上皮样血管内皮瘤的临床病理分析

目的：探讨上皮样（组织细胞样）血管内皮瘤的临床病理学特点及其意义。方法：对9例上皮样血管内皮瘤进行光镜和免疫组织化学SP法检测,1例作电镜观察,结果：年龄16－47岁,平均32岁,男女性别差异无显著性意义。部位：头面部4例,上肢3例,下肢2例,形态特征：瘤细胞具有上皮样或组织细胞样的形态;瘤细胞圆形或多角开,三五成群呈小巢状,索状,不规则状排列,分布于粘液间质中;间质可显著或少量粘液样变或玻璃样变,瘤细胞内含有原始血管腔,核分裂角,多形性及坏死少见,部分病例伴有梭形细胞血管内皮瘤改变,1例见破骨细胞样的多核巨细胞,免疫组织化学检测7例,7例波形蛋白均阳性,5例第八因子相关抗原,CD31,CD34阳性,2例细胞角蛋白弱阳性,1例CD68和a1-抗胰蛋白酶阳性,电镜观察1例见胞质内含丰富的微丝和少许的W－P小体,6例随访－8年,3例在原发部位有1或2次复发,但未见有转移,结论：上皮样血管内皮瘤是一种低度恶性的软组织肉瘤,其病因学尚不清楚,熟悉其形态特征对避免误诊为其他类似病变具具有重要意义。     

肝上皮样血管内皮瘤2例临床病理分析

目的 探讨肝上皮样血管内皮瘤(hepatic epithelioid haemangioendothelioma,HEH)的临床病理特点及诊断和鉴别诊断.方法 回顾分析2例HEH的临床病理和免疫组化特征.结果 2例均为女性,年龄分别为51、55岁.临床均表现为右上腹疼痛不适,均为单发,位于肝右叶,大小分别为5 cm×4 cm×3 cm、5 cm×4 cm×4 cm.例2伴肝门淋巴结转移.光镜下肿瘤细胞呈上皮样或梭形,伴胞质内管腔,腔内可见红细胞,间质黏液透明变,瘤细胞浸润周围肝组织,例1伴钙化,脉管腔内见乳头状生长瘤细胞,似瘤栓样结构;例2淋巴结转移瘤细胞异型明显,伴局灶坏死,除淋巴结转移瘤细胞仅CD31阳性外,2例瘤组织CD31、CD34、FⅧRAg因子均为阳性,其中例2表达部分上皮标记如CK8和LCK.结论 HEH少见,易误诊为转移癌.在遇到胞质内管腔含红细胞、间质黏液玻璃样变性时应考虑HEH.     

淋巴结原发上皮样血管内皮瘤1例及文献复习

目的 探讨淋巴结原发上皮样血管内皮瘤的诊断及鉴别诊断。方法 1例淋巴结原发上皮样血管内皮瘤进行HE切片、组织化学、免疫组化等观察，并复习文献进行讨论。结果 光镜下肿瘤细胞呈多角形或类圆形上皮样，无明显核分裂象，组织结构多种，呈单个细胞条索、实性巢状及管腔样结构排列，瘤细胞形成小的细胞内管腔，见胞质内空泡，并见红细胞，此特征为细胞原始管腔结构。玻璃样间质。网状纤维染色显示血管腔隙结构。肿瘤细胞表达Vim、CD31、FⅧRAg，少数细胞表达CD34。结论 淋巴结原发上皮样血管内皮瘤是极少见的低度恶性血管性肿瘤，可出现多种组织结构，有一定的特征性。应与转移性癌、转移性恶性黑色素瘤、上皮样血管肉瘤、上皮样血管瘤、上皮样肉瘤及硬化性上皮样纤维肉瘤等鉴别。     

骨卡波西样血管内皮瘤临床病理特点

目的 探讨骨卡波西样血管内皮瘤（KHE）的临床病理特点、鉴别诊断及生物学特征。方法 对2例发生于儿童长骨的KHE进行组织病理学、免疫表型和电镜观察,结合临床资料进行分析并复习相关文献。结果 2例患儿临床以肢体持续性隐痛为主,1例伴局部皮温升高,病程较长（发病1年余就诊）,影像学示局限性骨密度减低或蜂窝状骨质破坏。临床未见血小板减少及低纤维蛋白血症（Kabasach-Merritt syndrome,卡梅综合征）。光镜下肿瘤呈多结节状或小叶状分布,浸润性生长,见特征性的肾小球样结构。部分瘤细胞梭形,呈束状排列并形成裂隙状血管腔,肾小球样结构区瘤细胞多为上皮样,见空泡形成及胞质内脂褐素沉积,部分区域见明显的毛细血管瘤样结构。免疫表型：瘤细胞呈CD31、CD34和Fli-1强阳性表达、SMA灶性阳性,GLUT1、CKpan和FⅧRAg均为阴性,Ki-67有少量散在阳性表达。结论 KHE是一种罕见的好发于儿童的潜在恶性肿瘤,诊断主要靠病理组织学及免疫组化确诊,须与幼年性毛细血管瘤、卡波西肉瘤、丛状血管瘤、梭形细胞血管内皮瘤等鉴别,病变切除干净是治疗KHE的最佳手段。     

