恶性外周神经鞘膜瘤和细胞性神经鞘瘤的形态学和免疫表型特征

细胞性神经鞘瘤较为少见,是一种公认的良性外周神经鞘膜肿瘤,可被误诊为恶性外周神经鞘膜瘤。为制定一个细胞性神经鞘瘤诊断共识标准,作者回顾性分析来自两家医学机构的115例恶性外周神经鞘膜瘤和26例细胞性神经鞘瘤的临床病理学特征。临床资料从电子医疗记录档案中获得,并对形态学特征、最高核分裂计数、Ki-67增殖指数和免疫表型(SOX10、SOX2、p75NTR、p16、p53、EGFR和NF)进行评估。结果提示以下几个特征可以区分细胞性神经鞘瘤和恶性外周神经鞘膜瘤：(1)与恶性外周神经鞘膜瘤患者相比,细胞性神经鞘瘤不发生远处转移或者死于该病。更具特征性的是5年无进展生存率细胞性神经鞘瘤为100%,恶性外周神经鞘膜瘤仅18%,5年疾病特异性生存率分别为100%和32%;(2)神经鞘漩涡：瘤周包膜,包膜下丰富的淋巴细胞、巨噬细胞的浸润,和缺乏束状生长模式等支持细胞性神经鞘瘤的诊断,而出现血管周围细胞密集,肿瘤突入血管腔内和坏死则支持恶性外周神经鞘膜瘤的诊断;(3)在恶性外周神经鞘瘤中SOX10、NF及p16表达完全缺失,而表达EGFR( P值均<0．001)。 p75NTR在80%的恶性外周神经鞘膜瘤中表达,在细胞性神经鞘瘤阳性率为31%( P<0．001);(4) Ki-67增殖指数在20%以上者高度提示恶性外周神经鞘膜瘤的可能,其敏感性为87%,特异性为96%。总之,结合组织病理学及免疫表型特征可以提供一些有用的具有较高敏感性和特异性的标准,以鉴别恶性外周神经鞘膜瘤和细胞性神经鞘瘤。     

丛状神经鞘瘤2例临床病理分析

目的探讨丛状神经鞘瘤的临床病理学特征、诊断及鉴别诊断。方法观察2例丛状神经鞘瘤的临床表现、组织学形态及免疫表型并复习相关文献。结果 2例患者中,男女各1例,年龄分别为16岁和31岁,肿瘤均位于躯干皮肤。镜下见肿瘤在皮下呈多结节状分布,结节内瘤组织以细胞致密区(Antoni A)为主,相对缺乏细胞疏松区(Antoni B)。瘤细胞长梭形或波浪状,呈栅栏状、漩涡状排列,可见verocay小体。免疫表型:肿瘤细胞S-100及vimentin均弥漫阳性,GFAP部分阳性,EMA、CD57及Ki-67均阴性。结论丛状神经鞘瘤是一种较少见的良性周围神经鞘膜瘤,需与丛状神经纤维瘤、丛状纤维组织细胞瘤、丛状恶性外周神经鞘膜瘤等相鉴别。     

富于细胞性神经鞘瘤30例临床病理分析

目的探讨富于细胞性神经鞘瘤(cellular schwannoma, CS)的临床病理学特点、免疫表型及鉴别诊断。方法回顾性分析2014～2020年北京积水潭医院诊治的30例CS的临床及影像学特点、病理学特征及免疫表型等,并复习相关文献。结果 30例CS中,女性22例,男性8例,年龄12～65岁,平均44.6岁。发生部位分别为椎管内、肢体及腹腔,其中2例为多发病变。术前病程14天～30年,大多表现为缓慢生长的肿块,可伴感觉障碍,最大径1～20.5 cm,平均5.8 cm。镜下大部分肿瘤边界清楚,厚薄不等的纤维性包膜中可见淋巴细胞浸润,丰富的梭形细胞呈束状、编织状或漩涡状排列,与经典型神经鞘瘤Atoni A区类似,细胞有轻度异型性,可见核分裂象(0～8个/10 HPF),间质内血管壁玻璃样变性。免疫表型:30例S-100、SOX10及H3K27me3均弥漫阳性,12例GFAP阳性(12/30,40%),2例CD34小灶阳性(2/30,6.7%),Ki-67增殖指数1%～20%。28例患者获得随访,随访时间5～62个月,4例局部复发。结论 CS是一种罕见的良性神经源性肿瘤,免疫组化有助于与恶性外周神经鞘瘤及滑膜肉瘤鉴别。多数患者预后良好,复发与病变部位和手术切缘是否阳性直接相关。     

