Bcl-2和血管内皮生长因子受体3在子宫静脉内平滑肌瘤病与普通型平滑肌瘤中的表达差异

  目的  探索bcl-2和血管内皮生长因子受体3(vascular endothelial growth factor receptor-3, VEGFR-3)在子宫静脉内平滑肌瘤病(intravenous leiomyomatosis, IVL)及普通型平滑肌瘤(leiomyoma, LM)中表达的区别。  方法  回顾性分析20例IVL, 并匹配10例LM作为对照, 挑选存档组织块进行bcl-2和VEGFR-3免疫组织化学染色, 分析肿瘤细胞的表达情况并与LM进行对比分析。  结果  20例IVL病例bcl-2免疫组织化学染色全部呈中-强着色, 阳性率100%;VEGFR-3免疫组织化学染色19例呈中-强着色, 1例阴性, 阳性率95%。10例LM的bcl-2免疫组织化学染色均呈弱-中等着色, 阳性率100%;而VEGFR-3免疫组织化学染色则均为阴性, 阳性率为0。  结论  Bcl-2和VEGFR-3在IVL中均呈高表达, 尤其VEGFR-3表达与LM有明显差异, 提示IVL具有与LM不同的分子改变, 而这些分子改变可能在IVL的发生、发展中起重要作用。     

Titin抗原决定簇在正常胸腺、胸腺瘤和胸腺癌组织中的表达

  目的  探讨在正常胸腺、胸腺瘤、胸腺癌组织中titin抗原决定簇的表达及其与重症肌无力(myastheniagravis, MG)临床表现的关系。  方法  选取4例MG伴胸腺瘤患者的胸腺瘤、1例胸腺癌、1例MG伴胸腺瘤患者胸腺瘤旁增生的胸腺组织以及1例正常胸腺组织标本, 对所有标本作连续冰冻切片, 分别进行HE染色、细胞角蛋白(cytokeratin, CK)和titin抗原的免疫组化染色, 同时测定MG伴胸腺瘤患者血清titin抗体的浓度。  结果  免疫组化染色结果显示, titin在胸腺瘤中表达呈强阳性, 其分布与胸腺瘤上皮细胞的分布一致, 胸腺瘤内淋巴细胞和其他结构titin染色呈阴性; 在胸腺癌组织内titin染色呈阴性; 在正常胸腺中titin染色位于胸腺小体, 呈弱阳性, 其他结构未见着色。4例MG伴胸腺瘤患者中有3例血清titin抗体阳性。  结论  MG伴胸腺瘤患者的胸腺瘤组织中有titin抗原决定簇的表达, 且仅表达于胸腺瘤上皮细胞, 而正常胸腺组织中titin表达于胸腺小体, 提示伴MG的胸腺瘤中发生了免疫微环境的变化。     

CT及MRI诊断子宫平滑肌瘤病累及下腔静脉及右心腔

目的 观察子宫平滑肌瘤病累及下腔静脉及右侧心腔的CT及MRI影像学表现。方法 收集8例病理诊断为子宫平滑肌瘤病累及下腔静脉及右侧心腔患者,回顾性分析术前CT及MRI表现。结果 子宫平滑肌瘤病累及下腔静脉及右侧心腔CT及MRI影像学特点:病灶大部分游离于静脉或心腔内,中等不均匀强化,病变与子宫静脉平滑肌瘤原发灶或复发灶相延续;累及右心腔时可见似"拐杖头"或"蛇头"状改变。子宫平滑肌瘤病的特异性表现为子宫或宫旁血管增粗、纡曲成团。结论 CT及MRI不仅可显示子宫平滑肌瘤病累及下腔静脉及右心受累范围,同时可以检出盆腔原发或复发病变,对指导治疗及评价预后有重要意义。     

乳腺纤维瘤病的临床病理特征

  目的  探讨乳腺纤维瘤病的临床病理特征及鉴别诊断要点。  方法  回顾性分析5例乳腺纤维瘤病患者的临床资料并进行组织形态学、免疫组织化学研究, 对其临床表现、病理形态特点及鉴别诊断进行总结。  结果  5例均为女性, 发病年龄24~75岁, 平均年龄53岁。3例行乳腺病灶活检术, 2例行乳腺单纯切除术。乳腺纤维瘤病由梭形细胞(纤维母细胞/肌纤维母细胞)构成, 背景是丰富的胶原纤维, 有程度不等的玻璃样变性。细胞数量不等, 没有或仅有轻度的细胞不典型性和多形性, 核分裂象少见。免疫组织化学染色5例vimentin均阳性, SMA均部分阳性, 3例β-catenin阳性, 而cytokeratin、desmin、EMA、S-100和CD68均阴性。  结论  乳腺纤维瘤病是一种罕见乳腺肿瘤, 呈进行性、侵袭性生长。     