中枢神经系统中具有脊索样特征肿瘤的临床病理分析

目的探讨中枢神经系统中具有脊索样特征肿瘤的临床病理特征、诊断及鉴别诊断。方法回顾性分析81例脊索瘤、9例脊索样脑膜瘤、4例脊索样胶质瘤及2例上皮样血管内皮瘤的临床资料、组织学特征,并复习相关文献。结果脊索样特征肿瘤镜下瘤细胞呈条索状或巢状分布于黏液样间质中。脊索瘤好发于人体中轴,多见于颅底及骶尾部,镜下呈分叶状结构,可见胞质含空泡的液滴细胞及星芒状细胞。免疫表型:CK、EMA、vimentin均阳性,绝大多数S-100阳性,GFAP均阴性,PAS染色阳性。脊索样脑膜瘤好发于幕上,与脑膜组织关系密切,镜下可见脊索样成分与典型脑膜瘤结构混杂,间质炎性细胞浸润,可见淋巴滤泡。免疫表型:vimentin、EMA、PR均阳性。脊索样胶质瘤好发于第三脑室,女性多见,镜下可见部分瘤细胞胶质细胞分化,胶质原纤维突起,核分裂象罕见,间质大量淋巴细胞、浆细胞浸润但不形成淋巴滤泡,可见Russell小体,瘤周组织星形细胞反应性增生。免疫表型:GFAP、vimentin均阳性,CK阴性。上皮样血管内皮瘤具有上皮样细胞和血管内皮细胞的组织学特征,镜下见卵圆形或多角形瘤细胞形成原始血管腔,内含红细胞,肿瘤细胞轻度异型性,电镜下可见W-P小体。免疫表型:CD31、CD34、vimentin均阳性。结论脊索瘤、脊索样脑膜瘤、脊索样胶质瘤及上皮样血管内皮瘤具有共同的脊索样特征,发生于中枢神经系统时,其鉴别诊断主要依据临床、形态学特征及免疫表型综合分析。     

MAML2重排阴性腮腺Warthin瘤样黏液表皮样癌1例并文献复习

目的 探讨原发于腮腺MAML2重排阴性的Warthin瘤样黏液表皮样癌(Warthin-like mucoepidermoid carcinoma, WT-MEC)的临床病理特征、诊断和鉴别诊断。方法 收集1例原发于腮腺的WT-MEC临床资料,采用免疫组化EnVision两步法染色和FISH检测,分析其临床病理特征和预后的关系,并复习相关文献。结果 眼观：肿瘤呈实性,多小叶状,边界大部分清楚,切面灰白、灰黄色,局部呈蜂窝状伴点灶性出血。镜检：大量呈多层排列的嗜酸性上皮、部分透明细胞样上皮,丰富的非肿瘤性淋巴细胞间质,可见淋巴滤泡。嗜酸性上皮细胞呈圆形或卵圆形、泡状核和嗜酸性胞质,核仁清楚,上皮内可见异型黏液细胞,还可见灶性分布的中间细胞和表皮样细胞。免疫表型：上皮细胞CK35βH11阳性、CD117部分阳性,表皮样细胞和中间细胞CK5/6、p63局灶阳性,S-100阴性,AB染色黏液呈阳性,Ki67增殖指数<5%。FISH检测：MAML2重排阴性。结论 MAML2基因易位是诊断WT-MEC的金标准,当MAML2重排阴性不能除外时,需根据组织病理学特征和免疫组化综合分析。     