成人经典型腺泡状横纹肌肉瘤1例并文献复习

目的探讨经典型腺泡状横纹肌肉瘤(alveolar rhabdomyosarcoma,ARMS)的临床病理学特征、鉴别诊断、治疗及预后。方法观察1例成人经典型ARMS的临床病理学及免疫表型特征,并复习相关文献。结果肿瘤发生于左肩部,镜下见肿瘤边界不清,浸润周围纤维及脂肪组织,瘤细胞呈片状或巢状排列,部分瘤细胞形成特征性的腺泡状排列结构,腺泡之间可见纤维血管性间隔。肿瘤由未分化的原始间叶性细胞组成,呈圆形、卵圆形或小多边形,核深染,核分裂易见,胞质少。免疫表型:瘤细胞desmin、Myo D1弥漫强阳性,vimentin、Myoglobin、CD56阳性,Syn、Cg A、CD20、CD79a、CD3、CD99、CK(AE1/AE3)、EMA均阴性,Ki-67增殖指数约70%。结论 ARMS是横纹肌肉瘤的一种特殊类型,形态学上以瘤细胞形成腺泡状结构为特征,需与腺泡状软组织肉瘤、骨外尤因肉瘤/外周原始神经外胚层瘤、小细胞神经内分泌癌及恶性淋巴瘤等相鉴别。     

混合型神经鞘肿瘤2例并文献复习

目的探讨混合型神经鞘肿瘤(hybrid nerve sheath tumor,HNST)的临床病理学特征、免疫表型、诊断、鉴别诊断及预后。方法回顾分析2例HNST的临床资料、病理学形态及免疫表型,并进行随访。结果 2例HNST均为女性,平均年龄43岁,肿瘤均发生于皮下,术后随访情况良好。眼观:界限清楚,灰白色,实性质韧肿物。镜检:例1为神经纤维瘤背景中查见典型神经鞘瘤形态结节,例2为梭形细胞肿物,大部分席纹状结构,间杂漩涡状结构。免疫表型:例1 S-100表达强弱不等,例2 S-100阳性与EMA阳性细胞混杂排列。结论 HNST是一种良性外周神经鞘肿瘤,其组织学图像具有双相成分,包括神经鞘瘤和神经束膜瘤、神经纤维瘤和神经束膜瘤、神经纤维瘤和神经鞘瘤。确诊主要依赖病理学形态和免疫表型相结合;肿瘤预后良好。     

骶骨神经源性肿瘤临床病理分析

目的:探讨骶骨神经源性肿瘤的临床病理学特征、诊断及鉴别诊断.方法:收集21例发生在骶骨的神经源性肿瘤,通过光镜观察及免疫组织化学分析其临床、影像学、病理学特征、免疫表型、鉴别诊断及手术预后.结果:21例中女15例,男6例,平均年龄44.9岁.临床上以骶尾部疼痛为主,影像学上表现为骶骨或骶骨及骶前肿块.神经鞘瘤17例,其中经典型神经鞘瘤8例,富于细胞神经鞘瘤9例.神经纤维瘤3例,节细胞神经瘤1例.累及骶骨的肿块,多数有不同程度的骨质破坏.免疫表型:神经鞘瘤均弥漫强阳性表达S-100蛋白,不表达NF.神经纤维瘤和节细胞神经瘤表达NF.富于细胞神经鞘瘤有4例为复发病例,平均复发时间6.5年.经典型神经鞘瘤和神经纤维瘤各有1例为复发病例.结论:骶骨神经源性肿瘤是少见肿瘤,以良性多见.各病理类型及亚型在形态学和生长方式上有一定的差异,故诊断时应明确病理类型及亚型,以供临床随访、治疗.     