气管上段腺样囊性癌的临床病理

  目的  探讨气管上段腺样囊性癌的临床病理特征及诊断和鉴别诊断要点。  方法  收集2000年1月至2014年2月在北京协和医院确诊的4例气管上段腺样囊性癌病例, 通过光镜、免疫组织化学及组织化学染色分析其临床病理特征、免疫组织化学特点、诊断及鉴别诊断要点。  结果  4例气管上段腺样囊性癌患者中, 男1例, 女3例, 平均年龄47岁(38~57岁); 既往均无腺样囊性癌病史, 1例患者既往有结节性甲状腺肿手术史。镜下检查4例均为筛状/管状型腺样囊性癌, 3例累及甲状腺组织, 3例累及神经组织, 未见淋巴结受累。免疫组织化学染色示4例P16、CD117、BCL-2、P63、SMA均阳性, Ki-67指数平均8%, TTF-1和P53均阴性; 4例中基底膜样物胶原Ⅳ阳性; 组织化学染色示AB/PAS阳性。术后均接受总剂量为48~56 Gy的放疗, 随访6~120个月, 平均72.5个月, 1例术后96个月复发, 3例随访期间无复发及转移。  结论  气管上段腺样囊性癌是罕见的原发于气管的低度恶性肿瘤, 肿瘤生长缓慢, 就诊时多数已侵及甲状腺组织, 需要与原发于甲状腺的恶性肿瘤相鉴别, 特别是在甲状腺穿刺及术中冰冻检查时。结合电子喉气管镜下表现、典型的形态学及免疫组织化学和组织化学染色有助于准确诊断。手术难以切除干净, 术后放疗对延缓疾病复发有一定帮助。     

胸腺原发性黏膜相关淋巴组织淋巴瘤2例及文献复习

  目的  探讨胸腺原发性黏膜相关淋巴组织淋巴瘤的临床病理特征、免疫组化和基因重排特点。  方法  复习2例胸腺原发性黏膜相关淋巴组织淋巴瘤的临床资料, 通过光镜观察、免疫组化染色(EnVision, 抗体包括CD3, CD20, CD79α, CD5, Bcl-2, Bcl-6, CD10, CD23, CyclinD1, AE1/AE3)、聚合酶链式反应(polymerase chain reaction, PCR)分析其临床病理特征、免疫组化和基因重排特点。  结果  2例患者均为汉族, 男女各1例, 年龄分别为57岁和53岁, 女性患者伴干燥综合征(Sj?gren's syndrome, SS)及过敏性紫癜。术前CT、磁共振成像(magnetic resonance imaging, MRI)及术后大体检查均可见边界清楚的肿物, 内含小囊。镜下可见成片的小到中等大小弥漫淋巴细胞, 主要是中心细胞样细胞和/或单核样淋巴细胞, 偶见中心母细胞或免疫母细胞样细胞。小血管丰富, 血管周围可见较多浆细胞; 并可见多房囊肿, 囊壁被覆鳞状上皮细胞及柱状上皮细胞, 伴淋巴上皮病变, 囊壁Hassall小体增多并囊性退变, 内见粉染物; 存在反应性增生的淋巴滤泡, 周围脂肪组织内散见多处小结节。免疫组化显示CD20、CD79α和Bcl-2弥漫阳性, 囊肿上皮及赫氏小体(Hassall's corpuscle)AE1/AE3阳性, 上皮间可见CD20阳性的淋巴细胞浸润。PCR显示2例免疫球蛋白重链可变区(immunoglobulin heavy chain variable region, IgHV)基因重排阳性。2例患者均开胸行肿物及部分胸腺切除术, 术后分别随访27和6个月, 未见复发及转移。  结论  胸腺黏膜相关淋巴组织淋巴瘤少见, 具有明显特点:亚洲人多发, 伴有自身免疫性疾病, 大体及镜下可见多房囊肿, 血管周围可见明显的浆细胞分化, 缺乏凋亡抑制蛋白2-黏膜相关淋巴瘤转位基因1(API2-MALT1)融合基因等。手术切除为首选治疗, 临床结局良好。     

