节细胞神经母细胞瘤1例

患者男,59岁.因左腹、腰部胀痛,逐渐加重1个月于1998年1月8日入院.平素健康.查体:左上腹触及一6cm×5cm质硬肿块,固定、轻压痛.B超检查:左肾前上极探及一8.0cm×7.8cm,低回声不均质肿块,包膜完整、增厚.CT扫描示左侧腹膜后肿瘤,与胰、左肾关系密切.X线胸片未见心肺异常.CEA<20μg/ml.经手术证实,肿瘤位于腹膜后、胰体尾部及左肾上极之间,并侵犯左肾门及肾蒂血管,血供丰富.肿瘤为灰红色,卵圆形9cm×7cm×6cm大小,切面见大片出血、坏死,部分囊性变,伴有鱼肉样组织.肿瘤免疫组化结果:NSE( )、FN(-)、GFAP(-)、S_(100)节细胞( )、小细胞(-).病理诊断为节细胞神经母细胞瘤.     

小儿肾上腺节细胞性神经瘤1例

节细胞性神经瘤是罕见的良性肿瘤,来源于交感神经系统,发生于小儿肾上腺极为少见。本文报告1例如下。     

肾上腺节细胞神经瘤5例报告

肾上腺节细胞神经瘤是一种少见的良性肿瘤,多发于肾上腺髓质。我们自1992年2月—1998年10月共收治5例,均经病理证实,现报告如下。1　临床资料本组5例,男4例,女1例。年龄20岁～66岁,平均57岁。肿瘤发生在右侧肾上腺3例,左侧2例。瘤体直径在6.0cm以上者4例,最大者9.0cm×8.0cm×5.0cm,最小5.5cm×4.5cm×4.0cm。5例均为体检B超发现肾上腺占位收稿日期:2000-01-03作者单位:1.江苏省淮阴市第一人民医院,江苏　淮阴　223300;2.上海长海医院,上海　200433而就诊,均无头痛、头晕、出汗及高血压史。5例作CT检查,均显示患侧肾上腺占位性病变,呈椭圆…     

肾上腺巨大节细胞神经瘤1例报告

1　病例报告患者 ,男性 ,47岁。Ｂ超检查发现左肾上腺肿物 10天。患者于 2 0 0 0年 8月 11日体检时行Ｂ超检查 ,于左肾上腺区检查探及一 110ｍｍ× 88ｍｍ× 86ｍｍ实质性肿物 ,考虑为肾上腺无功能性腺瘤。患者平时无明显自觉症状 ,入院行 2 4ｈ血压动态监测正常 ,无腰背压痛、满月脸、水牛背、皮肤痤疮及精神异常。逆行肾盂造影检查发现左肾盂肾盏受推压向下移位 ,左肾上方见一软组织块影 ,边界不清。彩色多普勒检查发现左肾上腺区有一 113 .2ｍｍ× 10 6 .1ｍｍ× 97.3ｍｍ的等回声偏低的实质性团块 ,包膜完整 ,其内为星点状。 2 0 0 0年 …     

咽节细胞神经瘤1例

患者，男，３３岁。５个月前因感冒后出现咽痛、发烧，吞咽时不畅，４～５天后缓解。自觉双侧扁桃体增大，左侧尤甚。１周后又出现吞咽不畅，自觉轻度呼吸困难。在当地医院行扁桃体切除术，术后出现吞咽、呼吸困难。于２００１年１０月１１日住院治疗。查体：T３６．１℃，P７２次／分，R１８次／分，Bp１５／１０kPa。专科检查：咽部粘膜充血，双侧扁桃体已切除，表面有白膜生长。左侧咽组织明显膨隆，超越中线，悬雍垂向右移位。CT示左侧咽旁有一圆形低密度影，界限清楚。２００１年１０月３０日手术。术中见：左侧咽部组织明显膨隆，…     