肺上皮样血管内皮瘤临床病理观察

目的 探讨肺上皮样血管内皮瘤的临床病理特点。方法 4例肺上皮样血管内皮瘤,3例女性,1例男性,年龄28～40岁,无自觉症状或有轻度咳嗽、气短。肺活检或手术切除标本经甲醛固定,石蜡包埋,常规HE及免疫组织化学(Envision法)染色。所用抗体包括CD31、CD34、细胞角蛋白(AEl／AE3)、TTF-1、波形蛋白和上皮膜抗原。结果 本组肺上皮样血管内皮瘤病例女性多于男性,胸部CT显示双肺多发弥漫性小结节影。病理形态特点为结节周边上皮样肿瘤细胞呈花冠状充填于肺泡腔,病变中心为黏液透明样变间质,肺泡壁结构保留,肿瘤细胞胞质内有空泡形成,空泡内偶见红细胞,肿瘤细胞异型性不明显,核分裂和坏死均少见,免疫组织化学染色示CD31、CD34阳性,AE1／AE3偶见灶状阳性,其他抗体呈阴性。结论 肺上皮样血管内皮瘤是一种具有独特临床病理特点的低度恶性血管来源肿瘤。     

骨软化或佝偻病相关的间叶组织肿瘤临床病理分析

目的 探讨骨软化或佝偻病相关的间叶组织肿瘤的临床病理特点。方法 回顾分析10例患者的临床资料,观察10例骨软化相关的肿瘤组织的形态和免疫表型[免疫组织化学SP法染色,所用抗体包括波形蛋白、S-100、平滑肌肌动蛋白（SMA）、结蛋白、CD34、AE1／AE3、Ki-67、HMB45]。结果 患者男性6例,女性4例,年龄范围28～69岁（平均45．6岁）;患者均有2～27年（平均9．6年）骨痛、关节痛和活动困难的病史,检查发现低血磷、高尿磷;肿瘤最大径1～7cm不等（平均3．5cm）;瘤组织为间叶组织来源（仅2例颌骨病变中见不明显的条索状上皮）,可见多少不等的梭形纤维母细胞样细胞、脂肪细胞、软骨样细胞、黏液样细胞等,瘤组织富于血管,8例病变中有少见的絮状或不规则砂砾样钙盐沉积,2例发生于软组织的肿瘤周边见骨壳形成。3例组织中可见非尿酸盐结晶：9例细胞分裂象少见,1例核分裂象多见并且异型性明显;瘤细胞波形蛋白阳性,5例SMA部分阳性,3例CD34部分瘤细胞阳性,结蛋白、S-100、AE1／AE3均阴性,Ki-67指数（8例≤4％,仅1例为30％）;AB／PAS染色：8例肿瘤黏液基质和血管周围黏液样变呈AB染色阳性。结论 骨软化相关的肿瘤多为良性或低度恶性的间叶组织肿瘤,因组织学具有多样性而易误诊,掌握其共性特征并结合临床资料方能正确诊断。     

毛细胞黏液样星形细胞瘤3例报道并文献复习

目的 探讨毛细胞黏液样星形细胞瘤(pilomyxoid astrocytoma,PMA)的临床病理特点及鉴别诊断.方法 报道3例PMA患者的临床资料,并对其进行组织学观察及免疫组化(EnVision-plus法)标记,其中1例行电镜观察.结果 本组PMA均为女性,年龄分别为13岁、4岁和17岁.肿瘤分别位于右侧丘脑、右侧颞叶基底节区及下丘脑/视交叉区.镜下见双极性的梭形瘤细胞呈星网状散在分布于黏液背景中,部分区域呈血管中心性生长,瘤细胞异型性小,核分裂象偶见,间质血管增生明显.1例见大片出血,1例见少许嗜酸性颗粒小体及小灶坏死,3例PMA均未见双相性结构及Rosenthal纤维.免疫组化标记示瘤细胞表达GFAP、vimentin、S-100及Olig-2,而CKpan、EMA、NF、CgA、Neu-N、p53均呈阴性表达,Syn有2例局灶阳性,Ki-67阳性率为1%～2%.仅有的1例电镜表现除具有伸长细胞特征外,胞质内还可见神经内分泌样颗粒.结论 PMA为毛细胞星形细胞瘤(pilocytic astrocytoma,PA)的亚型,与经典PA相比,具有独特的组织学特征,并具有更强的侵袭性,故应加强对其的认识.     

脑上皮样血管内皮瘤1例及文献复习

目的：探讨脑上皮样血管内皮瘤（ＥＨＥ）的诊断及鉴别诊断。方法：通过ＨＥ、免疫组化及电镜观察１例脑原发性多灶性上皮样血管内皮瘤、并复习文献。结果：肿瘤由排列呈巢状、索状的上皮样细胞组成,胞浆丰富、嗜酸性;内含明显空泡呈“印戒”状,有的空泡内可见红血球,间质含粘液样基质。免疫组化染色显示ＦⅧ、ＣＤ３４、ＣＤ３１、ＵＥＡ－１和ｖｉｍｅｎｔｉｎ阳性;而ＣＫ、ＣＥＡ、ＥＭＡ、ＧＦＡＰ、Ｓ－１００蛋白及Ｍａｃ     

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.     

