胰腺实性-假乳头状肿瘤内PTEN和nestin的表达

目的 探讨胰腺实体假乳头肿瘤（SPTP）细胞可能的组织学起源。方法 复习25例SPTP的临床病理特征,常规HE和超微结构观察,免疫组化EnVision法染色检测肿瘤细胞PTEN和nestin等的表达。结果 25例SPTP中女性22例,男性3例,中位年龄22．3岁。肿瘤主要位于胰腺内,1例位于后腹膜并与胰腺相连。1例伴有肝转移。肿瘤呈囊实性,出血、坏死明显。肿瘤细胞大小形态较一致,实性和假乳头状排列,部分似“室管膜样”菊形团。核卵圆形,有核沟,核仁不明显,核分裂象少见。肿瘤细胞均有PTEN阳性表达（18／18,100％）,44％（8／18）的病例有nestin的表达。超微结构观察细胞内可见酶原样分泌颗粒和神经内分泌颗粒。结论 胰腺实体假乳头肿瘤可能起源于胰腺多能干细胞。     

胰腺实性—假乳头状肿瘤8例临床病理学分析

目的探讨胰腺实性-假乳头状肿瘤(solid-pseudopapillary tumor of pancreas,SPTP)的临床病理学特点、诊断和鉴别诊断、治疗及其预后。方法对8例SPTP患者的临床、病理学特点及免疫组化结果进行研究,并复习相关文献。结果 8例均为女性,年龄19～38岁,平均28岁。肿块直径3～15 cm。镜下肿瘤由乳头区和囊实区混合组成,瘤细胞围绕纤维血管轴心形成特征性假乳头结构。免疫表型:CK(3/8)、vimentin(8/8)、NSE(8/8)、CD56(8/8)、ER(0/8)、PR(5/8)、α-AT(8/8)、CgA(1/8)、Syn(8/8)、E-cadherin(0/8)、β-连环蛋白(8/8)和cyclinD1的核阳性率均大于40%。随访6例患者17～131个月,均无肿瘤复发及转移,并存活至今。结论 SPTP是一种好发于年轻女性,具有低度恶性潜能的少见胰腺肿瘤,其病理形态特征和免疫组化标记对SPTP的诊断和鉴别诊断具有重要价值。     

胰腺实性假乳头状肿瘤3例并文献复习

目的探讨胰腺实性假乳头状肿瘤(solid pseudopapillary tumor of the pancreas,SPTP)复发和转移病例的临床病理特征、诊断、鉴别诊断、治疗及预后。方法采用HE染色和免疫组化EnV ision法分析3例SPTP的病理学及免疫表型特征,并复习相关文献。结果镜下可见肿瘤细胞围绕小血管形成特征性的假乳头状结构,可见少量出血及坏死、黏液样变性及胆固醇沉积等现象;细胞分化较好,异型性不明显,未查见细胞核分裂象。免疫表型:β-catenin、vimentin、CK、CK8、PR、CD56等均呈阳性,Syn、CEA、CgA等呈弱阳性;ER呈阴性,Ki-67增殖指数5%。结论 SPTP是胰腺外分泌肿瘤的特殊类型,临床较少见,恶性程度低,预后良好,诊断主要依赖其组织病理学特征及免疫表型。     

胰腺神经内分泌肿瘤与胰腺实性假乳头状瘤中β-catenin、E-cadherin及CD10的鉴别诊断价值

目的探讨胰腺神经内分泌肿瘤(pancreatic neuro endocrine neoplasms,PNEN)与胰腺实性假乳头状瘤(solid-pseudopapillarytumor of pancreas,SPTP)的临床病理学特征,并分析联合应用免疫组化标记β-catenin、E-cadherin及CD10的鉴别诊断价值。方法收集2007年3月～2017年2月经明确诊断的53例PNEN和40例SPTP,回顾性分析其临床病理资料,采用免疫组化法观察免疫标志物β-catenin、E-cadherin、CgA、Syn、CD56、CD10、CK在两组肿瘤中的表达。结果 PNEN和SPTP的病理学形态有重叠,β-catenin在PNEN中均为细胞膜、细胞质阳性,而在SPTP中则为细胞核阳性;E-cadherin在PNEN中为细胞膜、细胞质阳性(100%),而在SPTP中均显示细胞膜表达丢失;CD10在SPTP中的阳性率为100%(40/40),在PNEN中不表达。CgA、Syn、CD56、CK在PNEN及SPTP均有不同程度表达。结论β-catenin、E-cadherin及CD10阳性对PNEN和SPTP的鉴别诊断具有特异性,是较有价值的免疫组化标志物。     