肾上腺节细胞神经瘤四例报告并文献复习

目的探讨肾上腺节细胞神经瘤的病理特点、临床症状及诊断治疗方法。方法回顾性分析临床所见4例肾上腺节细胞神经瘤并总结国内外病例文献资料。结果经手术治疗,术后病理诊断为肾上腺节细胞神经瘤,4例均痊愈出院。结论肾上腺节细胞神经瘤是一种罕见的良性肿瘤,多数临床症状不明显。诊断主要依赖B超、CT、MRI,确诊需要病理学检查,手术切除是其主要的治疗方法,多数预后良好。     

颌下节细胞神经瘤1例

节细胞神经瘤是发生于外周交感神经系统的罕见良性肿瘤,由成熟神经节细胞、施万细胞和神经纤维构成。因节细胞神经瘤的临床症状、体征并无特异性,目前其诊断主要依靠影像学检查,超声为首选的检查方法,现将本院1例颌下节细胞神经瘤患者的诊治经过报告如下。     

节细胞神经母细胞瘤1例

患者,男,13岁.因右侧腰痛伴低热3天,在襄樊市中医院做双肾CT示右肾上腺肿瘤于2003年6月入我院.     

肾上腺节细胞神经瘤3例报告并文献复习

目的:探讨肾上腺节细胞神经瘤的临床症状、影像及病理表现、治疗方法.方法:回顾性分析2012年至今收治的3例肾上腺节细胞神经瘤患者的临床病例并复习相关文献.结果:3例肾上腺节细胞神经瘤患者无明显临床症状,CT平扫低密度、增强扫描无明显强化,经腹腔镜手术切除肿瘤,术后镜下病理见瘤组织内散在神经节细胞分布,免疫组化:S100(+).结论:肾上腺节细胞神经瘤是一种罕见的良性肿瘤,大多无明显临床症状.诊断主要依赖CT,术后病理及免疫组化确诊.腹腔镜手术为目前首选治疗方法,大多数预后良好.     

椎管内节细胞神经瘤12例临床病理学分析

目的:探讨椎管内节细胞神经瘤的临床与病理特征.方法:收集12例椎管内节细胞神经瘤的病例,以1例发生于纵隔的节细胞神经瘤为对照,进行临床、症状、体征、影像学特点分析;标本经常规检查及NF,S-100,MBP,PMP22,Ki-67,SMA,GFAP免疫组织化学染色,对其病理学和临床特点进行分析.结果:12例椎管内节细胞神经瘤发生于30～40岁.女性较多发.部位均位于颈髓节段,临床上多以根性疼痛及脊髓压迫症状为主.形态学上可见成熟或未成熟的肿瘤性节细胞散在或成束穿插于肿瘤性Schwann细胞间质中,肿瘤中可见正常的神经纤维呈束状排列.免疫组化:NF(节细胞+),S-100(含神经鞘瘤样结构的肿瘤成分+,高于含神经纤维瘤样结构者;另外节细胞周围的卫星细胞+).MBP(成熟的神经纤维+),PMP22(弥漫+),Ki-67(含神经鞘瘤结构的区域约3%,其余区域0～1%).SMA(血管内皮无明显增生).GFAP结果不恒定.结论:发生在椎管内的节细胞神经瘤较罕见,肿瘤本身为良性(WHO I级).常引起根性疼痛.由于发生部位为椎管内甚至伴行神经根长出椎间孔,因此在形态学上要与发生在椎间孔区的粘连了神经根的神经鞘瘤或神经纤维瘤相鉴别.手术切除的完全与否对预后影响大.     

Late occurrence of malignancy in a ganglioneuroma 19 years following radiation therapy to a neuroblastoma

Neuroblastoma is a common solid tumor of childhood. Maturation to a ganglioneuroma or regression has been reported to occur spontaneously or following minimal treatment. Malignant degeneration of such a ganglioneuroma is extremely rare. We present a report of a malignant tumor arising in a ganglioneuroma 19 years following abdominal radiation therapy for neuroblastoma.     

