羊水栓塞2例临床病理分析

目的：探讨羊水栓塞的病理诊断依据。方法收集2例羊水栓塞患者的血液样本及切除的子宫标本,对血液涂片、组织及血液提取物沉渣石蜡包埋,行HE染色、免疫组化EnVision两步法染色及 Giasma 染色,观察其病理学特征。结果在HE染色的子宫壁静脉内找到羊水成分,免疫组化标记角化上皮CK及CK14阳性,CD31阴性;血液标本经离心沉渣包埋切片,可见血液上层有角化上皮及黏液样物质, Giasma染色角化上皮胞质呈粉红色,黏液样物质呈紫蓝色。结论羊水栓塞的病理诊断应先取患者的下腔静脉血或切除子宫,查找是否存在羊水成分,对可疑线索以HE切片形态学为基础,免疫组化及特殊染色作为辅助的方法。     

黏液在羊水栓塞病理诊断中的价值

目的探讨黏液在羊水栓塞(amniotic fluid embolism,AFE)病理诊断指标中的价值。方法从12例AFE中提取15份标本(6例尸解、其中3例尸解同时取腔静脉血、6例腔静脉血)采用HE染色、黏液(p H 2. 5)染色及免疫组化En Vision法标记CKpan、CD34、CR,显微镜下观察、计数羊水有形成分。另收集15例作为对照组进行对比分析。结果在6例AFE尸解病例的肺小血管中及9例腔静脉血标本中检出不同比例的羊水有形成分,其中上皮占23. 56%,黏液占65. 60%,脂肪占1. 27%,胎粪占8. 91%,毳毛占0. 63%。5种有形成分的检出率总体比较,黏液(P 0. 001)、上皮(P 0. 001)、脂肪(P 0. 001)阳性检出物显著高于对照组,两者差异有统计学意义;胎粪(P=0. 430)及毳毛(P=0. 183)两者差异无统计学意义。结论 AFE的黏液检出率显著高于对照组,而在检出的羊水有形成分中黏液检出率较高,其在AFE病理诊断中有较高的应用价值。     

肺原发性良性黏液上皮性病变冷冻切片误诊为黏液腺癌12例分析

目的探讨冷冻切片误诊为黏液腺癌的良性黏液上皮性病变的病理形态学特征,并分析联合使用免疫组化标记CK5/6及p63的鉴别诊断价值。方法收集宁波临床病理诊断中心2011年8月～2018年1月冷冻切片误诊为黏液腺癌的良性黏液上皮性病变12例,行CK5/6、p63免疫组化染色。结果冷冻切片误诊的12例病变最大径为0.4～1.2 cm,平均0.8 cm,病变边界清楚,黏液上皮间可见纤毛柱状上皮(12/12),CK5/6及p63标记可见完整的基底细胞层(12/12)。12例中,位于肺外周9例,位于肺中央区3例。结论良性黏液上皮性病变都具有双层结构,黏液上皮之间可见到纤毛柱状上皮,细胞缺乏异型性,当病变位于肺外周,黏液上皮丰富,纤毛柱状上皮零散分布时易误诊为黏液腺癌。     

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。     

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。     

原发性甲状腺上皮样血管肉瘤1例报告

目的:观察1例原发性甲状腺上皮样血管肉瘤患者的临床病理特征,并结合相关文献进行讨论。方法和结果:本例原发性甲状腺上皮样血管肉瘤患者行术中快速冰冻切片及常规石蜡切片,HE染色和网状纤维染色、并行免疫组化检查。镜下见,肿瘤主要由实性片状或巢状排列的上皮样细胞组成,肿瘤细胞核较大,呈空泡状,核仁明显,部分肿瘤细胞胞质内可见含有红细胞的空泡或小管腔形成;可见迷路状血管,网状纤维染色能清晰显示这些管腔结构。免疫组化染色,肿瘤细胞CD31、CD34、AE1/AE3阳性,TG、TTF-1、calcitonin、CK19、S-100、EMA和HMB45阴性,Ki-67高表达。结论:原发性甲状腺上皮样血管肉瘤是一种少见的恶性肿瘤,致死率高。确诊主要依靠组织学和免疫组化检查,临床要与未分化癌、低分化癌及上皮样血管内皮瘤等鉴别。     