The dominant diseases of modernized societies as omega-3 essential fatty acid deficiency syndrome: Substrate beriberi

About 1900, modern food selection and processing caused widespread $\text{epidemics}$ of the B vitamin deficiency diseases of beriberi and pellagra which, for genetic reasons, often expressed as different diseases ranging from bowel and heart disease to dermatoses and psychoses. But the B vitamins merely help convert essential fatty acids (EFA) into the prostaglandin (PG) tissue regulators and it now turns out that, through hydrogenation, milling and selection of w3-poor southern foods, we have also been systematically depleting, by as much as 90%, a newly discovered trace Nordic EFA (w3) of special importance to primates and sole precursor of the PG3(4) series, even as a concurrent fiber deficiency increases body demand for EFA. Since substrate EFA is processed by many B vitamin catalysts, an EFA deficiency will mimic a $\text{panh}$ypovitaminosis B, i.e., a mixture of $\text{substrate}$ beriberi and $\text{substrate}$ pellagra resembling vitamin beriberi and pellagra but exhibiting as even more diverse $\text{endemic}$ disease. This would consitute a second stage of the Modern Malnutrition and explain why some workers now hold the dominant diseases of modermized societies to be new, nutritionally based, pellagraform yet lipid-related and to range, once again, from heart disease to psychosis. It is an assumption that our dominant diseases are unrelated to each other or are merely revealed by our diagnostic acumen and therapeutic success; and that hydrogenating millions of tons of food oils annually, to destroy the rancidity producing w3-EFA, is safe for $\text{primates}$. Extensive beriberiform disease is reported here in 32 typical cases taken from medical practice which responds strikingly to linseed oil supplements (60% w3-EFA) in confirmation of identical results in Capuchins.     

'Very sore nights and days': the child's experience of illness in early modern England, c.1580-1720

Sick children were ubiquitous in early modern England, and yet they have received very little attention from historians. Taking the elusive perspective of the child, this article explores the physical, emotional, and spiritual experience of illness in England between approximately 1580 and 1720. What was it like being ill and suffering pain? How did the young respond emotionally to the anticipation of death? It is argued that children’s experiences were characterised by profound ambivalence: illness could be terrifying and distressing, but also a source of emotional and spiritual fulfilment and joy. This interpretation challenges the common assumption amongst medical historians that the experiences of early modern patients were utterly miserable. It also sheds light on children’s emotional feelings for their parents, a subject often overlooked in the historiography of childhood. The primary sources used in this article include diaries, autobiographies, letters, the biographies of pious children, printed possession cases, doctors’ casebooks, and theological treatises concerning the afterlife.     

Guidelines for the Validation and Use of Immunoassays for Determination of Introduced Proteins in Biotechnology Enhanced Crops and Derived Food Ingredients

Recent advancements in agricultural biotechnology have created a need for analytical techniques to determine introduced proteins in crops enhanced through modern biotechnology techniques. These proteins are expressed in plant tissues and may be present in food ingredients. Immunoassays are ideally suited for protein detection and may be used as both quantitative and threshold methods. Microplate ELISA and lateral flow devices are two of the most commonly used immunoassay formats for agricultural biotechnology applications. This paper provides general background information and a discussion of criteria for the validation and application of immunochemical methods to the analysis of proteins introduced into plants and food ingredients using biotechnology methods. It is the result of a collaborative effort of members of the Analytical Environmental Immunochemical Consortium. This collaborative effort represents the combined expertise of several organizations to reach consensus on establishing guidelines for the validation and use of immunoassays. Further, the paper offers developers and users a consistent approach to adopting the technology as well as aid in producing accurate and meaningful results.     

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.     

Ultracryotomy: development and application (with examples from the yeast cytology) (author's transl)

The preparation steps usually necessary for obtaining ultrathin frozen sections of biological material (chemical prefixation, enclosing, cryoprotective treatment, freezing, sectioning, and post-staining the sections for transmission electron microscopy) are submitted to a critical analysis. The application of cryo-ultramicrotomy, in particularly for cytochemical purposes, is reviewed. Fundamental considerations of chemical prefixation and poststaining are supported by examples from yeast cytology. Furthermore, the efficiency of the cryo-ultramicrotomy (electron optical resolution of ultrastructural details) is demonstrated on yeast cells and protoplasts.     

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.