Ultrasonographic diagnosis in abdominal tuberculosis

Sonographic findings were retrospectively analysed in 39 patients with proven abdominal tuberculosis (TB). The patients were treated over 15 years at a major teaching hospital, Mubarak Al-Kabber Hospital, in Kuwait. The findings included clear or complex ascites with fine strands, loculations and debris. The other findings were lymphadenopathy, bowel wall thickening, omental mass, focal lesions in the liver and spleen and psoas abscess. The sonographic findings in abdominal TB are not specific but may give valuable information to prevent unnecessary laparotomy.     

3-D CT成像技术在小儿腹膜后神经母细胞瘤治疗中的应用

目的：探讨CT三维成像（3-DCT）在小儿腹膜后神经母细胞瘤手术中的应用。方法：对12例小儿神经母细胞瘤应用CT机工作软件对图像进行多平面重建、容积再现及透明重建，根据情况制订手术方案。结果：完整切除术5例；大部切除术3例；部分切除2例；明确诊断，放弃手术治疗2例。1例发生并发症是由于术中肿瘤包绕腔静脉，术前腔静脉腹段显示不清。所有病例经3-D CT成像后，显著提高了对肿瘤与周围脏器的解剖关系的了解，血供及供应血管等信息可清晰显示。结论：CT三维成像可以立体直观地显示小儿神经母细胞瘤的形态轮廓及与邻近结构的关系，帮助医生了解病理解剖、体积测定、制订手术计划、最大限度地降低术中损伤。     

ACY-1在儿童神经母细胞瘤组织和细胞中的表达及其意义

目的:探究氨基酰化酶-1(aminoacylase-1,ACY-1)在儿童神经母细胞瘤组织和细胞中的表达及其意义.方法:采用qRT-PCR和Western blot技术对儿童神经母细胞瘤患者的肿瘤组织和癌旁组织中ACY-1的表达进行检测.然后对人神经母细胞瘤细胞(SH-SY5Y细胞株)进行体外细胞功能实验,运用重组腺病...     

儿童纵隔神经母细胞瘤临床特征分析

  目的  探讨纵隔神经母细胞瘤临床特征。  方法  收集2008年3月至2012年9月中国医科大学附属第四医院儿科收治的神经母细胞瘤110例, 其中26例肿瘤位于纵隔, 其他部位84例。将纵隔神经母细胞瘤的临床表现、肿瘤标志物、生物学预后因素与其他部位对比。  结果  纵隔组平均年龄25.5个月, 与其他部位组相近。初诊时纵膈组88.5%有症状, 高于其他部位组60.7%（P < 0.05）; 早期病例（Ⅰ、Ⅱ期）34.6%, 高于其他部位组8.3%（P < 0.05）; 血清NSE>100 ng/L者达21.4%, 低于其他部位组86.1%（P < 0.001）。纵隔组N-myc基因拷贝数均 < 10, 其他部位组N-myc基因拷贝数>10者为23.1%（P < 0.05）。纵隔组4年总生存率为80.0%, 其中局限性肿瘤（Ⅰ、Ⅱ、Ⅲ期）达100%, 分别高于其他部位组44.0%、82.0%。  结论  多数纵隔神经母细胞瘤表现出临床分期早（Ⅰ、Ⅱ期）和良好的生物学预后因素, 这些特征可能与纵隔神经母细胞瘤良好的预后相关。      