肺原发性恶性外周神经鞘瘤的临床病理分析

目的 探讨肺脏原发性恶性外周神经鞘瘤(MPNST)的临床病理学特征及诊断、鉴别诊断要点。方法 对2例肺MPNST进行临床病理学分析及免疫组织化学与超微结构研究。结果 2例光镜下均显示MPNST的形态特点，免疫组织化学显示S-100蛋白、MBP、Vim、NSE肿瘤细胞呈阳性表达，电镜观察1例可见特征性Luse小体。结论 肺原发性MPNST极为罕见，临床诊断较困难。根据其光镜病理形态特征，S-100蛋白、MBP免疫组织化学检测和(或)电镜检查可确诊。结合文献，该肿瘤早期无明显临床症状，一般发现时已属晚期，预后差。病理学上应与肺脏平滑肌肉瘤、纤维肉瘤、单相型滑膜肉瘤、肉瘤样癌鉴别。     

恶性神经鞘瘤免疫组织化学鉴别诊断研究

应用免疫组化ABC法对51例恶性神经鞘瘤和27例原误诊为恶性神经鞘瘤的软组织肿瘤进行免疫组织化学鉴别诊断研究。结果显示恶性神经鞘瘤S—100阳性率达72.5%,平滑肌肉瘤、恶性纤维组织细胞瘤和滑膜肉瘤分别呈Desmin、α_1—ACT或α_1—AT和Keratin阳性。提示恶性神经鞘瘤尤其是无VRD无神经干累及类型与梭形细胞性软组织肉瘤较易混淆,采用多种标记物联合应用是较为实用的方法。     

良、恶性外周神经鞘膜肿瘤新类型和少见亚型的病理诊断

外周神经肿瘤的类型和亚型繁多 ,组织形态多变 ,造成病理诊断、鉴别诊断的困难 ,尤其是恶性外周神经鞘膜瘤的诊断更为困难。本文就近年来有关良、恶性外周神经鞘膜肿瘤的新类型和少见亚型进行了文献复习 ,并简介如下 ,仅供同仁们参考。1　神经鞘瘤及亚型 (ｎｅｕｒｉｌｅｍｏｍａｏｒｓｃｈｗａｎｎｏｍａａｎｄｔｈｅｉｒｖａｒｉａｎｔｓ)神经鞘瘤是最常见的一种良性外周神经鞘膜肿瘤。一般不伴有神经纤维瘤病。罕见恶变 ,有报道发生血管肉瘤者。神经鞘瘤的亚型颇多〔1〕,下面仅就其中的几种重点介绍。1.1　细胞性神经鞘瘤 (ｃｅｌｌｕｌ…     

眼眶软组织肿瘤65例临床病理学分析

目的探讨眼眶软组织肿瘤的病理学分类及一些少见病变的临床病理特点。方法回顾性分析65例眼眶软组织肿瘤的临床病理学特征,并复习相关文献。结果良性肿瘤57例(87.7%),数目位居前4位的依次为海绵状血管瘤37例(56.9%),神经鞘瘤(包括退变性)8例(12.3%),静脉血管瘤5例(7.7%),孤立性纤维性肿瘤2例(3.1%)。恶性肿瘤8例(12.3%),其中胚胎性横纹肌肉瘤5例(7.7%),均为维族患者,上皮样血管内皮瘤、硬化性横纹肌肉瘤和未分化多形性肉瘤各1例。结论眼眶良性软组织肿瘤主要为脉管性肿瘤和神经鞘瘤;恶性主要为胚胎性横纹肌肉瘤,维族发病率高。孤立性纤维性肿瘤、上皮样血管内皮瘤、硬化性横纹肌肉瘤和未分化多形性肉瘤为少见病例,诊断困难。     

Schizophrenia in a North Swedish geographical isolate, 1900–1977. Epidemiology, genetics and biochemistry

