子宫Müllerian腺肉瘤伴肉瘤过度生长临床病理分析

目的探讨子宫苗勒管腺肉瘤伴肉瘤过度生长(M櫣llerianadenosarcomaoftheuteruswithsarcomatousovergrowth,MASO)的临床病理特征、生物学行为及预后。方法对3例MASO采用光镜、免疫组化染色观察,并复习相关文献。结果3例患者分别表现为不规则阴道出血、绝经后阴道出血及子宫增大。大体上,宫腔内见息肉状肿物,直径2~13cm,2例浸润浅肌层,1例浸润深肌层达浆膜。组织学上,3例MASO均见典型腺肉瘤结构,即由良性腺体和低度恶性肉瘤样成分混合而成,其中纯粹肉瘤成分比例均超过25%,并且肉瘤级别比相邻的腺肉瘤高;例2含有异源成分。3例肉瘤成分均显示vimentin阳性,而EMA、CD10、desmin、SMA、S-100蛋白等均阴性。3例均行全子宫 双侧附件切除术,1例进行了盆腔淋巴结清扫。2例术后分别随访3年及5年均无瘤生存,1例术后1个月死亡。结论MASO是一种少见的M櫣llerian混合上皮和间质肿瘤,具有独特的临床病理形态特征;与经典的子宫M櫣llerian腺肉瘤相比,它更具有侵袭性,预后差。     

子宫Müllerian腺肉瘤5例临床病理分析

目的 探讨子宫Müllerian腺肉瘤的临床病理学特征。方法 收集5例子宫Müllerian腺肉瘤的临床资料,行HE、免疫组化EnVision法染色,分析其临床病理学及免疫表型特征,并复习相关文献。结果 5例患者年龄41～63岁,中位年龄44岁。镜下肿瘤呈分叶状结构,富于细胞的间质呈乳头状和息肉状突入囊性扩张的腺腔内或压迫良性腺体呈成角的裂隙。轻～中度异型的间质细胞围绕腺体呈套袖样增生。免疫表型：肿瘤间质细胞ER、PR、SMA均弥漫阳性,腺体周围CD10阳性,Ki-67增殖指数3%～30%。结论 子宫Müllerian腺肉瘤常表现为阴道异常出血,多为低度恶性肿瘤,手术治疗为主,不良预后与肿瘤过度生长、脉管内癌栓、FIGO分期等有关。     

肺梭形细胞横纹肌肉瘤1例并文献复习

目的探讨成人梭形细胞横纹肌肉瘤(spindle cell rhabdomyosarcoma,SCRM)的临床病理学特征。方法回顾性分析1例肺内SCRM的临床病理学与免疫表型特征,并复习相关文献。结果患者男性,72岁,因咳嗽、胸闷2个月入院,胸部CT检查示右肺上叶见一巨大软组织肿块影。镜检:肿瘤无包膜,呈不规则束、片状排列,局部呈巢状结构,间质纤维组织增生。瘤细胞小圆形、梭形或上皮样,胞质稀少,胞核深染、染色质细颗粒状,部分可见核仁,核分裂象达25个/10 HPF,局部可见横纹肌母细胞。免疫表型:vimentin、desmin、Myogenin、CD56、CD99均阳性(阳性细胞数>50%),CK(AE1/AE3)、CK7、Syn、CgA、CD45、TTF-1、MyoD1、NSE、BCL-2、S-100蛋白均阴性,Ki-67增殖指数约60%。结论肺成人原发性SCRM临床罕见,组织学形态多样,预后较差,需与肺小细胞癌、滑膜肉瘤、淋巴瘤等鉴别。     

伴横纹肌样特征的肺癌1例报道及文献复习

目的报道1例伴横纹肌样特征的肺癌,并复习相关文献。方法石蜡包埋组织连续切片分别做HE染色、Alcianblue黏液组化染色和免疫组化染色,光镜观察。结果肿瘤突出于右上肺支气管腔,HE染色显示在差分化癌组织中可见横纹肌样特征的癌细胞,约占30%,其细胞质丰富,细胞质内可见明显的嗜酸性包涵体,细胞核偏位,可见核仁。癌组织黏液染色阴性。免疫组化染色显示,vimentin和p63呈弥漫性强阳性,CKpan、CAM5·2、TTF-1和synaptophysin呈灶性阳性,嗜酸性包涵体对CAM5·2呈阳性反应,CEA、chromograninA、SCLC、myoglobin及desmin阴性。结论伴横纹肌样特征的肺癌是原发性肺癌少见的特殊亚型,可存在于差分化非小细胞肺癌中,并可显示灶性神经内分泌分化。     

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.     

