蛋白阴性。组织化学染色PAS阴性，网状纤维染色显示网状纤维包绕单个或小巢肉瘤性间质细胞。结论子宫腺肉瘤伴间质横纹肌样瘤分化是一种罕见的混合性Muellerian肿瘤，应与子宫内膜间质肉瘤、子宫横纹肌肉瘤和低分化癌等鉴别。     

横纹肌样脑膜瘤1例并文献复习

目的 探讨横纹肌样脑膜瘤(rhabdoid meningiom,RM)的病理组织学特征、诊断及鉴别诊断.方法 应用光镜观察和免疫组化标记,对1例RM进行观察,同时复习相关文献进行讨论.结果 本例RM主要由横纹肌样形态区域构成,并可见典型的纤维性脑膜瘤形态区.肿瘤细胞体积中等偏大,可见胞质内包涵体样结构;细胞核大,偏位,可见明显核仁,核内假包涵体多见,核分裂象多见,并见出血坏死区.免疫组化染色肿瘤细胞vimentin((+++)),S-100((+++)),EMA局灶(+),p53弱(+),PCNA弱(+),GFAP、HMB-45和desmin均(-).结论 RM是发生于颅内的较少见的脑膜瘤亚型,需要与恶性黑色素瘤、肥胖细胞型星形细胞瘤、室管膜瘤、浆细胞瘤、非典型畸胎样/横纹肌样瘤等鉴别.     

伴横纹肌样特征及微乳头结构的肺腺癌临床病理学观察

目的 探讨伴横纹肌样特征及微乳头结构的肺腺癌的形态学、免疫表型及鉴别诊断.方法 对1例伴横纹肌样特征及微乳头结构的肺腺癌进行免疫组化检测并复习相关文献.结果 肿瘤由分化好的腺样区域及横纹肌样区域组成,两者相互移行或有纤细的纤维组织分隔,部分区域有微乳头结构.横纹肌样成分特征:瘤细胞黏附性差,具有丰富的嗜酸性胞质,核偏位,核仁明显,免疫组化标记vimentin呈强阳性.结论 伴横纹肌样特征及微乳头结构的肺腺癌是一种具有较高侵袭性的肿瘤,由于其预后较差,对常规治疗不敏感,认识这些特征具有重要的临床意义.     

颞部上皮样血管瘤1例及文献复习

上皮样血管瘤(epithelioid hemagioma，EH)是一种少见的特殊类型的良性血管瘤，笔者报道1例颞部EH，结合文献对其进行光镜、免疫组化研究，讨论其临床病理特征及诊断和鉴别诊断。     

肺肝样腺癌1例及文献复习

肝样腺癌（hepatoid adenocarcinoma,HAC）是一种发生于肝外的具有腺样及肝细胞样两种分化的特殊类型腺癌,此型腺癌发生在胃内的屡见报道,但原发于肺的HAC极为罕见。经检索英文和国内文献,截止2002年仅见10例报道。现将我们遇到的1例报道如下,并结合文献复习进行探讨。     

纵隔恶性横纹肌样瘤伴颈部淋巴结转移1例及文献复习

横纹肌样瘤是好发于婴幼儿肾脏、形态特殊、预后较差的一种少见肿瘤。近年来,国内外关于肾外横纹肌样瘤的报道不断增多,且可发生于成年人。现报道1例纵隔原发性恶性横纹肌样瘤伴颈部淋巴结转移的光镜、免疫组化和电镜表现,并结合文献讨论其临床病理特征及诊断与鉴别诊断。1材料     

伴横纹肌样特征的肾细胞癌1例

患者男性,73岁。因右腰部偶有不适,查体发现右肾占位入院。CT检查示右侧肾脏存在实性占位性病变,恶性肿瘤可能性大。专科检查无明显阳性体征。彩超示右肾体积增大,肾上极可见一8.4 cm×7.5 cm×7.3 cm大小的低等回声占位,边界尚清,占位可见血流信号;左肾大小正常,实质回声均匀,皮髓质界限清晰。术中见右肾增大,于肾上极见一肿物,直径约8 cm,手术切除右肾送病理检查。     

