肠系膜上皮样型炎性肌纤维母细胞瘤1例并文献复习

目的探讨肠系膜上皮样型炎性肌纤维母细胞瘤(epithelioid inflammatory myofibroblastic tumor,EIMT)的临床病理学特征、免疫表型、分子遗传学改变、诊断和鉴别诊断、治疗及预后。方法回顾性分析1例肠系膜EIMT的临床病理学形态、免疫表型、分子遗传学以及治疗特点,并复习相关文献。结果肿瘤细胞排列疏密不等,部分呈片状或弥散,间质明显黏液样变性,见大量炎细胞浸润;瘤细胞呈圆形、多角形,少数为梭形,胞质丰富,嗜双色性,呈上皮样,染色质空泡状,见明显大核仁,核分裂象罕见;未见肿瘤性坏死。免疫表型:瘤细胞ALK、desmin、vimentin、CD30和D2-40均阳性,Ki-67增殖指数约30%,CK(AE1/AE3)、EMA、CD117、DOG1、CD34、SMA、S-100蛋白、HMB-45、CAM5.2、CD31、BCL-2、CD99、Calretinin、FⅧRAg和HBME-1均阴性。FISH检测显示ALK基因重排。结论 EIMT是一种炎性肌纤维母细胞瘤的罕见组织学类型,其诊断主要依据典型的组织学改变、免疫表型,特别是ALK在核膜和胞质阳性,分子遗传学改变将为靶向治疗提供可靠依据。治疗以手术治疗为主。     

恶性腹膜间皮瘤临床病理进展

恶性腹膜间皮瘤(malignant peritoneal mesothelioma,MPM)是一种非常罕见的腹膜恶性肿瘤,预后差.MPM在诊断病理学上面临一些挑战,诊断MPM时需搜集详细的临床信息,MPM的一些形态变异亚型,在实际工作中常联合采用一组抗体进行诊断.MPM每个组织学分类均有其独特的鉴别诊断.MPM常见的遗...     

近端型上皮样肉瘤1例及文献复习

目的 探讨近端型上皮样肉瘤(proximal-type epitheliod sarcoma,PES)的临床病理特点及鉴别诊断.方法 报道1例PES患者的临床资料,并对其进行组织学观察、免疫组化(EnVision-plus法)标记及电镜观察.结果 患者女性,24岁.临床主要表现右侧大阴唇逐渐增大的无痛性肿块.组织学特征:瘤组织呈结节状生长,瘤细胞椭圆形、多边形,胞质嗜双色或嗜酸性,核空泡状,有明显核仁.常见横纹肌样细胞,未见肉芽肿样改变.免疫表型:瘤细胞vimentin、β-catenin阳性,EMA局灶阳性;而SMA、MyoD1、desmin、CD117、S-100、HMB-45、Melan A、CD99、Syn、CD57、CKAE1/AE3、CK8/18、CK19、CA125、CEA、FⅧRAg、CD31、CD34、Calretinin、 CD45和CD68阴性.电镜:细胞胞质内见较多线粒体、少量粗面内质网及溶酶体,未见黑色素小体、神经内分泌颗粒及肌丝,细胞间连接不明显,未见肿瘤间质.结论 PES为一种罕见的软组织肿瘤,与经典型ES相比,具有独特的组织学特征,并具有更强的侵袭性.     

免疫组化在鉴别上皮样间皮瘤和肺鳞状细胞癌中的比较研究

上皮样间皮瘤和鳞状细胞癌时常表现为多样的组织学形态因此难以鉴别。为了比较既往的间皮瘤和鳞癌标记在二者鉴别时的价值，作者对30例组织学上主要表现为实性结构的胸膜上皮样间皮瘤和30例非角化型肺鳞状细胞癌进行免疫组化比较研究。结果：间皮瘤中免疫组化阳性率分别为calretinin（100％），mesothelin（100％），keratin7（100％），podoplanin（93％），WT1（93％），keratin5／6（93％），Ber—EP4（13％），p63（7％），MOC-31（7％），BG-8（7％），B72．3（0），CEA（0），leu—M1（0）和TTF1（0）。     

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。     

卵巢成熟性畸胎瘤伴发的黏液上皮性肿瘤形态学异质性及其与腹膜假黏液瘤的关系

卵巢成熟性畸胎瘤伴发的黏液上皮性肿瘤是一组异质性的肿瘤，其组织学特征、免疫表型及其与经典的腹膜假黏液瘤（PMP）的关系除近期的几篇报道外，目前尚无全面的研究。本研究通过42例卵巢成熟性畸胎瘤伴发的黏液上皮性肿瘤评估其形态学特征、免疫表型和临床特点。患者年龄17至66岁（平均39岁），肿瘤大小平均10．1cm，中位8cm。除1为双侧性外，余均为单侧性。所有畸胎瘤均为成熟成份。     