Over 200 schizophrenic patients belonging to three major and interrelated pedigree complexes have been investigated over the past 30 years in a North Swedish geographically isolated population, presently numbering about 6,000. An intensive investigation of a number of biochemical correlates and genetic markers in a few selected families belonging to one of the major pedigrees has indicated new strategies for the current research program.
Schizophrenia, as defined operationally, is significantly associated with decreased activities of two enzymes (1) blood platelet monoamine oxidase, (2) plasma dopamine-β-hydroxylase, and (3) with the genetic marker Gc² (group specific antigen). Both enzymes are subject to genetic variation. A positive score for linkage between schizophrenia and low plasma DBH activity has been calculated, but, so far, available data are insufficient for discrimination between linkage and partial contribution of genetically controlled low plasma DBH to the pathogenesis of the disease. Alternatively, both mechanisms could be involved.
As a model for continued research, schizophrenia is explained as based on a double dominant-recessive genotype (Aabb), representing a vulnerability which in about 50 % of cases develops into clinical schizophrenia. It is suggested that the dominant mutation (A) operates on or affects MAO activity, and that the recessive genotype (bb) is instrumental in low variates of DBH activity and very likely such variates within the normal range of physiological variation. Moreover, it is suggested that the combined effects of MAO- and DBH-reduced efficiency on the metabolism of e.g. dopamine could be an essential pathogenic mechanism for the schizophrenic illness which is segregating in this population.     

Renal dysplasia and asplenia in two sibs

A family is reported in which two sibs, one male and the other female, both died within 24 hours of birth with enlarged polycystic kidneys. Postmortem histology in the second child showed gross renal dysplasia. In both children the pancreas was enlarged, nodular and cystic but the liver appeared macroscopically normal. In the second child, histological examination confirmed pancreatic fibrosis with cystic dilation of ducts, but showed portal fibrosis with bile duct proliferation in the liver.
This combination of findings is very reminiscent of those in a girl and her brother reported by Ivemark et al. (1959). The children reported here also showed absence or hypoplasia of the spleen, cardiac anomalies and other features of the Ivemark syndrome (Ivemark 1955), a quite different, usually sporadic, congenital disorder. It is suggested that the children described here have a distinct lethal congenital disorder, probably inherited in an autosomal recessive manner.     

HLA in North Colombia Chimila Amerindians

HLA-A,-B,-C,-DRB1 and -DQB1 alleles have been studied in Chimila Amerindians from Sabana de San Angel (North Colombian Coast) by using high resolution molecular typing. A frequent extended haplotype was found:HLA-A*24:02-B*51:10-C*15:02-BRB1*04:07-DQB1*03:02 (28.7%) which has also been described in Amerinndian Mayos Mexican population (Mexico, California Gulf, Pacific Ocean). Other haplotypes had already been found in Amerindians from Mexico (Pacific and Atlantic Coast), Peru (highlands and Amazon Basin), Bolivia and North USA. A geographic pattern according to HLA allele or haplotype frequencies is lacking in Amerindians, as already known. Also, five new extended haplotypes were found in Chimila Amerindians. Their HLA-A*24:02 high frequencies characteristic is shared with aboriginal populations of Taiwan; also, HLA-C*01:02 high frequencies are found in New Zealand Maoris, New Caledonians and Kimberly Aborigines from Australia. Finally, this study may show a model of evolutionary factors acting and rising one HLA allele frequency (-A*24:02), but not in others that belong to the same or different HLA loci.     

The universal muscular chamber. A basic unit of the genitourinary, cardiovascular, gastro-intestinal, skeletal, cutaneous, respiratory and other systems, endocameral hypertension; and spasm, the key to their idiopathic diseases and arthropathies

Starting with the integument, we see many organs are contractile sacs or multiples thereof, which tubes or bags constitute the major part of the entire body. Recognition of this basic unit and its characteristics sheds new light, individually and collectively, on many disorders previously considered unrelated. Muscular tears and perforations develop in the walls of these chambers, being no way peculiar to those organs, wherein, hydrochloric acid occurs. So, it is not necessary to explain the absence of excessive acid from patients who exhibit holes in the gastric, uterine, aortic, duodenal, rectal, pulmonary, retina, and other walls. Muscle, not acid is the great common factor relating idiopathic disorders in the gastrointestinal tract to each other and to similar diseases in other systems. When the units are linked together, the lesions tend to appear as arthropathies, i.e. at the joints. Rephrasing common-place observations, frees us from conventional, conceptual cul-de-sacs. An observation is only as good as its interpretation, so all possibilities must be considered, otherwise, we will remain blinded by our misconceptions.     

