卵巢Brenner肿瘤14例临床病理分析

目的探讨卵巢Brenner肿瘤的临床病理学特征、诊断和鉴别诊断。方法回顾性分析9例良性Brenner瘤、1例交界性Brenner瘤、4例恶性Brenner瘤的临床病理资料,并复习相关文献。结果 9例良性Brenner瘤特征是移行细胞型细胞组成的实性或囊性细胞巢,位于纤维瘤样间质内,囊腔内衬移行细胞型细胞、纤毛细胞、黏液细胞、立方细胞或扁平细胞,细胞无异型,细胞核卵圆形,核仁小而明显,部分有明显核沟。1例交界性Brenner瘤与良性Brenner瘤形态相近,但上皮细胞增殖程度超过良性Brenner瘤,细胞巢大小不一,层次增多,细胞轻度异型,但无间质浸润。4例恶性Brenner瘤有明显的间质浸润,并可见良性或交界性Brenner瘤成分。肿瘤细胞明显异型,核大深染,核分裂象易见,其中1例肿瘤累及输卵管和大网膜。免疫表型:14例肿瘤中CK7、p63均呈阳性,GATA-3阳性但部分低分化肿瘤区域失表达,CK20、Pax-8、WT-1均呈阴性,但黏液上皮化生区CK20呈阳性,Ki-67增殖指数恶性浸润区为20%～70%,交界性为3%,良性3%。结论卵巢Brenner肿瘤属于少见的卵巢上皮源性肿瘤,确诊需根据组织病理学特征和免疫表型。肿瘤细胞异型程度和有无间质浸润是良恶性Brenner瘤的鉴别关键。诊断恶性Brenner瘤必须有良性或交界性Brenner成分,与卵巢其他肿瘤的鉴别需结合临床病史、组织学特征和免疫表型。     

低度恶性纤维黏液样肉瘤1例及文献复习

目的探讨低度恶性纤维黏液样肉瘤(low-grade fibro-myxoid sarcoma,LGFMS)的临床病理学特征。方法对1例LGFMS进行大体、组织病理学和免疫组化染色观察,并复习相关文献。结果眼观:肿瘤大小约14 cm×11 cm×4 cm,边界清楚,无明显包膜,切面灰白色,质硬、韧。镜检:瘤细胞细长梭形或短梭形,富含瘤细胞胶原纤维区和瘤细胞稀少的大量黏液区混合存在,瘤细胞围血管形成旋涡状结构。瘤组织内可见长形或弓状簿壁血管,侵及周围横纹肌组织。免疫组化:瘤细胞表达vimentin和CD99。结论 LGFMS是一种具有局部复发和转移潜能的低度恶性肉瘤。     

肾嗜酸细胞腺瘤侵袭性的病理特征分析

目的探讨肾嗜酸细胞腺瘤侵袭性的临床病理学特征。方法回顾性分析4例肾嗜酸细胞腺瘤侵袭性的临床病理学及免疫表型特征,并复习相关文献。结果 4例嗜酸细胞腺瘤中1例侵及肾被膜纤维囊,1例侵透肾被膜纤维囊,2例侵及肾周脂肪;侵透肾被膜纤维囊者局部区域肿瘤细胞核异型明显。患者均行肾部分切除术,随访3个月~6年,无复发或转移。结论肾嗜酸细胞腺瘤虽然是一种良性肿瘤,但部分具有侵袭性的组织学特征,属于一种具有恶性潜能的肿瘤。由于部分病例具有局部异型性及侵袭性组织学特征,容易误诊为肾细胞癌而被过度治疗。因此正确评估肾嗜酸细胞腺瘤的预后,有助于对组织学特征的全面分析。     

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。     

非典型性纤维黄色瘤8例临床病理特征

目的 探讨非典型性纤维黄色瘤临床病理学特征、诊断及鉴别诊断。方法 收集8例非典型性纤维黄色瘤的临床资料，采用免疫组化EnVision两步法检测Connexin43、procollagen-1、CK(AE1/AE3)、EMA、CD68、CD99、vimentin、CD10、SMA、CD34、S-100、Ki-67的表达，并复习相关文献。结果 8例非典型性纤维黄色瘤患者，男女性各4例，年龄48～84岁，平均67.5岁；发生于面部4例，头颈部2例，右膝皮肤1例，右大腿皮肤1例；肿瘤最大径0.5～4.0 cm;临床均表现为皮肤表面隆起或溃疡。镜下肿瘤位于真皮内，由梭形细胞构成，细胞排列无序或呈条束状；可见散在分布的多形性细胞、多边形组织细胞样细胞、上皮样细胞、单核或多核瘤巨细胞，细胞异型明显，并可见病理性核分裂象。免疫表型：瘤细胞表达CD10(8/8)、CD68(8/8)、vimentin(8/8)、Connexin43(6/8)、procollagen-1(5/8)、CD99(1/8),不表达S-100、CD34、CK(AE1/AE3)、EMA、SMA,Ki-67增殖指数为5%～50%。结论...     

