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。     

恶性外周神经鞘瘤52例临床病理分析

目的观察恶性外周神经鞘瘤的临床病理学特征、诊断及鉴别诊断。方法回顾性分析52例恶性外周神经鞘瘤的临床病理学及免疫表型特征并复习相关文献。结果 52例患者中,男女发病率为1∶1,年龄4~71岁,头颈部18例(35%),四肢12例(23%),躯干9例(17%),深部组织8例(15%),椎管内4例(8%),生殖道1例(2%)。镜下肿瘤组织呈束状或漩涡状排列,瘤细胞短纺锤形、卵圆形、梭形,核分裂象易见。免疫表型:瘤细胞局灶表达S-100蛋白,Ki-67增殖指数10%~70%。结论恶性外周神经鞘瘤罕见,侵袭性高,预后差,其组织形态复杂多样,需与滑膜肉瘤、纤维肉瘤、血管外皮瘤、富于细胞性神经鞘瘤、纤维型脑膜瘤以及平滑肌肉瘤等鉴别。     

植物凝集素对5种软组织肉瘤的鉴别诊断价值

本文采用6种植物凝集素(RCA,WGA、ConA、PNA、DBA及UEA),对5种软组织肉瘤及二种正常软组织进行亲合细胞化学研究,以探讨它们对几种软组织肿瘤的诊断及鉴别诊断意义。材料取自本院附一院病理科及广州军区169医院病理科经临床及病理确诊的石蜡存档组织,其中包括横纹肌肉瘤32例,脂肪肉瘤8例,恶性神经鞘瘤13例,恶纤组10例,滑膜肉瘤8例;正常横纹肌组织及脂肪组织各4     

尿路膀胱软组织肿瘤（Ⅱ）：恶性肿瘤

大多数原发膀胱癌为移形细胞（尿路上皮）癌，鳞状细胞癌、原发腺癌或小细胞癌均较少碰到，其它膀胱癌均非常少见。在上一期杂志中，作者提出了“尿路膀胱的软组织肿瘤第1部分：肌纤维母细胞增生、良性肿瘤和具有不确定恶性潜能的肿瘤”，此两部分综述的第Ⅱ部分描述了不同类型的膀胱恶性间质病变，包括：平滑肌肉瘤、横纹肌肉瘤、血管肉瘤、恶性纤维组织细胞瘤（未分化肉瘤）、原始神经外胚叶肿瘤、恶性外周神经鞘瘤、血管外皮细胞瘤和腺泡状软组织肉瘤，并描述了这些病变的临床表现、形态学特点和免疫组化特征。     

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…     

恶性多形性细胞肿瘤的免疫组织化学与鉴别诊断

报道恶性多形性细胞肿瘤５０例，其中多形性横纹肌肉瘤９例，恶性纤维组织细胞瘤１８例，多形性平滑肌肉瘤１２例，多形性脂肪肉瘤５例，恶性神经鞘瘤４例，恶性蝾螈瘤２例。按组织病理形态进行ＰＡＳ、脂肪染色、Ｍａｓｓｏｎ、ＰＴＡＨ等特殊染色和免疫组织化学Ｄｅｓ、ＨＨＦ－３５、Ｍｙｏｇｌｏｂｉｎ、α－ＡＴ、Ｌｙｓ、Ｓ－１００、ＮＳＥ、Ｃ－Ｋｅｒａｔｉｎ等染色。着重讨论各类肿瘤的病理形态特征和应用特殊染色、免疫酶标在上述肿瘤诊断中的价值和正确应用、合理评价等问题。强调正确的免疫组化诊断必须以病理形态为基础，选择标准化系列抗血清及正确的病理诊断必须结合临床及其它检查。     