挖空细胞的本质及尖锐湿疣病理诊断

目的 从分子生物学角度，寻找尖锐湿疣比较确切可行的病理诊断标准。方法 采用HE、HPV-CAg免疫组化、HPV-DNA原位杂交组化染色和超薄切片等方法检测50例尖锐湿疣；用原位末端标记TUNEL染色，对20例尖锐湿疣进行观察。结果 鳞状上皮呈尖细的乳头状增生，角化不全和乳头纤维轴索中毛细血管丛状增生，棘层中上部和颗粒层有多少不等的挖空细胞，单个散在、成群或广泛分布，胞质空亮，核皱缩成星形或毛虫样，无炎细胞反应。原位杂交组化HPV-DNA的阳性细胞比免疫组化HPV-CAg阳性细胞明显增多。原位末端标记TUNEL染色及光镜、电镜观察，显示所有的挖空细胞均呈现细胞凋亡变化。结论 尖锐湿疣挖空细胞是由HPV所引起的凋亡细胞。从形态学上确认其凋亡特征即可确定尖锐湿疣的诊断，角化不全和乳头间质血管丛状增生可作佐证。难以确认挖空细胞的疑难病例，可采用原位杂交组化技术。     

阴茎假血管肉瘤样鳞状细胞癌1例及文献复习

目的探讨阴茎假血管肉瘤样鳞状细胞癌(SCC)的诊断、组织学和免疫组化特征以及鉴别诊断。方法通过HE、免疫组化及特殊染色观察1例阴茎原发假血管肉瘤样鳞状细胞癌，并复习文献。结果镜下可见显著的棘细胞松解，肿瘤由排列呈腔隙状、网状结构的长梭形细胞组成，细胞异型性明显，核分裂象易见，胞质大多红染，局部区域弥漫出血坏死。免疫表型：CK、Vim阳性，CD34、CD31、SMA、FⅧRAg阴性。网状纤维染色不显示血管外形。结论假血管肉瘤样SCC是一种罕见的易与敌国管肉瘤混淆的特殊形态的SCC．需与血管肉瘤、癌肉瘤等鉴别。     

血管树突状细胞在人主动脉粥样硬化早期病变中的分布

目的：探讨血管树突状细胞在人早期动脉粥样硬化(AS)病变中的分布模式。方法：人主动脉标本15例主要取自尸检和外科手术，常规连续切片，分别行HE及S100／CD1a免疫细胞化学染色，光镜下观察S100／CD1a阳性细胞分布情况。结果：15例HE染色标本中，2例正常，13例人动脉血管可见内膜的增厚及泡沫细胞等AS早期病理表现。9例S100／CD1a染色阳性，阳性率为69．2％。S100／CD1a阳性细胞分布在病变的内膜和外膜，外膜的S100／CD1a阳性细胞主要分布在滋养血管的周围。结论：在AS早期病变部位有血管树突状细胞的聚集，主要分布在病变血管的内膜和外膜，提示血管树突状细胞可能参与了AS早期的免疫反应。     

肺上皮样血管内皮瘤的临床病理观察

目的探讨肺上皮样血管内皮瘤(pulmonaryepithelioidhemangioendothelioma,PEH)的临床病理特征、诊断及鉴别诊断。方法对3例PEH进行光镜观察和免疫组化标记,并结合文献进行分析。结果镜下见肿瘤细胞具有上皮样或组织细胞样形态,瘤细胞为圆形或多角形,呈小巢状、索状甚至腺样结构或不规则状,分布于黏液间质中;间质可见少量黏液样变或玻璃样变;瘤细胞内含有原始血管腔,核分裂象、多形性及坏死少见。肿瘤细胞表达CD34、FⅧRAg、CD31等血管内皮细胞标记,部分病例同时表达CK和(或)Vim。结论PEH是一种低度恶性肿瘤,其病理形态具有一定的特征性,诊断时需要与肺转移癌、肺上皮样血管肉瘤、肺淋巴管肌瘤病鉴别。     

Maternal death analysis based on data from the nationwide registration system in Japan (2010–2018)

The maternal mortality rate in Japan was 3.5 per 100 000 live births in 2017, similar to that reported in other developed countries. To reduce the number of maternal deaths, a Japanese nationwide registration and analysis system was implemented in 2010. Between January 2010 and April 2018, 367 maternal deaths were reported. Among them, by reviewing 80 autopsy records, the direct obstetric causes of death were identified in 52 women. The major causes of deaths were amniotic fluid embolism and acute pulmonary thromboembolism. The other 26 maternal deaths were associated with indirect obstetric causes including invasive Group A Streptococcus infection, aortic dissection, cerebral stroke and cardiomyopathies. This review highlights the importance of autopsy in maternal deaths. On analyzing 42 autopsy specimens obtained from registered cases of maternal death during 2012–2015, the 36% of causes of death by autopsy were discordant with the clinical diagnosis. Moreover, of the 38% of non-autopsied maternal death, the cause of death could not be clarified from the clinical chart. We emphasized that detailed autopsies are necessary to clarify the precise pathologic evidence related to pregnancy and delivery, especially causes of unexpected death such as amniotic fluid embolism.     