小肠隐睾并发精原细胞瘤的临床病理特点

目的:探讨隐睾并发精原细胞瘤的临床病理特征.方法:对2例手术切除的小肠精原细胞瘤进行临床、组织学及免疫组织化学观察.结果:肿瘤由大小基本一致的瘤细胞构成,被纤维间隔分隔成巢状或不规则条索状;细胞圆形或多角形,边界清楚;胞质较丰富,多数透亮,部分呈嗜酸性;核圆或椭圆,大而深染,基本位于中央,核分裂多见;可见灶状、片状或大...     

胃肠道SMARCA4缺失性未分化癌9例临床病理学分析

目的探讨胃肠道SMARCA4缺失性未分化癌的临床病理学特征、免疫表型及鉴别诊断。方法回顾性分析2018—2021年复旦大学附属肿瘤医院9例胃肠道SMARCA4缺失性未分化癌的临床资料, 总结其病理学特征和免疫表型, 并复习相关文献。结果患者中男性7例, 女性2例, 平均年龄58岁(范围39～74岁)。患者多以消化道症状就诊。肿瘤分别发生于胃(6例)、右半结肠(2例)和十二指肠(1例)。5例行手术治疗, 平均最大径6.7 cm(范围5.0～8.7 cm)。镜下观察见片状分布的圆形至上皮样未分化细胞, 核仁明显, 核分裂象易见, 局灶区域瘤细胞呈横纹肌样, 并失黏附性。瘤细胞形态相对一致, 但1例瘤细胞显示有明显的多形性, 1例含有梭形细胞成分。5例肿瘤内可见凝固性坏死, 1例间质伴有黏液样变性。免疫组织化学标记显示, 8例肿瘤完全失表达BRG1(SMARCA4)和BRM(SMARCA2), 1例部分失表达BRG1和BRM。除1例部分表达广谱细胞角蛋白外, 其余8例或极少量瘤细胞表达(<5%, 5例)或为阴性(3例)。此外, 4/7例、4/6例和4/5例还分别表达CD34、SOX2和...     

指趾纤维黏液瘤3例临床病理分析

目的探讨指趾纤维黏液瘤(digital fibromyxoma, DF)的临床病理学特征、诊断及鉴别诊断。方法回顾性分析3例DF的临床资料、病理学形态及免疫表型特征,并复习相关文献。结果 3例DF中男性2例,女性1例,年龄30～57岁,平均44岁,且病变均发生于手指。肿瘤最大径1.5～2 cm。镜下见肿瘤细胞呈梭形和星形纤维母细胞样,束状分布于黏液样基质内;黏液样基质富含纤细的血管,瘤细胞异型性不明显或仅轻度异型,核分裂象罕见。免疫表型:梭形和星形细胞表达CD34、CD10和CD99,灶性表达EMA,不表达MUC4、desmin和S-100蛋白,Ki-67增殖指数1%～2%。3例患者手术切除后均痊愈。结论 DF属少见的良性肿瘤,为防止局部复发,临床上应行肿瘤完整切除术。     

隆突性皮肤纤维肉瘤20例临床病理分析

20例隆突性皮肤纤维肉瘤临床特点为发生于真皮内、病程长、常反复局部复发。组织学特征为梭形瘤细胞围绕小血管排列成车辐状结构且遍及整个瘤体;瘤细胞成分单一、核轻度异型、核分裂象少见;瘤体常包裹较多脂肪细胞和皮肤附件;瘤组织常向皮下脂肪呈树枝状浸润性生长。本文介绍了新近的色素型和纤维肉瘤型两个亚型。     

Askin瘤尸检1例并文献复习

目的 探讨Askin瘤的临床病理特点、诊断及鉴别诊断.方法 总结1例Askin瘤患者的临床资料和尸检病理学特点;通过光镜和免疫组化及网状纤维染色,对尸体解剖后进行组织病理学观察.结果 临床上表现为青少年胸肺部肿块伴疼痛,患者生存期短,预后差.病理组织学特征主要为肿瘤组织呈弥漫性或结节状分布.组织学上以稀少的纤维血管间质将形态单一的小圆、卵圆形瘤细胞围绕成巢状或小叶状结构.免疫组化显示:瘤细胞CD99(+),NSE弱(+),CgA弱(+).网状纤维染色示肿瘤细胞间网状纤维少.结论 Askin瘤为一种位于胸肺部神经内分泌源性的高度恶性肿瘤,它实际上是PNET/Ewing肉瘤家族的一员,只是发生部位较特殊,预后不良.     

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.