特殊类型周围神经肿瘤

目的：介绍特殊类型周围神经肿瘤的病理诊断。方法：收集外检和会诊中遇到的各种类型周围神经肿瘤进行回顾性分析，并作必要的免疫组化标记和鉴别诊断，报道９例罕见的特殊类型周围神经肿瘤。结果：９例中：上皮样恶性周围神经鞘膜瘤，恶性嵘螈瘤，黑色素性神经鞘瘤，婴儿黑色素性神经外胚叶瘤腺性神经纤维瘤，神经鞘粘液瘤，节细胞性神经母细胞瘤，恶性颗粒细胞瘤和透明细胞肉瘤各１例，结论：特殊类型周围神经肿瘤的形成与原始神经     

恶性小圆细胞肿瘤免疫组织化学及病理组织学分析

本文分析57例恶性小凶细胞肿瘤(MSRCT)免疫组化表型及病理形态。结果表明:兔抗鸡结蛋白及(或)兔抗人肌红蛋白可作为横纹肌肉瘤的标志;神经母细胞瘤、嗅神经母细胞瘤及原始外周神经外胚层瘤的免疫组化特征为NSE阳性,而Vim阴性;抗NSE抗血清特异性差,其他MSRCT亦可与之反应。尤文肉瘤Vim阳性,有些病例NSE阳性。未分化滑膜肉瘤Vim阳性;部分病例Ker及(或)EMA阳性,可有假阴性。LCA是恶性淋巴瘤特异性标记。α_1-ACT对MSRCT鉴别诊断无价值。     

腹腔软组织平滑肌肿瘤临床病理分析

目的探讨腹腔软组织平滑肌肿瘤临床病理、免疫组化、超微结构特点及鉴别诊断。方法用desmin、α-SMA、MSA以及CD117、CD34为主的一组抗体对腹腔软组织原诊断为平滑肌瘤、平滑肌母细胞瘤及平滑肌肉瘤等病例进行研究,获得34例腹腔、系膜及腹膜后平滑肌肿瘤,其中2例行超微结构观察。结果男性8例,女性26例,年龄26~77岁,平均47·6岁,中位年龄48岁,其中腹膜后28例,肠系膜4例,腹腔2例,肿瘤直径5~18cm,平均9·86cm。梭形细胞型32例,其中1例伴有反应性破骨样巨细胞,1例有高度恶性软组织肉瘤区域(即去分化平滑肌肉瘤),上皮型和混合型各1例。此组抗体表达分别为desmin(21/22)、α-SMA(21/21)、MSA(21/21)、CD117(1/21)、CD34(1/21)、S-100蛋白(3/21)、PGP9·5(2/21)。超微结构可见基板、质膜下连续的微饮泡、胞质内丰富的密体和密斑,而细胞器较少。其中4例检测c-kit基因11号外显子序列,均为野生型。平滑肌瘤4例,其中2例分别健在5年和10年;交界性1例;平滑肌肉瘤29例,获访13例,11例(85·0%)在3年内复发、转移或死亡,1例5年后死亡,仅1例无瘤生存10年。结论腹腔软组织平滑肌肿瘤主要发生于腹膜后,女性多见,以恶性居多,肿瘤性坏死、核分裂象≥4/50HPF及细胞明显异型性对判断恶性有重要参考价值。需与相同部位的胃肠道外间质瘤、恶性神经鞘膜瘤等鉴别。     

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。     

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.     

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.     

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.     

What will studies of Fulani individuals naturally exposed to malaria teach us about protective immunity to malaria?

There are an estimated over 200 million yearly cases of malaria worldwide. Despite concerted international effort to combat the disease, it still causes approximately half a million deaths every year, the majority of which are young children with Plasmodium falciparum infection in sub-Saharan Africa. Successes are largely attributed to malaria prevention strategies, such as insecticide-treated mosquito nets and indoor spraying, as well as improved access to existing treatments. One important hurdle to new approaches for the treatment and prevention of malaria is our limited understanding of the biology of Plasmodium infection and its complex interaction with the immune system of its human host. Therefore, the elimination of malaria in Africa not only relies on existing tools to reduce malaria burden, but also requires fundamental research to develop innovative approaches. Here, we summarize our discoveries from investigations of ethnic groups of West Africa who have different susceptibility to malaria.     

'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.