胰腺实性假乳头状瘤1例并文献复习

胰腺实性假乳头状瘤(solid-pseudopapillary tumor of pancreas,SPTP)是一种极其少见的低度恶性肿瘤,好发于年轻女性,常常易被误诊为良性疾病,临床表现没有明显的特异性,主要是腹痛,诊断和鉴别诊断依靠其临床特征、影像学表现、病理组织学特点及免疫组织化学结果,首选治疗方法是手术切除,这类病人大多预后良好.     

恶性血管周上皮样细胞肿瘤2例并文献复习

目的 探讨恶性血管周上皮样细胞肿瘤(malignant perivascular epitheliod cell tumor,PEComa)的临床、病理特征及鉴别诊断.方法 对2例恶性PEComa进行临床、病理形态学、免疫表型及电镜观察,并复习相关文献.结果 镜下见肿瘤组织由梭形细胞及上皮样细胞构成,细胞异型性明显,细胞胞质透明或嗜酸,细胞核大,有核仁,瘤细胞由纤细的纤维结缔组织及薄壁血管将其分隔成巢状、腺泡状、片状结构,部分区域可见多核瘤巨细胞伴肿瘤坏死,核分裂多见.免疫表型:肿瘤细胞表达HMB-45、S-100、SMA及desmin;电镜下见肿瘤细胞胞质内可见高电子密度核心的内分泌小体和少许不成熟的黑色素小体.结论 恶性PEComa罕见,病理形态多样,熟悉其临床、病理形态、免疫表型及电镜下改变,有助于临床、病理医师对其正确诊断及鉴别诊断.     

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。     

肾小球旁器细胞瘤的临床病理观察

目的 研究肾小球旁器细胞瘤的病因、临床病理及超微结构特点。方法 对5例患者的临床症状、肿瘤的大体和镜下特征、免疫组化及超微结构进行观察与分析。结果 多数患者术前都有高血压等临床症状;肿瘤大体包膜完整,光镜下可见腺管样及乳头样结构,部分区域有血管外皮瘤样图像;免疫组化部分细胞呈actin阳性反应;电镜下瘤细胞胞质内可见菱形或圆形的结晶颗粒。结论 肾小球旁器细胞瘤的诊断应结合临床、组织病理及电镜,其中电镜观察到结晶颗粒是诊断最可靠的依据。     

儿童胰腺实性假乳头状瘤多排螺旋CT表现

目的:探讨儿童胰腺实性假乳头状瘤（SPTP）的多排螺旋CT（MDCT）表现。 方法:回顾性分析经手术病理证实为SPTP的10例患儿临床及MDCT资料,分析总结其特征表现。 结果:10例患儿均为女性,SPTP均为单发病灶,位于胰腺头颈部3例,胰腺体尾部7例。肿瘤最大径为2.5~13.8 cm,平均值为5.94 cm。8例呈类圆形,2例呈分叶状。10例均边界清晰,8例有完整包膜。平扫1例肿瘤呈均匀低密度,增强无明显强化;其余9例呈囊实性,8例以实性为主,1例囊实性比例相当,其中1例可见包膜下弧形钙化,实性成分动脉期呈轻度强化,强化程度低于正常胰腺组织,门脉期呈渐进性不均匀强化,囊性成分无强化。1例肿瘤出现肝内胆管和主胰管轻度扩张,1例出现主胰管轻度扩张。所有肿瘤均未见肝脏及淋巴结转移。 结论:儿童SPTP好发于女性,其CT表现具有一定的特征性,对诊断和鉴别诊断具有一定意义。     

甲状腺显示胸腺样分化的癌6例临床病理分析

目的探讨甲状腺显示胸腺样分化的癌(carcinoma showing thymus-like differentiation,CASTLE)临床病理学特点及其鉴别诊断。方法对6例CASTLE进行组织形态学观察、免疫组化标记及电镜观察。结果镜下肿瘤组织常呈巢状分布伴较多淋巴细胞反应,癌细胞呈多边形或梭形,核卵圆形,呈空泡状,有清楚的小核仁,核分裂象少。免疫组化示癌细胞表达CD5及CD117等。电镜下癌细胞含较多的线粒体及张力原纤维,细胞间桥粒丰富。结论甲状腺CASTLE的诊断依靠形态学及免疫组化标记,超微病理结构特征有助于与其他甲状腺恶性肿瘤相鉴别。     