Maternal Death Following Cardiopulmonary Collapse After Delivery: Amniotic Fluid Embolism or Septic Shock Due to Intrauterine Infection?

Citation Romero R, Kadar N, Vaisbuch E, Hassan SS. Maternal death following cardiopulmonary collapse after delivery: amniotic fluid embolism or septic shock due to intrauterine infection? Am J Reprod Immunol 2010; 64: 113–125 Problem The amniotic fluid embolism (AFE) syndrome is a catastrophic complication of pregnancy frequently associated with maternal death. The causes and mechanisms of disease responsible for this syndrome remain elusive. Method of study We report two cases of maternal deaths attributed to AFE: (1) one woman presented with spontaneous labor at term, developed intrapartum fever, and after delivery had sudden cardiovascular collapse and disseminated intravascular coagulation (DIC), leading to death; (2) another woman presented with preterm labor and foul‐smelling amniotic fluid, underwent a Cesarean section for fetal distress, and also had postpartum cardiovascular collapse and DIC, leading to death. Results Of major importance is that in both cases, the maternal plasma concentration of tumor necrosis factor‐α at the time of admission to the hospital and when patients had no clinical evidence of infection was in the lethal range (a lethal range is considered to be above 0.1 ng/mL). Conclusion We propose that subclinical intraamniotic infection may be a cause of postpartum cardiovascular collapse and DIC and resemble AFE. Thus, some patients with the clinical diagnosis of AFE may have infection/systemic inflammation as a mechanism of disease. These observations have implications for the understanding of the mechanisms of disease of patients who develop cardiovascular collapse and DIC, frequently attributed to AFE. It may be possible to identify a subset of patients who have biochemical and immunological evidence of systemic inflammation at the time of admission, and before a catastrophic event occurs.     

Comparison of clinical and post-mortem findings in intensive care unit patients

The autopsy has long been regarded as an important tool for clinical confrontation, education and quality assurance. The aims of this study were to examine the correlation between the clinical diagnosis and autopsy findings in adult patients who died in an intensive care unit (ICU) and to identify the types of errors in diagnosis to improve quality of care. Autopsies from 289 patients who died in the ICU during a 2-year period were studied. Post-mortem examination revealed unexpected findings in 61 patients (21%) including malignancy, pulmonary embolism, aspergillosis, myocardial or mesenteric infarction and unsuspected bacterial, viral or fungal infection. These unexpected findings were classified as Goldman class I errors in 17 (6%), class II in 38 (13%) and class III in six (2%) cases. Although the incidence of unexpected findings with clinical significance was low, post-mortem examination remains a valuable source of pertinent information that may improve the management of ICU patients.     

Amniotic fluid embolism

The disastrous entry of amniotic fluid into the maternal circulation leads to dramatic sequelae of clinical events, characteristically referred to as Amniotic fluid embolism (AFE). The underlying mechanism for AFE is still poorly understood. Unfortunately, this situation has very grave maternal and fetal consequences. AFE can occur during labor, caesarean section, dilatation and evacuation or in the immediate postpartum period. The pathophysiology is believed to be immune mediated which affects the respiratory, cardiovascular, neurological and hematological systems. Undetected and untreated it culminates into fulminant pulmonary edema, intractable convulsions, disseminated intravascular coagulation (DIC), malignant arrhythmias and cardiac arrest. Definite diagnosis can be confirmed by identification of lanugo, fetal hair and fetal squamous cells (squames) in blood aspirated from the right ventricle. Usually the diagnosis is made clinically and by exclusion of other causes. The cornerstone of management is a multidisciplinary approach with supportive treatment of failing organs systems. Despite improved modalities for diagnosing AFE, and better intensive care support facilities, the mortality is still high.     

The pathologic findings of the fetal membranes in very prolonged amniotic fluid leakage

We examined the fetal membranes in five patients with prolonged amniotic fluid leakage. Four patients had a clinical history of fluid leakage of at least six weeks' duration, while, in the fifth patient, prolonged leakage was only an inferred diagnosis. Four of the infants died within the first two days of life, while one infant survived. The pathologic findings were varied. Two cases showed, to our knowledge, a previously unreported subchorionic accumulation of squames, which were presumably from cells that were shed into amniotic fluid. One other case showed a subchorionic foreign-body reaction. The two remaining cases showed only necrosis and hemorrhage.     