Cyclin D1在乳腺浸润性小叶癌的表达及其意义

  目的  探讨Cyclin D1在乳腺浸润性小叶癌中的表达及其意义。  方法  采用免疫组化染色方法对75例具有经典型乳腺浸润性小叶癌结构的标本进行Cyclin D1免疫组化检测。  结果  Cyclin D1阳性表达率为93.33%(70/75), 其中41例(54.67%)呈弥漫强阳性表达(3+, 6~7分), 21例(28%)呈中阳性表达(2+, 4~5分), 8例(10.67%)呈弱阳性表达(1+, 2~3分); 仅有5例(6.67%)呈阴性表达(0~1分)。Cyclin D1的阳性表达在患者年龄是否≥ 50岁、肿瘤最大径是否≥ 2 cm、淋巴结是否有转移、是否累及乳头间质及其下大导管、雌孕激素受体有无及P53是否突变等因素的分组中无统计学差异(P > 0.05);而Cyclin D1阳性表达程度与淋巴结是否有转移具有一定的负相关性(P < 0.05)。  结论  Cyclin D1在乳腺浸润性小叶癌中呈过表达状态, 该蛋白过表达提示其在基因水平上可能出现了某种程度的异常, 这一假设尚需进一步行基因检测加以证实。     

1例静脉内平滑肌瘤延伸至下腔静脉行胸腹联合术的护理体会

正静脉内平滑肌瘤病(intravenous leiomyomatosis,IVL)是一种具有不良生物学行为的特殊类型子宫肌瘤,其中有10%~30%的肿瘤累及下腔静脉、右心腔及肺动脉,成为心脏内平滑肌瘤病(intracardi-ac leiomyomatosis,IVL)~([1])。以下腔静脉或右心房病变为首发症状,该病发展极为隐袭,临床表现多样且缺乏特异性,诊断非常困难~([2])。本科于2015年6月收治1例静脉内平滑     

累及甲状腺的朗格汉斯组织细胞增生症6例报道及文献复习

  目的  探讨累及甲状腺的朗格汉斯组织细胞增生症(Langerhans' cell histocytosis, LCH)的临床特点。  方法  回顾性分析2004年1月至2014年12月北京协和医院明确诊断有甲状腺受累的6例LCH患者的临床资料并总结其临床表现、诊断、治疗和预后情况。  结果  6例患者中男性4例, 女性2例, 起病年龄9~24岁, 平均年龄(16.1±5.7)岁; 6例患者均有垂体后叶受累, 4例有肺脏受累, 3例有肝脏受累; 4例患者甲状腺自身抗体阴性, 2例阳性; 4例患者通过甲状腺粗针穿刺活检获得病理证实; 5例患者采用化疗方案治疗; 随访4例患者中3例病情稳定无进展。  结论  在有多器官受累的甲状腺肿大患者中, 需进一步排除LCH甲状腺受累的可能性, 必要时进行甲状腺穿刺, 特别是粗针穿刺以明确病理诊断, 同时在LCH甲状腺受累患者中要特别注意有无肝脏受累。     

子宫静脉内平滑肌瘤病临床病理及免疫表型分析

目的 探讨子宫静脉内平滑肌瘤病(IVL)的临床生物学行为、病理学特点及其免疫表型的临床意义.方法 应用免疫组化方法 检测子宫IVL肿瘤细胞vimentin、desmin、SMA、CD10、CD34、ER和PR的表达情况.结果 6例患者术前均诊断为子宫多发性平滑肌瘤,未曾怀疑为IVL;3例行全子宫切除术,3例行全子宫及双侧附件切除术,其中1例术中见灰白色条索样肿物蔓延致右侧宫角和宫旁组织静脉内;病理检查发现6例标本除合并血管外子宫平滑肌瘤外,子宫肌壁间大小不一的静脉内见平滑肌细胞构成的瘤栓.6例患者经随访6～51个月未见复发.免疫组化染色显示,IVL瘤细胞vimentin、desmin和SMA(+),CD10和CD34(-);其中5例肿瘤细胞ER和PR呈强(+),1例呈(+).结论 IVL是组织学表现为良性却具有类似恶性肿瘤生长方式的一类罕见肿瘤,有远期复发的倾向.IVL肿瘤细胞存在雌、孕激素高表达特性,提示卵巢去势治疗或术前、术后应用雌激素拮抗剂可能是有效的辅助治疗手段.     

Intravenous leiomyomatosis: CT findings

Intravenous leiomyomatosis (IVL) is a rare smooth muscle tumor. Although IVL is histologically benign, it might be aggressive in its behavior and can grow into pelvic veins and the inferior vena cava (IVC) extending into the heart chambers and pulmonary vasculature. Occasionally, it was found to have lung metastasis. We describe four cases of IVL in the IVC with a history of hysterectomy for uterine leiomyoma, one extending into the left renal vein and three growing into the right heart chamber. We report the computed tomography (CT) findings in the four cases and briefly discuss the CT features of IVL in order to help making accurately preoperative diagnosis and improve the rate of surgical resection and survival.     