Solid-pseudopapillary tumor of the pancreas: a neoplasm with distinct and highly characteristic cytological features

The solid-pseudopapillary tumor of the pancreas (SPTP) is an unusual low-grade malignant epithelial tumor affecting predominantly adolescent girls and young women. Although approximately 500 cases of SPTP have been described in the last 40 yr, its pathogenesis remains uncertain. However, the clinical features of this neoplasm are very characteristic and SPTP must be suspected in any young woman with a cystic or partially cystic pancreatic mass. In this report, we describe the cytologic features of seven cases of SPTP investigated by preoperative fine-needle aspirates. The analysis of the cytologic features in these cases and in 43 cases collected from the literature reveals that they are highly characteristic and quite distinct from those of other cystic or solid tumors of the pancreas. On this basis, a cytologic diagnosis of SPTP may be rendered with great confidence, not only in clinically typical examples, but also in unusual presentations, such as in older patients, in males, in ectopic locations, and in metastatic sites.     

胰腺实性假乳头状瘤的 CT 表现与病理分析

目的：探讨胰腺实性假乳头状瘤（ SPTP）的CT特征及病理改变。方法：回顾性分析6例SPTP的临床资料,其中男1例、女5例,平均年龄24．5岁。所有患者均进行CT平扫及增强扫描。病灶位于胰头2例,胰体1例,胰尾3例;胰尾3例中,1例发生肝脾多发转移,1例发生肝脏多发转移。总结其CT表现及病理学表现特征。结果：CT平扫病灶大小约3 cm ×5 cm～11 cm ×10 cm,呈圆形、类圆形或分叶状囊实性肿块,均可见包膜;1例病灶周缘可见细条状及斑点状钙化,2例病灶内可见斑片状出血灶。增强扫描病灶实性部分呈渐进性持续强化,可见特征性表现“浮云”征。所有病例均未见胆管或胰管扩张。6例病变病理检查均显示肿瘤有完整或不完整的纤维包膜,瘤组织由实性区、假乳头区混合组成,瘤细胞形态较一致,部分区域有出血、坏死及退行性变。结论：SPTP好发于青年女性,其CT表现有一定特征性,多期增强扫描有助于诊断与鉴别诊断;肿瘤细胞以纤细的纤维血管为轴心形成特征性的分支状假乳头状结构是其组织病理学特征性改变。     

Endoscopic ultrasound-guided fine-needle aspiration cytology diagnosis of solid-pseudopapillary tumor of the pancreas: a rare neoplasm of elusive origin but characteristic cytomorphologic features

Clinical histories, endoscopic ultrasound (EUS)-guided fine-needle aspiration (FNA) material, and immunohistochemical stains performed on cell block samples of 6 solid-pseudopapillary tumors of the pancreas (SPTPs) were reviewed in the cases of 5 females (13-58 years) and 1 man (57 years); all had abdominal pain. Preliminary cytologic diagnoses at endoscopy included 1 SPTP 2 low-grade neoplasms, and 3 pancreatic endocrine tumors. Variable numbers of branching fragments with central capillaries and myxoid stroma were seen in the smears of 5 of 6 cases but were more apparent in the cell block material of all cases. The cells had bland nuclear features and rare grooves. Extensive necrosis was noted in 1 case and rare mitotic figures in 1. SPTPs showed strong cellular immunoreactivity for vimentin and focal weak keratin reactivity. Neuron-specific enolase, alpha1-antitrypsin, and alpha1-antichymotrypsin stains performed in 2 cases were strongly positive. Subsequent surgical resection confirmed all diagnoses. EUS-guided FNA diagnosis of SPTP is accurate. The characteristic branching papillae with myxoid stroma are best seen in cell block slides. Clinical setting, cytomorphologic features, and immunostains of the cell block help distinguish SPTP from pancreatic endocrine tumors, acinar cell carcinoma, and papillary mucinous carcinoma.     