新型PLAG1基因重排可用于子宫黏液样平滑肌肉瘤与黏液样间质肿瘤的鉴别

子宫黏液样平滑肌肉瘤是一种罕见且具侵袭性的恶性肿瘤,易误诊为良性,目前遗传学改变尚不明确。作者选取19例既往诊断为子宫黏液样平滑肌肉瘤或具有黏液样特征的平滑肌肉瘤,分析其临床病理、免疫表型及分子学特征。该系列肿瘤均进行RNA测序,并通过免疫组化标记BCOR、PLAG1和ALK(D5F3)和FISH检测PLAG1、BCOR、BCORL1、HMGA2和ALK基因,排除4例伴BCOR和ALK重排的黏液样肿瘤。剩余15例确诊为黏液样平滑肌肉瘤。患者中位年龄为50岁,其中8例FIGO分期分别为I期(3例)、II期(2例)、Ⅲ(2例)和Ⅳ(1例);另7例分期情况不明。肿瘤直径为10~24 cm,所有病例肿瘤基质均呈黏液样,其中2例伴嗜酸性。肿瘤细胞呈梭形或上皮样形,细胞核伴轻至重度异型,核分裂数<1~14个/10 HPF。6例(40%)伴肿瘤坏死。7例免疫组化弥漫表达PLAG1,其中4例(27%)测序检测有新型TRPS1-PLAG1或RAD51B-PLAG1基因融合,且经FISH证实存在PLAG1重排;而有无PLAG1基因融合患者,其组织学特征上并无差异。     

腹膜弥漫性蜕膜细胞样型恶性间皮瘤一例

患者男,76岁。因膀胱癌术后14年,腹胀1年,加重半年于2003年4月入院。患者14年前因无痛性血尿于我院确诊为膀胱癌,行电凝切除术,术后病理：膀胱移行细胞癌。术后定期膀胱镜复查,未发现复发及转移。1年前出现反复腹胀,半年前加重伴体重下降,2个多月减少10kg。既往有30多年吸烟史,无石棉接触史,无家族史。体检：生命体征     

黏液纤维肉瘤上皮样变异型：17例黏液纤维肉瘤临床形态学谱系种种

黏液纤维肉瘤（MFS）是老年患者最常见的软组织肉瘤之一，好发于四肢皮下组织。多年来，该瘤普遍被认为是“黏液样恶性纤维组织细胞瘤（MFH）”，然而几项研究显示：与现认为是未分化肉瘤废纸篓的多形性MFH相反，MFS具有可再生的细胞结构特点和一致的临床病理学特征，因此，MFS显然组成一个明了的肿瘤实体，更适用于目前的命名。本文报道了一种以往未被描述过的显示上皮样形态学特点的MFS的变异型。作者从570例MFS档案中检索出17例上皮样MFS，对其临床病理特点、自然病程和主要的鉴别诊断进行了分析讨论。[第一段]     

H-caldesmon:一个诊断上皮样型胸膜恶性间皮瘤的阳性标记物

胸膜上皮样间皮瘤和肺腺癌的鉴别在诊断外科病理中是一个难点,特别是仅凭HE形态进行诊断。目前间皮瘤的诊断主要依赖于一组阳性标记和阴性标记,但无一种抗体对该肿瘤的诊断有绝对的敏感性和特异性。在阳性标记中,thrombomodulin、CK5/6、WT1、calretinin、odoplanin和D2-40似乎     

Peripheral lymphadenopathy as the initial manifestation of pericardial mesothelioma: a case report

Lymphadenopathy is an unusual initial presentation of malignant mesothelioma. We describe a case of extrathoracic lymphadenopathy as the only initial manifestation of primary pericardial malignant mesothelioma. The diagnosis of metastatic malignant mesothelioma was made by excisional biopsy of the supraclavicular lymph node and later confirmed by cytologic examination of pericardial fluid. The clinical and pathological features of this case, including positron-emission tomography (PET), immunohistochemistry, and electron microscopy, are discussed.     

Early cytological diagnosis of diffuse malignant mesothelioma of the peritoneum: a case report

Mesotheliomas are the most frequent primary malignant tumors of serosal cavities with a poor prognosis. A definitive and early diagnosis on effusion samples is important, because recent advances in therapy for patients with mesothelioma may result in an improved outcome if they are applied to stage I disease.We report a case of malignant peritoneal mesothelioma diagnosed repeatedly by cytology in ascites fluids 1.5 yr before the diagnosis was confirmed by biopsy histology. The cytological diagnoses were supported by immunocytochemistry, DNA-cytometry, and AgNOR-analysis. Routine cytology supported by three adjuvant methods enabled us to correctly establish the diagnosis. Our case suggests that a cytological diagnosis of malignant mesothelioma supported by adjuvant methods should not be rejected even if based on negative histological results.     