子宫苗勒腺肉瘤6例临床病理学分析并文献复习

目的观察6例子宫苗勒腺肉瘤(uterus Müllerian adenosarcoma,UMA)的临床病理特点、免疫表型,提高对其诊断、鉴别诊断和治疗水平。方法回顾性分析病理确诊的6例UMA临床特点、肿瘤组织形态学特征及肿瘤免疫表型,并随访观察。结果 6例患者4例发生于子宫内膜,2例发生于宫颈。临床主要表现为阴道异常出血、盆腔疼痛和子宫颈息肉。全部行手术治疗,4例术后辅以化疗,2例放疗。4例发生于子宫内膜者形态学均为低级别腺肉瘤,术后辅以化疗至今无复发;2例发生于宫颈者均合并有软骨肉瘤异源性成分,术后辅助放、化疗2年后因肿瘤复发死亡。镜下所有病例均可见不规则囊性扩张、裂隙样腺体和肿瘤间质以腺周套袖样及息肉样突入腺腔的形态学特征,肿瘤间质核分裂象2～12个/10 HPF。2例伴有软骨肉瘤异源性成分者,其中1例同时伴有高级别多形性肉瘤成分过度生长并浸润肌层。免疫表型:6例肿瘤上皮CK均呈强阳性表达;上皮:ER、PR和vimentin均(+),CD10(-),Ki-67增殖指数1%;肉瘤间质:全部表达vimentin,4例表达ER、PR;1例表达SMA、desmin,3例表达CD10,Ki-67增殖指数4%;2例伴有软骨肉瘤分化者局灶表达S-100蛋白。间质细胞Ki-67增殖指数20%,明显高于低级别腺肉瘤4%的表达。结论 UMA临床罕见,患者症状、体征无特异性,正确诊断依赖病理形态学观察。低级别腺肉瘤治疗应采用手术为主的综合疗法,早期且完整切除病灶者预后较好,晚期且合并肉瘤成分过度生长者及伴有异源性分化者预后差。肿瘤的复发率较高,应长期密切随访。     

上皮样血管肉瘤1例报道并文献复习

目的 探讨软组织上皮样血管肉瘤(epithelioid angiosarcoma,EAS)的临床病理特点、诊断和鉴别诊断.方法 报道1例臀部软组织EAS临床资料、光镜及免疫组化染色结果,并结合文献复习进行讨论.结果 光镜下肿瘤成分几乎全部为上皮样肿瘤细胞组成,细胞排列成小巢状、片状或条索状,可见原始血管结构形成.免疫组化染色:瘤细胞高表达CD34、CKpan及vimentin,并表达CD31、CD99;而SMA、S-100、Myoglohin及HMB-45均阴性;Ki-67增殖指数＜20%.结论 软组织血管肉瘤罕见但上皮样型更为罕见,经组织病理学详细观察及免疫组化染色协助,对于正确诊断和鉴别诊断有重要意义.     

睾丸原发性腺泡状横纹肌肉瘤1例

睾丸原发性腺泡状横纹肌肉瘤１例胡双庆关键词睾丸肿瘤，腺泡状横纹肌肉瘤患儿，男性，４岁。右侧睾丸肿大８个月，加重３个月。于１９９４年４月１４日入院。查体：右侧睾丸肿大，呈椭圆形，肤色正常，无烧灼感及触痛，质硬呈结节状，透光试验阴性。临床拟诊为右侧睾丸精...     

子宫恶性横纹肌样瘤一例

患者女,２６岁,未婚。因不规则阴道出血伴腹痛２个月余,于１９９１年１２月５日入院。曾在当地医院行子宫诊刮术２次,病理检查结果均为“子宫内膜息肉”。体检：精神欠佳,贫血貌。腹部叩诊有移动性浊音,无压痛及反跳痛。术中发现：子宫腔及颈管内充满肿瘤组织,大网...     

结肠腺泡状横纹肌肉瘤1例

患者男 ,41岁。因右下腹痛 4天而急诊手术。手术所见 :阑尾轻度充血 ,结肠肝曲有一 15ｃｍ× 10ｃｍ大小的肿物 ,质硬 ,与胃体远端黏连。病理检查　结肠一段长 2 5ｃｍ ,周径 10ｃｍ ,距一侧切缘10ｃｍ处有 2个结节 ,大小分别为 2ｃｍ× 2ｃｍ× 2ｃｍ ,6ｃｍ×6ｃｍ× 6ｃｍ。位于肠黏膜下。结肠与胃壁间有一 15ｃｍ× 3ｃｍ× 3ｃｍ的包块 ,检出肿物旁淋巴结 5枚 ,肠系膜淋巴结 2枚 (长径 0 5～ 1 0ｃｍ)。镜检 :结肠肿瘤细胞圆形、卵圆形 ,胞质丰富 ,红染 ,核偏位 ,大部分瘤细胞被不规则纤维分隔成实性巢或腺泡状 ,部分弥漫排列。瘤组织…     

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.     