软骨样脂肪瘤1例报道及文献复习

目的探讨软骨样脂肪瘤的临床、病理学特点和鉴别诊断。方法报道1例右小腿软骨样脂肪瘤的临床资料、光镜、组化、免疫组化及电镜观察结果,结合文献讨论。结果镜下见肿瘤由3种成分以不同比例混合构成:①较成熟脂肪细胞体积小的单泡状和多泡状脂肪母细胞,胞质淡染为主,少数细胞呈嗜酸性,核形多样,无异型性及核分裂象,排列成片状和巢状;②脂肪母细胞间黏液透明性软骨样基质;③多少不等的成熟脂肪细胞。PAS染色见脂肪母细胞的胞质内含许多可被淀粉酶消化的深红染颗粒,提示存在糖原。AB染色见黏液透明性软骨样基质呈阳性反应,并部分耐透明质酸酶消化,提示含有硫酸软骨素。脂肪母细胞和成熟脂肪细胞表达S-100蛋白,其中脂肪母细胞对S-100蛋白的表达与脂肪母细胞分化成熟程度相关。电镜观察可见处于不同发育阶段的脂肪母细胞,脂肪母细胞周围被絮状的软骨样基质围绕。结论软骨样脂肪瘤是一种十分罕见的良性脂肪细胞肿瘤的特殊类型,具有独特的组织学形态,应注意与黏液型脂肪肉瘤和骨外黏液样软骨肉瘤等肿瘤鉴别。     

Malignant rhabdoid tumor of the liver: case report and literature review

A case of malignant rhabdoid tumor (MRT) occurring as a primary hepatic neoplasm in a 12-month-old Japanese female infant is presented. The patient had a slight fever for 2 weeks and presented with a palpable mass in her left hypochondrial region. After admission, the hepatic artery was embolized due to intra-abdominal hemorrhage arising from the tumor. The patient received chemotherapy with cisplatin, cyclophosphamide and adriacin. Despite treatment, the patient developed dyspnea, pancytopenia and disseminated intravascular coagulation. Rupture of the tumor resulted in death within 3 weeks. A limited abdominal autopsy revealed that the liver weighed 1240 g and was occupied by multiple hemorrhagic and/or necrotic tumor nodules. Histologically, neoplastic cells had an abundant eosinophilic cytoplasm containing paranuclear inclusions, and vesicular nuclei with a centrally located prominent nucleolus. Ultrastructurally, the cytoplasmic inclusions were composed of whorled filaments measuring 10 nm. Immunohistochemically, almost all of the neoplastic cells were positive for vimentin and cytokeratins (CK) 8 and 18, some were positive for CK 7 and 19, while none were positive for CK 1, 10, 13-17 and 20. The tumor cells did not express desmin, myoglobin, and alpha-fetoprotein. We found 18 cases of MRT of the liver published in English language literature and then, adding the present case, we summarized the 19 cases. Hepatic MRT is an uncommon neoplasm. However, it should be considered in the differential diagnosis of an aggressive liver neoplasm in childhood.     

Primary rhabdoid cancer of the ileum: A case report and review of the literature

Since the first publication of a rhabdoid cancer, described as an infrequent variant of Wilms’ tumor, several cases of extrarenal rhabdoid tumor have been reported in the literature. Here, we report on a primary rhabdoid cancer of the small intestine, and give a review of the data available in the literature.     

Large cell carcinoma of the lung with a rhabdoid phenotype

A variant of large cell carcinoma showing a rhabdoid phenotype, which is rare among primary lung cancers, is presented. A 59-year-old man was admitted to hospital for an operation. Computed tomography scans showed a mass with a smooth border, invading the thoracic wall. A right upper lobe lobectomy was carried out with resection of a part of the thoracic wall. Pathological examination showed that the tumor was mostly composed of cells with prominent eosinophilic cytoplasmic globules and giant cells, which did not adhere to each other. Cytologically, the tumor cells contained nuclei with a reticular chromatin pattern and one to two prominent nucleoli, and hyaline-like and reticular inclusion bodies, which were immunohistochemically positive for vimentin, but not for alpha-smooth muscle actin, myoglobin or pan-actin. Radiological and laboratory examinations did not detect the presence of the tumor in other organs, indicating that the primary lesion was not situated elsewhere. Metastasis to the right adrenal gland was observed 1 year and 4 months after the operation; however, the patient has been free of the disease 3 years and 11 months after the second operation of an adrenalectomy. This case showed a relatively good prognosis, which is rare among rhabdoid tumors of various organs that generally have poor prognoses with rapid, fatal progression.     