双侧腹股沟脂肪疝1例

腹股沟疝是一种常见病、多发病,易于误诊.腹股沟脂肪疝是指腹膜肪经腹股沟深环(内口)突出,甚至进入阴囊,形成"脂肪疝",有的可与斜疝共存.     

耻骨上疝合并右腹股沟斜疝1例及文献总结

腹壁切口疝是腹部外科手术后常见的并发症,切口疝是由于原手术的腹壁切口筋膜或/和肌层未能完全愈合,在腹腔内压力作用下形成的腹外疝,其疝囊可有完整或不完整的腹膜上皮细胞,一般见于腹前壁切口[1]。其发生原因复杂多样,与患者年龄较大、营养状况不佳、糖尿病或肥胖、长期使用类固醇激素、免疫功能低下及长期吸烟史等均相关。     

Hepatic epithelioid hemangioendothelioma metastasized to the peritoneum,omentum and mesentery: a case report

Epithelioid hemangioendothelioma (EHAE) is a malignant vascular tumor derived from endothelial cell often misdiagnosed as Hepatic carcinoma on the basis of radiological features. Till now etiology of this rare curiosity is unknown but it is related with use of oral contraceptives pills (OCP), liver trauma, exposure to vinyl chloride and hepatitis. We herein report on a case which failed to be diagnosed by cytopathology, computed tomography (CT) and magnetic resonance imaging (MRI). Patient was a 46 yr old man presented with abdominal distension for a month. Initial liver function test (LFT) was increased whereas renal function test (RFT) and alpha-fetoprotein (AFP) were normal. His abdominal ultrasound revealed multiple hypoechoic nodules and multiple liver calcifications. Subsequently laparoscopic omental biopsy and Ultrasound guided liver biopsy was done showing the neoplastic cells scattered in fibrous stroma. The immunohistochemistry for endothelial tumor cells stained positive for Vimentin (+++), CD10 (+++), CD34 (++), CD31 (+), Factor VIII antigen (focal) (+) and low proliferative activity for ki-67. Our case is very interesting in which patient admitted with nonspecific symptoms of abdominal pain and diagnosed to be a Malignant Hepatic EHAE metastasized to the peritoneum, omentum and mesentery. The patient was on thalidomide 50 mg/day and increased to 100 mg/day. 5-Flurouracil (FU) intraperitoneal chemotherapy and other symptomatic and supportive treatment was given to the patient. Our case highlights on the importance of immunohistopathological diagnosis, compare the radiological findings of this disease and discuss the treatment strategy with review of available literature.     

Metastatic adenocarcinoma presenting as an inguinal hernia: a case report and review of literature

A 61 year old man presented with an inguinal hernia with no other significant symptoms. Histopathological examination of the hernial sac revealed metastatic deposits of a mucin secreting adenocarcinoma which was confirmed by subsequent tumor marker levels. Patient was put on chemotherapy for disseminated adenocarcinoma and is tolerating it well. This case emphasizes the need to carefully examine all hernial sacs received for pathological examination.     

Malignant mesothelioma of the peritoneum invading the liver and mimicking metastatic carcinoma: a case report

We present a case of malignant mesothelioma of the peritoneum with massive direct invasion to the liver in a 58-year-old Japanese woman. She had no history of asbestos exposure or other malignancies. Abdominal computed tomography revealed one 8-cm intrahepatic mass adjacent to the abdominal wall with peritoneal thickening, multiple smaller nodules in the peritoneal cavity, and intra-abdominal lymphadenopathy. Liver biopsy showed a small cluster of atypical cells similar to epithelial neoplasm, which formed a tubulopapillary structure. The tumor cells were positive for calretinin with strong nuclear and cytoplasmic expression together with podoplanin (D2-40) and some cytokeratins, but were negative for hepatocyte paraffin 1 and other adenocarcinoma markers. We confirmed a diffuse peritoneal mesothelioma with direct invasion to the liver. Liver masses with other peritoneal nodules are mostly encountered as metastatic diseases. However, the possibility of mesothelioma should be considered, even in women without an apparent history of asbestos exposure.     

Laparoscopic diagnosis and desincarceration of inguinal Richter hernia: a case report

In the cases of incarcerated Richter hernia the diagnosis is difficult especially for obese patients. Laparoscopic diagnostics and desincarceration, can be combined with mini laparotomy in lieu of bowel resection and open hernioplasty, or conversion to classic laparotomy and open hernioplasty. Here is presented a case of incarcerated Richter inguinal hernia managed through the combination of laparoscopy, mini laparotomy for segmental bowel resection and open hernioplasty.