Solid-pseudopapilläre Neoplasien

Solid pseudopapillary neoplasms (SPN) of the pancreas represent a special tumor entity, both morphologically and biologically. They form large solitary tumors that occur predominantly in young women. Histologically, they show solid, pseudopapillary, and pseudocystic patterns. The tumor cells are monomorphous and typically express vimentin, neuron-specific enolase, nuclear beta-catenin, and the progesterone receptor. Complete resection cures the tumor in about 90% of the cases. However, because recurrences and even metastases may occur in a small number of cases, SPN are classified as low-grade malignant tumors. Predicting malignancy histologically is not yet possible. The most important differential diagnosis to consider is neuroendocrine tumor of the pancreas. The etiology and pathogenesis of SPN are obscure.     

Solid and papillary epithelial tumor of the pancreas: cytologic and histologic features

Cytologic and histologic features in a case of solid and papillary epithelial neoplasm (SPEN) of the pancreas in a 34-year-old female are presented. In the fine-needle aspiration material, there was a predominance of pseudopapillary fragments with delicate fibrovascular core lined with one or more layers of uniform tumor cells with clear cytoplasm. There were also acinar-like or rosette-like structures and dispersed tumor cells. Tumor cells had small, round to oval nuclei, with finely granular chromatin and inconspicuous nucleolus. Rarely, nuclear grooves were present. Mitoses were not seen. Histologically, the encapsulated tumor was predominantly solid, with focal pseudocystic and pseudopapillary areas; foreign body granulomas were focally present. Tumor cells were small and uniform with clear cytoplasm. Mitoses were extremely rare. Immunohistochemically, the tumor cells revealed diffuse positivity of vimentin, and focal positivity for alpha 1-antitrypsin and CD68. No immunoreactivity for chromogranin, synaptophysin, cytokeratin, HMB 45, estrogen and progesterone receptors was found. In differential diagnosis, it is important to distinguish SPEN mainly from mucinous tumors of the pancreas, microcystic adenoma and pseudocysts, but also from endocrine tumors of the pancreas, acinic cell carcinoma, renal cell carcinoma, adrenal cortical adenoma and primary extrapulmonary sugar tumor.     

29例上皮样胃肠间质瘤的临床病理特征

目的:探讨29例上皮样胃肠间质瘤(gastrointestinal stromal tumors,GIST)的组织学形态和免疫组织化学特点以及诊断和鉴别诊断,对临床正确诊断治疗和判断预后具有十分重要的临床意义.方法:回顾性分析安阳市肿瘤医院病理科2009年月3月至2016年8月321例完整切除GIST标本,经筛查并重新阅片诊断上皮样GIST 29例.结果:29例上皮样GIST,发生胃部15例,小肠2例,肠系膜3例,直肠4例,腹腔3例,腹膜后1例,盆腔1例.恶性GIST 25例,良性4例.瘤细胞丰富,胞质嗜酸或透明,部分瘤细胞核质比高,核大小不等具有多形性,核分裂较多,可伴有多灶凝固性坏死,间质多数伴有黏液样变性.组织结构形成器官样、片状、巢状及腺泡状等.免疫组织化学CD34,DOG-1在上皮样GIST均弥漫阳性(阳性率100%),CD117阳性率(86%).结论:上皮样GIST发生部位广泛,形态多变,易误诊其他上皮样分化的肿瘤;免疫组织化学CD34,DOG-1,CD117在上皮样GIST诊断及鉴别诊断中具有重要价值;如肿物较大、细胞丰富、核分裂多、间质黏液样变性;绝大多数要考虑恶性GIST.     

Endoscopic ultrasound-guided fine-needle aspiration of undifferentiated carcinoma with osteoclast-like giant cells of the pancreas: a report of 2 cases with literature review

Undifferentiated carcinoma with osteoclast-like giant cells of the pancreas is rare. Histologically it mimics the giant cell tumor of the bone and may be associated with a ductal adenocarcinoma. We recently encountered two such cases, both of which were biopsied by EUS-guided FNA. Abundant multinucleated osteoclast-like giant cells and many uniform mononuclear cells were present in case 1 so that the diagnosis was made. In case 2, many mononuclear tumor cells with vacuolated and basophilic cytoplasm were present, and rare osteoclast-like giant cells were seen. A diagnosis of adenocarcinoma was made. In both cases, no conspicuous nuclear pleomorphism was noted in the mononuclear cells or the multinucleated giant cells. The histology of case 2 revealed a pure undifferentiated carcinoma with osteoclast-like giant cells. In addition, a liver biopsy revealed globular amyloidosis. To our knowledge, this is the first report of pancreatic undifferentiated carcinoma with osteoclast-like giant cells sampled by EUS-guided FNA and the first case of hepatic globular amyloidosis associated with this tumor.