Der Einfluß von Nahrungsmitteln und Rauchen auf die klinisch-chemische Diagnostik von Phäochromozytom,Neuroblastom und Karzinoid-Syndrom

Zusammenfassung Der Einfluß von verschiedenen Nahrungsmitteln auf Methoden zur Bestimmung von Adrenalin (AD), Noradrenalin (NA), Vanillinmandelsäure (VMS), Metanephrinen (MN), Homovanillinsäure (HVS) und 5-Hydroxyindolessigsäure (5-HIE) im 24 h-Harn zur Diagnose des Phäochromozytoms bzw. Karzinoid-Syndroms wurde untersucht. Die in die Untersuchung einbezogenen Nahrungsmittel waren: Tee, Kaffee, Mandeln, Ananas, Käse, Walnüsse, Vanillepudding, Bananen, Tomaten und Milchschokolade. Außerdem wurde der Einfluß des Zigarettenrauchens auf die Bestimmung von AD, NA, VMS und MN untersucht.Walnüsse führten zu einer starken Erhöhung der 5-HIE-Ausscheidung. Bananen erhöhten die Ausscheidung von AD, NA, VMS, MN und 5-HIE. Kaffee und Ananas bewirkten eine geringe Zunahme der MN-Werte. Rauchen von 20–30 Zigaretten/Tag beeinflußte keine der vier Variablen.Wenn die beschriebenen Methoden benutzt werden, sollte lediglich auf den Verzehr von Bananen und Walnüssen vor und während der Harnsammelperioden verzichtet werden, da die oberen Normgrenzen im Harn überschritten werden könnten. Ein Verzicht auf Kaffee und Ananas in normalen Mengen ist nicht erforderlich. Es besteht kein Anlaß, weiterhin die bisherigen umfangreichen Restriktionen der übrigen Nahrungsmittel beizubehalten.     

A comparative study of the effects of dimebon,obsidan, finoptin,and cordaron on the functional state of ischemic focus and size of necrotic zone in experimental myocardial infarction

Dimebon, an antihistamine agent, exerts a moderate antianginal effect, improving the function of ischemic focus in the myocardium and decreasing the necrotic zone in experimental myocardial infarction. Dimebon is less active than obsidan, finoptin (except for the size of the necrotic zone), and cordaron. Translated fromByulleten' Eksperimental'noi Biologii i Meditsiny, Vol. 122, No. 12, pp. 642–644, December, 1996     

Effects of sex steroid hormones on lipid peroxidation and glutathione antioxidant system in rat skin

Effects of estradiol and testosterone on the intensity of lipid peroxidation and contents of glutathione redox system components in the dermis and epidermis of rat skin were studied. Only estradiol induced considerable dose-dependent and tissue-specific biphasic antioxidant effects on the skin. Translated fromByulleten' Eksperimental'noi Biologii i Meditsiny, Vol. 128, No. 12, pp. 663–666, December, 1999     

Adhesion and growth of CaCo2 cells on surface-modified PEEK substrata

A series of surface-functionalized poly(ether ether ketone) (PEEK) films has been prepared by selective wet-chemistry; they are hydroxylated polymer (PEEK-OH) obtained by reduction, aminated polymer (PEEK-[]-NH2) prepared by coupling a diisocyanate reagent to PEEKOH (PEEK-[]-NCO) followed by hydrolysis, and carboxylated and aminocarboxylated polymers (PEEK-[]-GABA and PEEK-Lysine) resulting from the coupling of aminoacids to PEEK-[]-NCO. The aminated and carboxylated substrata promoted the adhesion and growth of CaCo2 cells in the presence of serum. Fibronectin (FN), an extra-cellular matrix protein, has been covalently fixed and/or adsorbed on various PEEK substrata, in the presence or not of a polymeric surfactant (Pluronic F68). The performances of the FN-grafted substrata (PEEK-[]-FN(1) and PEEK-[]-FN(2)) were significantly higher than those of reference substrata simply coated with FN (PEEK-OH(+FN)(1) and (2), PEEK-[]-NH2(+FN)(1) and (2)), considering the adhesion and spreading of CaCo2 cells in the absence of serum. Moreover, the stability of the adherent cells on the FN-adsorbed substrata dramatically depended on the experimental conditions applied during the PEEK coating with FN.