超声诊断静脉内平滑肌瘤病(附7例报告)

目的 回顾性分析静脉内平滑肌瘤病(IVL)的超声表现,提高对IVL的认识和诊断水平.方法 回顾性分析7例经手术病理证实确诊为IVL临床资料,总结所累及脏器的超声表现.结果 7例均为女性,6例有子宫肌瘤病史,5例已行子宫切除手术,1例超声见子宫肌壁间多发结节及盆腔多发肿物.7例血管彩色多普勒超声均发现一侧髂内静脉伴髂外静脉或髂总静脉占位,并见下腔静脉不同段实性中等回声占位:4例累及下腔静脉下段;3例累及下腔静脉全程,其中2例向上延伸至右心房.结论 IVL的临床表现复杂多样,超声表现有一定的特点,掌握IVL超声特点有助于提高诊断正确率.     

Interrupted inferior vena cava and left-sided subrenal inferior vena cava: prenatal diagnosis.

An interrupted inferior vena cava (IVC) and a left-sided subrenal (postrenal) IVC with azygos or hemiazygos continuation are very rare anomalies of the IVC. The prenatal sonographic and color Doppler features of these anomalies are reported here.     

下腔静脉超声指标对慢性肾衰竭合并高血压的维持性透析患者容量状态评价

目的探究下腔静脉（IVC）超声指标对接受维持性透析的慢性肾衰竭合并高血压患者的容量状态评估价值。方法纳入我院肾内科拟接受维持性透析治疗的慢性肾衰竭合并高血压的患者56例,根据CT心胸比值将患者分为容量过负荷组和容量正常组,于透析治疗前使用床旁超声动态监测下腔静脉内径（D-IVC）,并计算下腔静脉随呼吸变异度（V-IVC）。观察并分析两组间各指标的差异。通过受试者工作特征（ROC）曲线评价各指标对容量状态的预测价值。并将呼气末下腔静脉内径（D-IVCe）、V-IVC分别与CT心胸比进行相关性分析。结果容量过负荷组较容量正常组具有较高的D-IVCe（P=0.005）和较低的V-IVC（P=0.01）。以D-IVCe和V-IVC分别取17.7mm和30%为阈值,预测容量过负荷的敏感度为58.3%和81.3%,特异度为81.2%和58.3%,ROC曲线下面积分别为0.719（95%CI:0.584~0.855）和0.696（95%CI:0.554~0.838）。相关性分析显示,CT心胸比与D-IVCe呈正相关性（r=0.607）,与V-IVC呈负相关性（r=-0.585）。结论下腔静脉超声指标对慢性肾衰竭合并高血压的维持性透析患者容量状态有较好的评估价值。     

自然组织谐波显像技术评价左室收缩功能

目的：评价自然组织谐波显像技术（ＮＴＨＩ）测量左室收缩功能的准确性。方法：利用测定２０例患者的左室容量和射血分数,并与左室造影的测值进行比较。结果：ＮＴＨＩ所测左室容量和射血分数与左室造影相应测值高度相关,左室舒张末期容量和收缩末期容量及射血分数的相关系数分别为０．８７,０．９６,０．９２。ＮＴＨＩ左室容量测值低估约３０％。结论：超声ＮＴＨＩ技术能准确可靠测定左室容量和射血分数,为评价左室收缩功能提供简便易行的方法。     

Prenatal diagnosis of cardiosplenic syndromes: a 10-year experience.