Primary pleural epithelioid hemangioendothelioma with rhabdoid phenotype: report and review of the literature

Epithelioid hemangioendothelioma (EHE) is a rare malignant vascular tumor described in diverse locations including lung and liver. Relative to these sites, primary EHE of the serous cavities is uncommon. EHE in the serous cavities mimics mesothelioma and adenocarcinoma clinically, radiographically, cytologically, and histologically. EHEs have plasmacytoid epithelioid cells with cytoplasmic vacuoles. In addition to these features, we noted eccentric nuclei with abundant eosinophilic cytoplasm and nuclei displaced peripherally by globular cytoplasmic inclusions imparting a "rhabdoid" phenotype. These cells were often seen surrounding a hyaline core. Rhabdoid features are not unique to a single entity, and a comprehensive immunohistochemical panel is essential. We report the occurrence of pleural EHE with rhabdoid features presenting in a pleural effusion, and review the literature of primary serosal EHEs.     

肺原发性绒毛膜癌1例报道及文献复习

目的 探讨肺原发性绒毛膜癌临床病理特征、诊断标准、治疗及临床转归。方法 分析1例肺原发性绒毛膜癌的临床表现、组织病理学及免疫表型特征,并进行文献复习。结果 患者,男性,53岁。临床表现咳嗽、咳血痰。影像学检查示右肺下叶肿块,全身他处未见异常。病理学检查发现肿物几乎均可见出血、坏死,其中有少数肿瘤细胞呈簇团状或小片状分布。瘤组织由中等大小、胞质透明的细胞（细胞滋养层细胞）和大而异型、胞质丰富、粉染或空泡状的多核巨细胞（合体滋养层细胞）构成。免疫表型显示瘤细胞,特别是多核巨细胞表达CK（AE1／AE3）、CK7、HCG、PLAP、Ki-67及p53,但不表达CEA、TTF1、CK20、AFP等。术后联合化疗,无瘤生存12个月,仍在随访中。结论 肺原发性绒毛膜癌是一种罕见的高度侵袭性肿瘤,具有其独特的临床病理特征,手术联合化疗是最有效的治疗方案。     

Lung carcinoma with rhabdoid cells: a clinicopathological study and survival analysis of 14 cases

AIMS: We determined the clinicopathological features of primary lung carcinomas with rhabdoid cells by defining the immunophenotype of rhabdoid cells and analysing survival. METHODS AND RESULTS: Rhabdoid cells are distinctive in having an eccentric nucleus and a large intracytoplasmic inclusion on routinely stained sections. Based on the number of rhabdoid cells, 45 cases of large cell carcinoma were divided into the following three types: lung tumour with a rhabdoid phenotype (LTRP) (n=4), lung carcinoma with a small number of rhabdoid cells (LCSR) (n=10), large cell carcinoma containing no rhabdoid cells (LCNR) (n=31). LTRP is composed of at least 10% rhabdoid cells. In LCSR the percentage of rhabdoid cells is less than 10%. LTRP and LCSR are associated with locally advanced disease. Immunohistochemical stains were positive for epithelial markers in all LTRP and eight LCSR, for neuroendocrine markers in one LTRP and three LCSR. The outcome is worse for patients with LTRP than LCSR or LCNR. LCSR shows a trend close to LCNR. Stage-matched survival analysis, however, revealed no statistically significant difference among the histological subtypes. CONCLUSIONS: Rhabdoid cells are heterogeneous except for epithelial markers and vimentin positivity. Less than 5% of rhabdoid cells has a negligible effect on prognosis.     

肺动脉内膜肉瘤1例并文献复习

目的探讨血管内膜肉瘤的临床及病理特点。方法对1例肺动脉内膜肉瘤进行光镜、电镜观察和免疫组化检测并复习文献。结果肿瘤位于肺动脉血管腔内,阻塞管腔,并延伸至周围小血管。光镜下肿瘤细胞主要为梭形细胞,散在分布上皮样细胞和多核巨细胞,核分裂象约46／50HPF。电镜观察见细胞内含有丰富的粗面内质网、线粒体,无向特殊细胞分化的特征。免疫组化染色vimentin呈弥漫阳性、α—SMA灶性阳性、HMB-45散在弱阳性,CKpan、CD34、CD31和S-100蛋白等均阴性。结论内膜肉瘤是一种罕见的发生于大血管壁的恶性肿瘤,预后差,免疫表型无特异性,了解该肿瘤发病部位、临床及病理特点有助于做出正确诊断。