小儿原发性胃肠道非霍奇金淋巴瘤5例报告并文献复习

目的：对5例原发性胃肠道非霍奇金淋巴瘤（NHL）引发急腹症的临床资料进行分析,讨论其临床病理特点及手术效果。方法：收集2001年1月-2006年5月我院收治的5例原发性胃肠道非霍奇金淋巴瘤引发急腹症的病例,结合文献对其临床表现、诊断、治疗及预后等进行总结分析。结果：5例均为经病理、免疫组化等检查确诊的胃肠道非霍奇金淋巴瘤,其中肠梗阻2例,肠套叠3例（其中回结型2例,节结型1例）;病理免疫类型：5例均为B细胞性非霍奇金氏淋巴瘤。5例均急诊行手术治疗,1例术中死亡,术后辅以联合化疗,方案为COP或CHOP。4例可随访,随访率80%。结论：小儿胃肠道非霍奇金淋巴瘤以B细胞性最多见,本病术前诊断率较低,往往以急腹症就诊。完整手术切除是NHL的治疗关键,术后辅以化（放）疗及综合性治疗措施,可以提高患者生存期。     

Dickkopf-3 expression is a marker for neuroblastic tumor maturation and is down-regulated by MYCN

Neuroblastoma and ganglioneuroma are neuroblastic tumors originating from the developing sympathetic peripheral nervous system. Ganglioneuromas are usually benign, while neuroblastomas have a variable prognosis and include very aggressive tumors. Examples exist of neuroblastomas regressing to ganglioneuromas and ganglioneuromas progressing to neuroblastomas. Little is known of the molecular differences between the tumor types. Here we report that Dickkopf-3 (DKK3), a putative extra cellular inhibitor of the Wnt/beta-catenin pathway, showed a strongly differential expression between neuroblastoma and ganglioneuroma. Microarray analyses of 109 neuroblastic tumors revealed that DKK3 is strongly expressed in ganglioneuroma but only weakly in neuroblastoma. Low DKK3 expression in neuroblastoma correlated with a poor prognosis. The expression of DKK3 in the tumor series and in neuroblastoma cell lines was inversely correlated with the expression of the MYCN oncogene. Analysis of 2 neuroblastoma cell lines with inducible activity of MYCN showed that DKK3 is down-regulated by MYCN. We subsequently generated cell lines with inducible expression of DKK3, which revealed an inhibitory effect of DKK3 on proliferation. High DKK3 expression in the benign ganglioneuromas and down-regulation of DKK3 by MYCN in neuroblastoma might contribute to the strongly different clinical behavior of both neuroblastic tumor types.     

Neuroblastoma among children in Southern and Eastern European cancer registries: Variations in incidence and temporal trends compared to US

Neuroblastoma comprises the most common neoplasm during infancy (first year of life). Our study describes incidence of neuroblastoma in Southern–Eastern Europe (SEE), including – for the first time – the Nationwide Registry for Childhood Hematological Malignancies and Solid Tumors (NARECHEM‐ST)/Greece, compared to the US population, while controlling for human development index (HDI). Age‐adjusted incidence rates (AIR) were calculated for 1,859 childhood (0–14 years) neuroblastoma cases, retrieved from 13 collaborating SEE registries (1990–2016), and were compared to those of SEER/US (N = 3,166; 1990–2012); temporal trends were assessed using Poisson regression and Joinpoint analyses. The overall AIR was significantly lower in SEE (10.1/million) compared to SEER (11.7 per million); the difference was maximum during infancy (43.7 vs. 53.3 per million, respectively), when approximately one‐third of cases were diagnosed. Incidence rates of neuroblastoma at ages <1 and 1–4 years were positively associated with HDI, whereas lower median age at diagnosis was correlated with higher overall AIR. Distribution of primary site and histology was similar in SEE and SEER. Neuroblastoma was slightly more common among males compared to females (male‐to‐female ratio: 1.1), mainly among SEE infants. Incidence trends decreased in infants in Slovenia, Cyprus and SEER and increased in Ukraine and Belarus. The lower incidence in SEE compared to SEER, especially in infants living in low HDI countries possibly indicates a lower level of overdiagnosis in SEE. Hence, increases in incidence rates in infancy noted in some subpopulations should be carefully monitored to avoid the unnecessary costs health impacts of tumors that could potentially spontaneously regress.