OBJECTIVE: To assess the accuracy of fetal echocardiography in the prenatal diagnosis of cardiosplenic syndromes and the spectrum of associated anomalies. METHODS: This was a retrospective survey of fetuses in our databases over a period of 10 years with postnatally confirmed prenatal diagnosis of cardiosplenic syndromes. RESULTS: In 32 of 35 fetuses the prenatal diagnosis of cardiosplenic syndromes was confirmed postpartum. Twenty-two fetuses had left isomerism. Their main prenatal ultrasound features were interrupted inferior vena cava (n = 21), complete atrioventricular septal defect (n = 15), viscerocardiac heterotaxy (n = 15), persistent bradyarrhythmia (n = 12) and fetal hydrops or nuchal edema (n = 12). Twelve pregnancies were terminated, two fetuses were stillborn and eight infants survived. Ten fetuses had right isomerism. Their main sonographic features were juxtaposition of the descending aorta and inferior vena cava (n = 7), complete atrioventricular septal defect (n = 7), left persistent superior vena cava (n = 6) and viscerocardiac heterotaxy (n = 6). In this group there was one stillbirth, five infant deaths and four survivors. The overall survival rate and spectrum of other cardiac malformations were similar between the two groups. Prenatal diagnosis of other visceral features of cardiosplenic syndromes was inconsistent. CONCLUSION: Cardiosplenic syndromes can be diagnosed with high accuracy by prenatal sonography. A diagnosis of left isomerism should be strongly suggested in the presence of a combination of at least two of the following: (1) complete atrioventricular septal defect or other structural heart disease; (2) interruption of inferior vena cava with azygos continuation; (3) early fetal heart block; (4) viscerocardiac heterotaxy. Right isomerism should be suspected in the presence of a combination of at least two of the following: (1) structural heart disease, namely complete atrioventricular septal defect; (2) juxtaposition of inferior vena cava and descending aorta; (3) viscerocardiac heterotaxy.     

Leiomyosarcoma of the inferior vena cava presenting as a cardiac mass

Primary tumors of the inferior vena cava (IVC) are rare. The most common primary malignancy is leiomyosarcoma (LMS), with a severe prognosis. Right‐atrium (RA) involvement is seen in about 20 percent of cases. We report the case of a 53‐year‐old woman admitted to the intensive care unit because of progressive dyspnea with lower limb edema. Echocardiography demonstrated a large mass in the RA. At open‐heart surgery, the tumor appeared located in the IVC infrahepatic segment, completely obstructing the lumen and extending into the suprahepatic portion and the RA. Histopathological examination concluded to LMS.     

Scimitar syndrome; an unusual congenital abnormality occasionally seen in adults

Scimitar syndrome a very rare and variable congenital disorder characterized by an anomalous connection of the pulmonary vein with the IVC. The syndrome is mostly seen in very early infancy, but was now recognized in a 46-year-old woman, who was referred to the outpatient clinic of the department of cardiology with complaints of dizziness. Contrast enhanced computer tomography (CT) showed dextroposition of the heart and a large right pulmonary vein joined the inferior vena cava (IVC) just above the level of the diaphragm. The typical features of the syndrome are discussed.     

Sonography and Transthoracic Echocardiography for Diagnosis of Systemic Cardiovascular Metastatic Tumor Thrombi

Sonography and transthoracic echocardiography (TTE) are seldom used for assessment of metastatic tumor thrombi in the cardiovascular system in routine clinical practice. We performed this retrospective study to evaluate the combination of sonography with TTE for diagnosis of metastatic tumor thrombi in heart and systemic vessels. Vascular, abdominal, pelvic, and small‐part sonography was applied in 18 patients, and TTE was conducted simultaneously in 14 patients. Tumor thrombi invaded into the inferior vena cava system in 12 patients, superior vena cava system in 5 patients, and aorta in 1 patient; they extended to the right cardiac chambers in 11 patients. Six patients had diagnoses by pathologic examination. The primary neoplasms were identified by conventional imaging in 17 patients. The morphologic and echogenic characteristics of the tumor thrombi were diverse and depended on their original tumors. The thrombi were either contiguous or discrete from the original tumors. The neoplastic vascularity of the thrombi and the invasive extension were the primary characteristics that distinguished them from bland thrombi. Simultaneous application of sonography and TTE is a feasible way to comprehensively evaluate cardiovascular metastatic tumor thrombi in most patients.     

Focused cardiac ultrasound as a predictor of readmission in acute decompensated heart failure

Acute decompensated heart failure (ADHF) is a common reason for admission to the hospital, and readmission is frequent. Multiple factors contribute to rehospitalizations, but inadequate assessment of volume status leading to persistent congestion is an important factor. We sought to determine if focused cardiac ultrasound (FCU) of the inferior vena cava (IVC), as a surrogate of volume status, would predict readmission of ADHF patients after index hospitalization. Patients admitted with a primary diagnosis of ADHF were prospectively enrolled. All patients underwent FCU of the IVC on admission and then daily. 82 patients were enrolled. Patients demonstrated improvement in heart failure physical examination findings and symptoms during the hospitalization. There was a reduction in the size of the IVC and a significant increase in patients with small collapsible vena cava. Logistic regression analysis of physical examination, patient symptoms, and IVC parameters at discharge demonstrated IVC collapsibility and patient reported dyspnea improvement as the only significant variables to predict readmission or emergency department visit. FCU assessment of IVC size and collapsibility may be useful in patients with ADHF to predict risk of being readmitted within 30 days of hospital discharge.