儿童腺泡状软组织肉瘤13例临床病理学特征

目的探讨儿童腺泡状软组织肉瘤(alveolar soft part sarcoma,ASPS)的临床病理、分子遗传学特点、诊断及鉴别诊断。方法对北京儿童医院2009年8月至2018年11月13例儿童ASPS病例存档切片行HE染色及组织化学染色[包括过碘酸-雪夫(PAS)染色及淀粉酶消化PAS(D-PAS染色)]。采用免疫组织化学染色检测TFE3、INI1、CD68等的表达,应用荧光原位杂交(FISH)法检测TFE3基因断裂易位情况。结果13例ASPS中,男童4例,女童9例,年龄1岁2个月至13岁8个月,平均7.8岁,5岁以下4例。组织学上,11例肿瘤细胞呈腺泡状、巢状排列,2例肿瘤细胞呈实性、弥漫性生长。瘤细胞胞质嗜酸性,可见明显的空泡现象,核多形性,核仁突出,核分裂象罕见,3例可见血管浸润。免疫组织化学染色TFE3弥漫核阳性,INI1、CD68、波形蛋白阳性,MyoD1、Myogenin、细胞角蛋白、S-100蛋白等均阴性。7例PAS及D-PAS染色显示肿瘤细胞质内均可见紫红色针状或棒状结晶体。9例行FISH检测,均显示TFE3基因断裂易位。结论ASPS为儿童少见软组织肿瘤,肿瘤多位于深部肌肉内,瘤细胞排列成腺泡状或巢状,同时TFE3基因位点发生断裂易位,确定诊断需要结合临床、病理形态、免疫组织化学及基因检测综合考虑。     

羊水栓塞救治成功5例的临床研究

目的探讨羊水栓塞（AFE）的有效救活措施。方法回顾性分析我院经治的5例AFE病例，对其临床救治过程进行剖析，总结抢救成功因素。结果剖宫产术中发生的4例急性AFE，采用给氧、大剂量地塞米松、肾上腺素、阿托品、多巴胺等综合急救，全部救治存活。产后以出血为主的1例AFE经纠正DIC、子宫切除后救治存活。结论肾上腺素在救治急性AFE时有使用价值。AFE发生难以纠正的DIC时，切除子宫是重要治疗措施。     

Amniotic fluid embolism with haemostasis complications: primary fibrinogenolysis or disseminated intravascular coagulation?

Amniotic fluid embolism (AFE) is characterized by the passage of amniotic fluid (AF) into the maternal circulation during or just after childbirth. AFE is a rare disorder occurring in 1/8,000 to 1/80,000 deliveries but with a maternal morbidity ranging from 26% in a recent report to 86% in earlier ones. In patients who survive, AFE may affect coagulation resulting in severe bleeding. While disseminated intravascular coagulation (DIC) is usually seen in such cases, we reported a case of AFE in which the hemostatic abnormalities were compatible with primary fibrinogenolysis rather than with DIC.     

Correlation of clinical diagnoses with autopsy findings: A retrospective study of 2,145 consecutive autopsies

The protocols of 2,145 autopsies were retrospectively reviewed and the findings compared with the clinical diagnoses. A sudden decline in the autopsy rate that occurred during the period studied was followed by a highly statistically significant difference in clinical accuracy (P less than 0.01), in favor of the predecline period. The overall rate of major discrepancies was 29 per cent. The most frequently missed diagnoses were infections, which were found in 26 per cent of all autopsies and had not been diagnosed clinically in 63 per cent of these cases. Malignancies occupied second place among overlooked diagnoses in the selected disease categories; in 99 per cent of the cases the malignancy was the principal diagnosis, and it had been misdiagnosed clinically in 42 per cent of these cases. Cerebrovascular disorders were correctly diagnosed in most cases (87 per cent of the patients in this group). Among autopsy diagnoses labeled as the immediate causes of death, the most frequently overlooked were pulmonary embolism and gastrointestinal hemorrhage, which were not recognized in 84 and 78 per cent, respectively. In cases in which clinicians were not entirely confident in their impressions, their diagnoses were usually confirmed at autopsy. In these cases 15 per cent of the patients died soon after admission to the hospital, with accurate diagnoses in 71 per cent. The discrepancies disclosed should be regarded as sufficiently large to mandate continued emphasis on autopsy evaluation as the basis for the control of the quality of patient care.     

肠管子宫内膜异位6例临床病理分析

目的 探讨肠管子宫内膜异位的临床病理特征。方法 对6例肠管子宫内膜异位病变组织连续切片，并行HE和免疫组化染色。结果 肠管子宫内膜异位局部可出现继发性组织学改变和伴随相应的临床表现；ER和PR在异位内膜上皮呈强阳性表达，CK(AE1)在肠上皮呈强阳性表达。结论 肠管子宫内膜异位患者的临床表现与病变肠段的解剖部位和累及肠壁的组织学层次密切相关；异位内膜可引起局部肠黏膜腺体增生和息肉形成；ER、PR和CK(AE1)是鉴别肠源性上皮与子宫内膜异位上皮的重要标志。