Malignant rhabdoid tumor arising in a mixed epithelial,stromal tumor of kidney: report of a male case,review of the literature

Mixed epithelial and stromal tumor (MEST) of the kidney is a rare biphasic tumor composed of both stromal and epithelial components, the latter showing a variable proportion of solid to cystic areas. These tumors show a marked female predominance, commonly occur in perimenopausal age groups, and often have an ovarian-type stroma with ER and PR positivity, suggesting steroids may play a role in pathogenesis. Although typically benign, rare cases showing malignant transformation have been reported. We present a case of a 42-year-old man with a 10 cm right kidney mass located in the renal pelvis. Histologically, the majority of the tumor was composed of a diffuse, sheet-like growth of malignant cells demonstrating a rhabdoid morphology with large nuclei, prominent nucleoli, and eosinophilic eccentric cytoplasm. Brisk mitotic activity and coagulative type necrosis was also noted. Intimately associated with this malignant rhabdoid component was a much smaller portion of tumor featuring variably sized bland epithelial tubules embedded within a stroma composed of bland spindle cells and areas of hyalinization, diagnostic of MEST. By immunohistochemistry, the malignant rhabdoid tumor portion of the neoplasm showed complete loss of nuclear INI-1, while the MEST component retained nuclear expression of this antigen. With these features taken together, our case represents a malignant rhabdoid tumor arising in a background of MEST. To our knowledge, this case represents the first case of a MEST showing malignant transformation in the form of malignant rhabdoid tumor in a male patient in the English language literature.     

Endometrial stromal sarcoma of the uterus with rhabdoid features

A case of endometrial stromal sarcoma of the uterus with rhabdoid features, occurring in a 57 year old woman is reported. Electron microscopy and immunohistochemistry revealed that the rhabdoid cells contained intermediate filaments which were positive for vimentin, cytokeratin, alpha-smooth muscle actin, and muscle specific actin, but not for myoglobin and desmin. This indicated that the tumor in this case differed somewhat from the three rhabdoid tumors and an endometrial stromal sarcoma with rhabdoid differentiation previously reported and that, therefore, these tumors were heterogeneous.     

子宫腺肉瘤的临床病理特点及鉴别诊断

目的：分析子宫Ｍｕｅｌｌｅｒｉａｎ腺肉瘤的临床病理特点及其鉴别诊断。方法：观察１３例子宫腺肉瘤临床病理特点，并进行组织化学和免疫组化染色分析。结果：病人年龄２６￣６７岁，主要表现为阴道不规则流血和子宫增大。肿瘤位于宫体内膜、宫颈内膜和子宫肌壁。镜下腺上皮呈Ｍｕｅｌｌｅｒｉａｎ上皮系列。间质细胞异型分级为Ⅰ￣Ⅱ级，核分型４￣３２个／１０ＨＰＥ，具有腺周套袖和息肉样突入腺腔的结构。４例伴肉瘤过度生长。     

颈静脉球瘤1例及副神经节瘤文献复习

目的探讨颈静脉球瘤的临床病理特征和副神经节瘤的生物学行为。方法对1例颈静脉球瘤进行组织学观察和免疫组化标记,同时复习相关文献。结果颈静脉球瘤的组织学改变类似于其他部位的副神经节瘤,免疫组化标记:瘤细胞Syn、CgA和NSE均阳性,EMA、CK、HMB45、CD34和CD99均阴性。结论颈静脉球瘤是一种起源于副神经节的少见肿瘤,呈侵袭性生长,有低度恶性的生物学行为。根据肿瘤的组织学特征,结合CT或MR I影像学资料和免疫组化标记可以作出诊断。     

Malignant rhabdoid tumor of the vulva: Case report with cytological, immunohistochemical, ultrastructural and DNA ploidy studies and a review of the literature

A case of malignant rhabdoid tumor of the vulva in a 25-year-old female was examined. The patient presented with a subcutaneous nodule in the lefl labium majus. Smears of the material obtained by percutaneus fineneedle asplratlon demonstrated clusters of atypical cells with prominent nucleoli. The tumor measured 6 × 5 × 5 cm and appeared tan to brown on the cut surface and partly cystlc. Pathological findings obtalned from intraoperative frozen tlssue sections had been originally interpreted as rhabdomyosar-coma. Light microscopic examination revealed that polyge nal tumor cells having vesicular nuclei with prominent nucleoli were arranged in sheets and the great majority of the tumor cells contained an eosinophillc globular paranu-clear cytoplasmic Inclusion. Ultrastructurally, this cytoplas-mic inclusion corresponds to whirls of intermediate filaments. Vlmentln immunoreactlvity was detected in both the cytoplasm and cytoplamic inclusion of almost all the tumor cells. No cytokeratin and desmin immunoreactivlty were detected In the tumor cells. The Ki-67 labeling index was 36% and the DNA content of the tumor cells, which was examined by image cytometry, demonstrated diploidy (DNA Index = 0.95).     

肾素瘤1例临床病理及文献复习

目的：阐述肾素瘤的病因，病理特征及鉴别诊断要点。方法：对１例肾素瘤进行大体、光交易、免疫组化镜观察，并复习有关文献。结果：肾素瘤包膜完整光下酪似血管外皮瘤；免疫线化，部分细胞Ｖｉｍｅｎｔｉｎ呈阳性反应，瘤组织的腺样及乳头样结构区瘤细胞ｃｙｔｏｋｅｒａｔｉｎ呈阳性反应。电下瘤细胞胞浆内可见多量圆形分泌颗粒及棱莆和梯形结晶样物。结论：肾素瘤是发生于变更的肾小球入球小动脉血管平滑肌细胞的罕见衣性肿瘤，明